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Examination revealed generalized lymph node enlargement along with hepatomegaly.

A 45 year old Indian female of Nordic origin presented 5 years back with a lump in the right breast and the axilla. She underwent modified radical mastectomy. Histophotomicrograph of the excised breast lesion showed a 2.1 cm duct carcinoma, positive for ER and PR with 1 out of 25 lymph nodes positive for metastasis. She received 6 cycles of chemotherapy with cyclophosphamide, epirubicin, and 5-fluorouracil. This was followed by tamoxifen 20 mg per day for five years. She was doing well on follow up until the completion of fifth year of her disease, when she presented with complaints of mild fever and weakness. Examination revealed generalized lymph node enlargement along with hepatomegaly. Hemogram showed mild anemia, normal platelet count and a leukocyte count of 1.2 x 10(11)/L. Peripheral blood examination revealed medium sized lymphoid cells, constituting almost 75% of total nucleated cell population. Immunophenotying, established a diagnosis of post thymic T-cell prolymphocytic leukemia. Contrast-enhanced computed tomography of the chest and abdomen was done which revealed an anterior mediastinal mass with destruction of sternum along with multiple small nodular shadows in bilateral lung fields suggestive of lung metastasis. Fine needle aspiration cytology of the mass showed atypical ductal cells with nuclear pleomorphism, which were positive for ER, PR and Her2neu protein. This confirmed a co-existent metastatic breast carcinoma. She was started on chemotherapy for T-PLL along with hormonal therapy with aromatase inhibitor. Unfortunately, both her malignancies progressed after an initial stable disease of two months.

EN108139

In the immediate postoperative period, the patient developed hemodynamic collapse requiring the introduction of dobutamine.

A 50-years-old woman, hypertensive, hospitalized for a large cervical mass appeared 30 years ago. In the family history, her mother, sisters and cousins underwent a surgery for MNG. Despite of the large volume of the mass, the patient never described signs of cervical compression whatsoever respiratory, digestive, laryngeal, vascular or neurologic signs. She never suffered from thyroid dysfunction. Her neck was deformed by the voluminous formation classified grade III according to the WHO modified classification. The mass took the front and the two sides of the neck. Its surface was embossed and covered by a thin normal skin. There were some veins of the collateral circulation limited to the neck. The goiter measured 18 x 11 cm. The mass was firm, painless, and mobile with the swallowing movements. Lymphadenopathy research was difficult and found no palpable lymph nodes. The laboratory tests (T 3, T 4 and TSH) were normal. Thoracic radiography showed a large cervical opacity roughly round and strewn with microcalcifications associated with a right eccentricity of the trachea. Cervical and chest CT revealed the presence of a partially calcified thyroid mass slightly plunging in the anterior mediastinum. It took heterogeneously the contrast and then evocate a large MNG. The trachea was surrounded by the goiter, slightly narrowed and right deviated as well as the lower part of the larynx. The right and left vascular axes of the neck (carotid artery and jugular vein) were deviated backward. The patient underwent a surgery for her enormous MNG slightly plunging in the mediastinum. Endotracheal intubation was relatively easy by the laryngoscope. The incision performed was a Kocher cervicotomy. There was a multinodular, hypervascularized goiter. Its lower end plunges behind the sternal manubrium. The larynx was deviated towards the right side. The total thyroidectomy was performed in two steps: initially a right lobo-isthmectomy, then the left lobectomy. The retrosternal part of the goiter was released using the finger by the same incision. Both recurrent laryngeal nerves (RLN) were not identified because of the hemorrhage. One parathyroid gland was accidently devascularized and was autotransplanted to the ipsilateral sternocleidomastoid muscle. The operation was finished by double aspiration drainage. In the first hours after surgery, the patient developed a large cervical hematoma. She was readmitted to the operating room, and after evacuation of the hematoma there was no vessels bleeding. The operation was completed with a double suction drainage. In the immediate postoperative period, the patient developed hemodynamic collapse requiring the introduction of dobutamine. After 48 hours of hemodynamic support, the blood pressure stabilized and dobutamine was stopped. Histological study concluded in multinodular colloid goiter. The patient was discharged from the hospital after 20 days in good health.

EN100067

A 20 years old man presented with fever, myalgias, arthralgias, retro-orbital pain, vomiting and gum bleeding during a large dengue outbreak in Lahore, Pakistan.

A 20 years old man presented with fever, myalgias, arthralgias, retro-orbital pain, vomiting and gum bleeding during a large dengue outbreak in Lahore, Pakistan. On 7th day of illness patient became afebrile, but he developed severe headaches, unconsciousness followed by altered behavior. On 9th day of illness patient developed painful neck swelling accompanied by fever, tremors, palpitations, hoarseness of voice and odynophagia. Examination revealed acutely swollen, tender thyroid gland along with features of hyperthyroidism. Laboratory evaluation revealed stable hematocrit, thrombocytopenia and leukopenia. Patient had seroconverted for anti-dengue IgM antibodies on the 10th day of illness. A non-contrast Computed Tomogram (CT) of the brain showed right frontal lobe hematoma. Thyroid profile showed increased free T3 and T4 and low TSH. Technetium thyroid scan showed reduced tracer uptake. He was diagnosed as having subacute thyroiditis and treated with oral prednisolone and propranolol. Follow up CT brain showed resolving hematoma. Patient's recovery was uneventful.

EN107021

The patient was also given micronutrient supplementations, specifically vitamin A 100,000 units for the first week, vitamin C 100 mg 3 times a day, and zinc 20 mg once a day.

A 35-year-old mentally ill man was transferred from a local psychiatric hospital after attempted suicide by fire 3 days before admission. He acquired full-and partial-thickness injury in approximately 38% of his TBSA, including face and neck (5%), both upper extremities (8%), right thigh (4%), left thigh and leg (3%), and most of his anterior and posterior trunk (16%). The patient was hospitalized for 66 days and underwent five debridement surgeries. The psychiatric diagnosis was schizophrenia type ICD 10 F20.3, treated with Haloperidol 1.5 mg and Diazepam 5 mg daily. The patient was occasionally restrained due to rebellious and self-destruction behavior. The nutrition management of this patient was started after a week of hospitalization, which is considered late. In the initial nutrition assessment, the patient was 40 kg weight and 150 cm tall thus the body mass index (BMI) was 17,57 kg/m 2 or underweight. Gastrointestinal symptoms and signs were not found. Information regarding the patient's dietary intake in the last two weeks and weight change within the last 6 months could not be obtained, but his mother claimed that the patient seemed to lose weight because he refused any food that had been offered to him; he only smoked and drank coffee. Albumin level was 3.6 gr/L with imbalanced electrolyte levels. Signs of fluid accumulation were not found. According to the subjective global assessment (SGA) tool, the patient belonged to group C (severely malnourished). Energy and protein were given starting from 30 kcal/kg, increasing gradually to 50 kcal/kg, and 2 gp/kg divided into oral nutrition and oral nutrition supplements (ONS) with a 50:50 ratio. The patient was also given micronutrient supplementations, specifically vitamin A 100,000 units for the first week, vitamin C 100 mg 3 times a day, and zinc 20 mg once a day. During the early weeks of hospitalization, the patient's weight loss continued. At the end of the second week, his body weight was 36 kilograms while his serum albumin level was 2.7 gr/L. On the 28 th day, the patient's body weight was 33 kilograms while his serum albumin level was 2.1 gr/L. Energy and protein intake was maintained, but the ratio of oral intake and ONS was modified to 70:30. During the treatment, the patient's appetite was unstable. The patient was also hardly cooperative with enteral access. The patient often writhed in pain and became more aggressive. The patient did not respond well to analgesics and sedations. Partially parenteral nutrition (PN) enriched with branched-chain amino acid (BCAA) was administered to support protein needs. However, his BMI continued to decline, and on the 42 nd day, his body weight dropped to 32 kilograms with a serum albumin level of 2 gr/L. On the 66 th day, granulation tissues appeared on the wound bed. Considering the mental and nutrition status of the patient, it was decided not to continue with the skin graft surgery. The patient had lost 24% of body weight (BMI 11.96 kg/m 2) and the serum albumin level was 2.5 gr/L. The patient displayed severe muscle wasting and subcutaneous fat loss. However, the general and mental condition was relatively better. The patient was discharged from the hospital.

EN100658

The findings were: left ovary containing a gestational sac and the corpus luteum, normal right adnexae, normal left tube and normal uterus.

It is the case of a 29 years old woman, G2P1001, married, at 9 weeks of pregnancy. She consulted at our emergency service for the exacerbation of a left pelvic pain that has been evolving for two weeks prior to admission. A transient lull was observed following a treatment with a progesteron (hydroxyprogesterone caproate) and an antispasmodic (tiemonium methylsulfate) in a community clinic. The resurgence of that lancinating and permanent pain irradiating to the loins and associated with slight vaginal bleeding prompted consultation. She had first menses at 14 and her menstrual cycle is regular with a length of 30 days. She has never practiced contraception. She has been adequately treated for acute pelvic inflammatory disease five years earlier. Her only child is a girl born vaginally seven years ago. Her blood group is B rhesus positive and she has never undergone surgery. She presented with sympathetic signs of pregnancy and the urinary pregnancy test was positive but echography was not yet done. On admission, besides the main complain, the patient had vaginal bleeding and nausea but neither fever, nor vomiting. On physical examination her general condition was good and the blood pressure was 110/70 millimeters of mercury, the respiratory rate: 20 cycles / minute; the pulse rate: 70 pulsations/minute and the temperature: 37.3 degree Celsius. The conjunctivae were pink and the tongue was clean and moist. There were no cervical adenopathies. The breast and cardiopulmonary examination revealed no abnormalities. The abdomen was flat, and mobile with respiration. On palpation there was just a tenderness of the left iliac fossa and the bowel sounds were normal on auscultation. Inspection under speculum revealed normal gravid external cervical os and there was no vaginal discharge. On digital exploration, the cervix was posterior long and closed, the uterus was globular, increased in size and compatible with an eight weeks pregnancy. The left adnexae presented with a tender, smooth and mobile mass of seven centimeters diameter but the right one were normal. The posterior cervico-vaginal fornix was neither tender nor bulging. We suspected a non ruptured extra-uterine pregnancy with the following differentials: heterotopic pregnancy, torsion of ovarian cyst in pregnancy, and intra-cystic ovarian bleeding in pregnancy. Paraclinical investigations revealed: beta human Chorionic Gonadotropin (β hCG) level of 96 702 milli International Units per milliliter of plasma (mIU/ml). Ultrasonography revealed a heterogenous left ovarian mass of 82 millimeters in diameter, an empty uterus and no peritoneal effusion. This comforted our first diagnosis. After a normal pre-operative work up, an emergency laparotomy was done under general anesthesia. The findings were: left ovary containing a gestational sac and the corpus luteum, normal right adnexae, normal left tube and normal uterus. There was no hemoperitoneum. We dissected the ovarian capsule and carried out ablation of the gestational sac, then we did hemostasis. The specimen was analysed by the pathologist who found decidual cells and trophoblastic tissue within the ovarian capsule and thus confirmed the ovarian pregnancy. Post operative course was uneventful and the patient was discharged six days after surgery. The ?hCG level decreased and disappeared 25 days after surgery.

EN100068

A consultation letter had been received from her neurologist explaining the patient medical condition which starts with difficulty in breathing, chewing and swallowing, there was no external ocular weakness, motor weakness, sphincter dysfunction, sensory deficits, muscle wasting and no signs of diplopia, dizziness, double vision, ptosis or any difficulty in maintaining balance.

A 28-year-old female patient presented to outpatient dental clinics for full mouth rehabilitation. On physical examination, the patient was awake, alert, oriented, and her blood pressure, temperature and heart rate were within normal limits. Upon taking medical history the patient revealed that was diagnosed with thymoma MG and on medication including pyridostigmine bromide 60 mg, azathioprine 50 mg and prednisone 20 mg. Intraoral examination showed fair oral hygiene multiple fixed prosthodontic restorations and multiple carious teeth, multiple remaining roots and lipomatous tongue atrophy. The patient was referred to her neurologist to provide a detailed history about her medical condition and to coordinate with him regarding the steroid dose take to avoid any complication during patient management. A consultation letter had been received from her neurologist explaining the patient medical condition which starts with difficulty in breathing, chewing and swallowing, there was no external ocular weakness, motor weakness, sphincter dysfunction, sensory deficits, muscle wasting and no signs of diplopia, dizziness, double vision, ptosis or any difficulty in maintaining balance. Patient underwent chest CT scan with contrast which show evidence of thymoma benign tumor of the thymus that has been the possible causes of MG. Diagnosis was confirmed with measuring serum level of acetylcholine receptor antibodies, later treatment plan had been formulated including drugs to control the condition and then thymectomy to avoid sever complication to the patient. Before proceeding to the dental management of the case; CBC and blood chemistry had been requested form the patient. Creatinine level was below normal (42.23 umol/l), with normal levels of albumin, urea, uric acid, AST, ALT, cholesterol, triglycerides and bilirubin. Patient had elevated WBC “12.52 x 10 9 /L” and Platelet “453 x 10 9 /L” with reduction in RBC “4.4 x 10 9 /L” and Eosinophil count “0.07”. Consultation with patient neurologist regarding the steroid dose to reduce the susceptibility of developing adrenal crisis.

EN100437

The patient had an intense and regular rehabilitation program.

A 20-year-old right-handed female factory worker with no medical history is consulting in the emergency ward with an open trauma of the left hand resulting from an occupational accident. Her hand was crushed under a heavy object. Physical examination found an important swelling and obvious distortion at the dorsum of the hand associated with wounds of the ulnar and radial edges of the left hand. The neurovascular examination was normal; in particular no sensitive deficit in the median nerve area was noted. Plain radiographs with front and lateral views showed a dislocation of the three lesser fingers associated with fractures of the hamatum and the second metacarpal. The three lesser metacarpals and the distal fragment of the hamatum were medially displaced. The patient was immediately brought to the operating theatre. The treatment consisted of a reduction and stabilisation using a multiple carpo-metacarpal and cross inter-metacarpal pinning. The wound was cleaned up trimmed and sutured. The had and the wrist were immobilized with a splint for six weeks. The patient had an intense and regular rehabilitation program. At one year follow-up, the outcome is good: the patient is painless with good bone consolidation in right position, strictly normal range of motion (metacarpophalangeal 90°, proximal interphalagienne 100° and distal interphalageal 90°) and a 80% grip strength compared to the right side.

EN100345

Laboratory studies were remarkable for a HS-troponin level 181ng/L, a white blood cell count of 14270/mm³ with 48% lymphocytes, and elevated C-reactive protein at 90mg/L.

A 19-year-old healthy man was admitted to our cardiology department following a syncopal episode. His past medical history was unremarkable. He no family history of heart disease or sudden death. Two-days prior the syncope, he developed chest pain and an influenza-like illness consisting of fevers, rhinorrhea, and sore throat. At the physical exam, the patient was febrile at 39°C. He had an inflamed throat, bradycardia at cardiac auscultation and there were no signs of heart failure. The electrocardiogram (EKG) showed grade III atrioventricular (AV) bloc with a ventricular rate of 38 beats per minute. Laboratory studies were remarkable for a HS-troponin level 181ng/L, a white blood cell count of 14270/mm³ with 48% lymphocytes, and elevated C-reactive protein at 90mg/L. Serum electrolytes, kidney and liver functions were within normal limits. A chest X-ray showed an appropriate cardiac size. The initial transthoracic echocardiography (TTE) showed a normal global left ventricular function (left ventricular ejection fraction (LVEF) 60%) and wall motion abnormalities (anteroseptal hypokinesia), with reduced left ventricular global longitudinal strain (LV GLS= -14.1%). Cardiac magnetic resonance (CMR) showed increased signal intensity at the mid-lateral wall on T2-weighted images. Late enhancement revealed a patchy subepicardial hyperenhancement of the medio-distal segments of the anterior and anterolateral walls which confirm the diagnosis of myocarditis. The patient was treated with cefotaxime and doxycycline. All serological tests (chlamydia, coxiella, mycoplasma, rickettsia, lyme and bartonella) obtained during the hospitalization were negative. Five days later, the patient was asymptomatic. The 24 hours ECG monitoring showed a sinus rhythm. The TTE revealed a normal LVEF and an improvement in the LVGLS with segmental reduced values concordant with the CMR abnormalities. At one-month follow-up the patient made a full recovery without any recurring cardiovascular symptoms or abnormal electrocardiographic or echocardiographic findings.

EN100682

There were no cervical adenopathies.

It is the case of a 29 years old woman, G2P1001, married, at 9 weeks of pregnancy. She consulted at our emergency service for the exacerbation of a left pelvic pain that has been evolving for two weeks prior to admission. A transient lull was observed following a treatment with a progesteron (hydroxyprogesterone caproate) and an antispasmodic (tiemonium methylsulfate) in a community clinic. The resurgence of that lancinating and permanent pain irradiating to the loins and associated with slight vaginal bleeding prompted consultation. She had first menses at 14 and her menstrual cycle is regular with a length of 30 days. She has never practiced contraception. She has been adequately treated for acute pelvic inflammatory disease five years earlier. Her only child is a girl born vaginally seven years ago. Her blood group is B rhesus positive and she has never undergone surgery. She presented with sympathetic signs of pregnancy and the urinary pregnancy test was positive but echography was not yet done. On admission, besides the main complain, the patient had vaginal bleeding and nausea but neither fever, nor vomiting. On physical examination her general condition was good and the blood pressure was 110/70 millimeters of mercury, the respiratory rate: 20 cycles / minute; the pulse rate: 70 pulsations/minute and the temperature: 37.3 degree Celsius. The conjunctivae were pink and the tongue was clean and moist. There were no cervical adenopathies. The breast and cardiopulmonary examination revealed no abnormalities. The abdomen was flat, and mobile with respiration. On palpation there was just a tenderness of the left iliac fossa and the bowel sounds were normal on auscultation. Inspection under speculum revealed normal gravid external cervical os and there was no vaginal discharge. On digital exploration, the cervix was posterior long and closed, the uterus was globular, increased in size and compatible with an eight weeks pregnancy. The left adnexae presented with a tender, smooth and mobile mass of seven centimeters diameter but the right one were normal. The posterior cervico-vaginal fornix was neither tender nor bulging. We suspected a non ruptured extra-uterine pregnancy with the following differentials: heterotopic pregnancy, torsion of ovarian cyst in pregnancy, and intra-cystic ovarian bleeding in pregnancy. Paraclinical investigations revealed: beta human Chorionic Gonadotropin (β hCG) level of 96 702 milli International Units per milliliter of plasma (mIU/ml). Ultrasonography revealed a heterogenous left ovarian mass of 82 millimeters in diameter, an empty uterus and no peritoneal effusion. This comforted our first diagnosis. After a normal pre-operative work up, an emergency laparotomy was done under general anesthesia. The findings were: left ovary containing a gestational sac and the corpus luteum, normal right adnexae, normal left tube and normal uterus. There was no hemoperitoneum. We dissected the ovarian capsule and carried out ablation of the gestational sac, then we did hemostasis. The specimen was analysed by the pathologist who found decidual cells and trophoblastic tissue within the ovarian capsule and thus confirmed the ovarian pregnancy. Post operative course was uneventful and the patient was discharged six days after surgery. The ?hCG level decreased and disappeared 25 days after surgery.

EN100068

A second biopsy was performed and the microscopic features again showed a rich inflammatory infiltrate with mononuclear cells and histiocytic cells, characterized by pale histiocytes with lobed nuclei, resembling a bean.

A male, 63 years old, complaining about a "wound in the mouth" for 6 months, without any pain or spontaneous bleeding. His medical history was free of disease. The patient was a smoker for 33 years. Intraoral examination revealed a destructive ulcerative lesion around the upper left first and second molars that resembled an oral squamous cell carcinoma. Biopsy of the ulcerative lesion was performed and the microscopic features showed an inflammatory infiltrate rich in plasma cells. Based on this microscopical finding, the final diagnosis was periodontal disease associated with a proliferative non-neoplastic lesion. The patient was referred to a specialized dental surgeon and underwent periodontal therapy including surgical procedures. After that, according to follow-up with the patient, there were no signs of disease remission. The lesion increased in size, although the patient did not complain of any symptoms. A second biopsy was performed and the microscopic features again showed a rich inflammatory infiltrate with mononuclear cells and histiocytic cells, characterized by pale histiocytes with lobed nuclei, resembling a bean. A varying number of eosinophils also were observed, without any evidence of atypical cells present in this infiltrate. An immunohistochemical staining panel was done to determine the nature of this inflammatory infiltrate by using antibodies S-100, CD1a, CD-68 and CD45RO that were positive. These immunohistochemical findings were fundamental for the final diagnosis of LCH. The treatment included surgical extraction of all superior teeth, radiation and systemic corticoid therapies. After 8 years of treatment, the patient is free of disease.

EN105114

Abdominal Ultrasound showed inflamed internal and external oblique muscles of the anterior abdominal wall.

A 13-year-old boy diagnosed with WHO diagnostic criteria of probable NS was referred from Atanga HC III in Pader district where he was enrolled and undergoing care at the nodding syndrome treatment center; he came with a history of progressive swelling and pain in the right lumbar region. The swelling was associated with a high grade fever which was constant and only partially relieved by analgesics. These symptoms were not associated with vomiting, constipation, yellow eyes, loss of appetite or weight loss. The patient reported a history of falling from a tree during one of the nodding episodes in October 2012 and hit his abdomen onto a tree branch. On further probe on his childhood history, his mother reported that he was born normally at home by a Traditional Birth Attendant (TBA) in one of the Internally Displaced peoples (IDP) camps in 2000. She reported that there was an uneventful pregnancy which was carried to term and delivery by Spontaneous vaginal delivery (SVD). She reported that during her pregnancy, she had exclusive feeding on the relief food provided by WFP (beans, yellow posho and cooking oil) during the IDP camps and denies history of ingestion of herbs or medications which caused adverse events during and after the pregnancy. She reported that her child had a normal physical, cognitive and social childhood development before the onset of nodding which began in May 27th 2011 immediately after returning home from IDP camps. The child was enrolled in Atanga treatment centre and was being managed with Carbamazepine, multivitamins and Ivermectin. She reported that in spite of these medications the child continued to have seizures and nodding at least twice a day and had since dropped out of school. On general examination, he was dehydrated, febrile and moderately wasted. There was a right lumbar region mass, tender, indurated and non-fluctuant. The spleen and liver were not palpable. There was no renal or supra-pubic tenderness. The rectum was full of faecal material which was of normal colour and consistency. The anal tone was normal and there was no blood on examining fingure. Haematological investigations were conducted and showed neutrophilia, lympocytosis, monocytosis, and eosinophilia. There were immature granulocytes and atypical lymphocytes seen on the peripheral film report. Other laboratory results including liver function tests (ALT, AST) were elevated while serum protein levels were low; renal functions tests (serum creatinine, blood urea and nitrogen level), and serum electrolytes (K+, Na+, Cl-, HC03-) which were within normal ranges. Abdominal Ultrasound showed inflamed internal and external oblique muscles of the anterior abdominal wall. The patient underwent incision and drainage at Gulu Hospital and wound left open for 14 days and thereafter secondary wound closure was conducted. He received supplementary food rehabilitation and his seizure medication was changed to Sodium Valproate 200mg once a day under direct observation therapy (DOTS) and close monitoring of the vital signs. The patient continued to have regular follow up in the surgical ward; seizures and nodding stopped completely from the time of intervention in the hospital. With these interventions for over one month the child had no seizures nor nodding and the child returned to normal life. A subsequent review of the haematological and clinical chemistry findings 2 weeks later showed that renal function tests, serum electrolytes were normal except the liver enzymes level were elevated and were still high.

EN100075

The postoperative course was simple.

Mrs B.S. 28 years old multiparous that we had received 02 hours after a vaginal delivery of a newborn alive weighing 2750 grams in a peripheral maternity 3 km away. It was referred for postpartum hemorrhage. His medical history was featureless. On admission, she presented hypovolemic shock with blood pressure 76/44 mmHg, regular tachycardia at 136 beats per minute, polypnea to 39 cycles per minute. The obstetrical examination objectified an involuted uterus, an absence of vaginal and perineal cervical lesions, a fluid and red blood originating of endouterine. Furthermore, there was purpura. We therefore concluded a significant postpartum hemorrhage complicated by coagulopathy. Resuscitation measures were taken and an transfusion 1120 ml whole blood, red cell pellets 350 ml and 860 ml fresh frozen plasma have helped to management coagulopathy and anemia. Blood count at admission highlighted severe thrombocytopenia 23 0000 platelets and hemoglobin levels of 3.9 g/dl. The post natal care was also administered. After seven days of hospitalization, she was going after an amendment to the clinical symptoms and normal laboratory tests. Twenty five days later, she returned to obstetric emergencies for significant bleeding with severe anemia with hemoglobin 9 g/dL without signs of hypovolemic shock. The gynecological examination was normal. The balance sheet of the coagulation was normal including prothrombin to 92% and a normal activated partial thromboplastin time. The treatments were based on oxytocin, tranexamic acid, etamsylate and strict regular monitoring. Transvaginal ultrasound coupled Doppler was normal. In the absence of arterial embolization, she was going with ambulatory monitoring. Two weeks later she presented again rebleeding requiring hospitalization resulting in a total hysterectomy after conditioning of the patient. Histological examination of the surgical specimen objectified in the cervix and uterine body the presence of arterial vessels proliferation homes large and small caliber associated with tissue hemorrhage. The postoperative course was simple.

EN100340

A male, 63 years old, complaining about a "wound in the mouth" for 6 months, without any pain or spontaneous bleeding.

A male, 63 years old, complaining about a "wound in the mouth" for 6 months, without any pain or spontaneous bleeding. His medical history was free of disease. The patient was a smoker for 33 years. Intraoral examination revealed a destructive ulcerative lesion around the upper left first and second molars that resembled an oral squamous cell carcinoma. Biopsy of the ulcerative lesion was performed and the microscopic features showed an inflammatory infiltrate rich in plasma cells. Based on this microscopical finding, the final diagnosis was periodontal disease associated with a proliferative non-neoplastic lesion. The patient was referred to a specialized dental surgeon and underwent periodontal therapy including surgical procedures. After that, according to follow-up with the patient, there were no signs of disease remission. The lesion increased in size, although the patient did not complain of any symptoms. A second biopsy was performed and the microscopic features again showed a rich inflammatory infiltrate with mononuclear cells and histiocytic cells, characterized by pale histiocytes with lobed nuclei, resembling a bean. A varying number of eosinophils also were observed, without any evidence of atypical cells present in this infiltrate. An immunohistochemical staining panel was done to determine the nature of this inflammatory infiltrate by using antibodies S-100, CD1a, CD-68 and CD45RO that were positive. These immunohistochemical findings were fundamental for the final diagnosis of LCH. The treatment included surgical extraction of all superior teeth, radiation and systemic corticoid therapies. After 8 years of treatment, the patient is free of disease.

EN105114

Platelet count, activated partial thromboplastin time, liver function tests, blood urea nitrogen, serum creatinine, erythrocyte sediment rate and total cholesterol were within normal limits.

A 77-year-old Tunisian woman was hospitalized because of massive painful ascites. Her family history did not include any autoimmune disease. She denied a history of hepatitis, jaundice or alcohol use. She had a history of diabetes, hypertension treated by glinide and calcium blocker. She was explored 4 years prior to admission for exudative pleuritis of the right lung without any established diagnosis after multiple explorations including thoracoscopy with biopsies. On admission, blood pressure was 150/70 mmHg, her rate was 80 /mn and body temperature was 37°C. The physical examination showed only abdominal distension related to the important ascites without collateral venous circulation. She had no skin lesions, lymphadenopathy, or hepatosplenomegaly and lower extremities showed no edema. Laboratory investigations showed: leucopenia with white blood cell count = 3100/mm3, lymphopenia = 840/mm3, hemoglobin = 10.5g/dL ; low serum albumin level = 29g/L, 47;-globulin = 19g/L. blood glucose levels = 180 mg/dl and hemoglobin A1c = 8.6 %. Urine analysis showed trace protein (0.03g/24h). Platelet count, activated partial thromboplastin time, liver function tests, blood urea nitrogen, serum creatinine, erythrocyte sediment rate and total cholesterol were within normal limits. Tests for HBsAg and HCV were negative. Abdominal ultrasonography showed ascitic fluid without any sign of bowel loop thickening or enteritis. Neither lymphadenopathy nor liver abnormality was present. A chest X-ray film disclosed bilateral pleural effusion. Ascitic obtained by aspiration contained 170 cells mm3 (67% lymphocytes), 46g/L protein, but no malignant cells. Cultures for bacteria and mycobacteria gave no growth. Tuberculosis investigation including initial tuberculin skin test and the research of Koch bacilli in sputum and urine were negative. Echocardiogram and electrocardiogram were normal. Computed tomographic scan showed massive ascites, no dysmorphic liver, and a mild bilateral pleural effusion. Pelvic examination was normal. Oesogastroduodenoscopy showed hiatal hernia without oesophageal varices. An exploratory laparoscopy was performed, revealing a large amount of ascite, a normal liver appearance, and no granulations suggesting tuberculosis or peritoneal carcinosis. Histological examination of the peritoneum showed non specific chronic inflammation. Systemic lupus erythematosus was suspected based on pleuritis, lymphopenia and leucopenia. An additional serologic survey revealed markedly elevated anti-nuclear antibody (ANA) titer of 1/1600 and a significantly elevated titer of antibody to double-stranded DNA (83 IU/mL ; normal < 30 IU/mL). She had also positive serum antibody against the Smith antigen and low serum level of C3 complement component: 67 mg/dL (serum normal range: 84 – 151). Relying on these findings, the patient was diagnosed with SLE since 4 of the 11 diagnostic criteria of the American College of Rheumatology were met. The SLEDAI score according to Systemic Lupus Erythematosus Disease Activity Index was estimated to be 7 on admission. Hydroxychloroquine 200 mg daily in combination with diuretics was initiated. One month later, there was no detectable ascitic fluid and no pleural effusions. Five months later she remained free from symptoms while continuing to take Hydroxychloroquine

EN100114

Histological study concluded in multinodular colloid goiter.

A 50-years-old woman, hypertensive, hospitalized for a large cervical mass appeared 30 years ago. In the family history, her mother, sisters and cousins underwent a surgery for MNG. Despite of the large volume of the mass, the patient never described signs of cervical compression whatsoever respiratory, digestive, laryngeal, vascular or neurologic signs. She never suffered from thyroid dysfunction. Her neck was deformed by the voluminous formation classified grade III according to the WHO modified classification. The mass took the front and the two sides of the neck. Its surface was embossed and covered by a thin normal skin. There were some veins of the collateral circulation limited to the neck. The goiter measured 18 x 11 cm. The mass was firm, painless, and mobile with the swallowing movements. Lymphadenopathy research was difficult and found no palpable lymph nodes. The laboratory tests (T 3, T 4 and TSH) were normal. Thoracic radiography showed a large cervical opacity roughly round and strewn with microcalcifications associated with a right eccentricity of the trachea. Cervical and chest CT revealed the presence of a partially calcified thyroid mass slightly plunging in the anterior mediastinum. It took heterogeneously the contrast and then evocate a large MNG. The trachea was surrounded by the goiter, slightly narrowed and right deviated as well as the lower part of the larynx. The right and left vascular axes of the neck (carotid artery and jugular vein) were deviated backward. The patient underwent a surgery for her enormous MNG slightly plunging in the mediastinum. Endotracheal intubation was relatively easy by the laryngoscope. The incision performed was a Kocher cervicotomy. There was a multinodular, hypervascularized goiter. Its lower end plunges behind the sternal manubrium. The larynx was deviated towards the right side. The total thyroidectomy was performed in two steps: initially a right lobo-isthmectomy, then the left lobectomy. The retrosternal part of the goiter was released using the finger by the same incision. Both recurrent laryngeal nerves (RLN) were not identified because of the hemorrhage. One parathyroid gland was accidently devascularized and was autotransplanted to the ipsilateral sternocleidomastoid muscle. The operation was finished by double aspiration drainage. In the first hours after surgery, the patient developed a large cervical hematoma. She was readmitted to the operating room, and after evacuation of the hematoma there was no vessels bleeding. The operation was completed with a double suction drainage. In the immediate postoperative period, the patient developed hemodynamic collapse requiring the introduction of dobutamine. After 48 hours of hemodynamic support, the blood pressure stabilized and dobutamine was stopped. Histological study concluded in multinodular colloid goiter. The patient was discharged from the hospital after 20 days in good health.

EN100067

Two weeks later she presented again rebleeding requiring hospitalization resulting in a total hysterectomy after conditioning of the patient.

Mrs B.S. 28 years old multiparous that we had received 02 hours after a vaginal delivery of a newborn alive weighing 2750 grams in a peripheral maternity 3 km away. It was referred for postpartum hemorrhage. His medical history was featureless. On admission, she presented hypovolemic shock with blood pressure 76/44 mmHg, regular tachycardia at 136 beats per minute, polypnea to 39 cycles per minute. The obstetrical examination objectified an involuted uterus, an absence of vaginal and perineal cervical lesions, a fluid and red blood originating of endouterine. Furthermore, there was purpura. We therefore concluded a significant postpartum hemorrhage complicated by coagulopathy. Resuscitation measures were taken and an transfusion 1120 ml whole blood, red cell pellets 350 ml and 860 ml fresh frozen plasma have helped to management coagulopathy and anemia. Blood count at admission highlighted severe thrombocytopenia 23 0000 platelets and hemoglobin levels of 3.9 g/dl. The post natal care was also administered. After seven days of hospitalization, she was going after an amendment to the clinical symptoms and normal laboratory tests. Twenty five days later, she returned to obstetric emergencies for significant bleeding with severe anemia with hemoglobin 9 g/dL without signs of hypovolemic shock. The gynecological examination was normal. The balance sheet of the coagulation was normal including prothrombin to 92% and a normal activated partial thromboplastin time. The treatments were based on oxytocin, tranexamic acid, etamsylate and strict regular monitoring. Transvaginal ultrasound coupled Doppler was normal. In the absence of arterial embolization, she was going with ambulatory monitoring. Two weeks later she presented again rebleeding requiring hospitalization resulting in a total hysterectomy after conditioning of the patient. Histological examination of the surgical specimen objectified in the cervix and uterine body the presence of arterial vessels proliferation homes large and small caliber associated with tissue hemorrhage. The postoperative course was simple.

EN100340

On the other side there was no history of jaundice, vomiting, urinary or bowel complaints, bleeding from any site or neurological complaints.

We present here a case of Macrophage activation syndrome associated with Griscelli syndrome type 2 in a 3-years-old male born of consanguineous parentage. His birth histories, his familie social history and developmental milestones were unremarkable. He was born at full term with no antenatal or perinatal complications, He was on exclusively breast-fed, food diversification was started at 6 months old, his weight, length and psychomotor development were within the normal range, the child was described as a good eater, was on a normal diet and was thriving appropriately. Furthermore the boy presented a progressive abdominal distension since birth, progressive pallor and recurrent episodes of fever since 1 year of age. There was history of blood transfusions for last 2 months. On the other side there was no history of jaundice, vomiting, urinary or bowel complaints, bleeding from any site or neurological complaints. On admission, he was very pale with silvery gray scalp hair, white eyelashes, he was hypotonic, tachycardic, fever of 40°C, lymphoadenopathy and hepato-splenomegaly. The blood count showed pancytopenia; 1980 white blood cells/µL (VN 4000-13 500/µL), neutrophils 820/µL, 910 cells/µL, hemoglobin (Hb) 6.3 g/dL (11.5-14 VN, 5 g/dL), platelets 44 000/µL (VN 150 000-400 000/µL), There were no giant cytoplasmic granules in leucocytes. The liver function tests were normal expect low albumin (2.1 mg/dL) and increased alkaline phosphatase (1196 mg/dL) with low fibrinogen (1.7g/l). Serum triglycerides, ferrintin and lactic dehydrogenase were very high; respectively 4.75g/L; 2763µg/L and 597U/L. the C reactive protein (CRP) was elevated at 83mg/l. A chest X-ray was normal while the abdominal ultrasound showed a hepatomegaly and a splenomegaly, free biliary ducts without ascites. Because of hepato-splenomegaly, pancytopenia, hyperferritinemia and hypofibrinogenemia the diagnosis of Macrophage activation syndrome was evoked indicating a bone marrow aspiration that showed numerous morphologically benign macrophages with haemophagocytic activity that comforting the diagnosis of a SAM according to Ravelli and HLH-2004 criteria. Blood culture, urine examination, malarial serology, Kala Azar serology, viral serologies such as EBV, hepatitis A, B, C and human immunodeficiency virus HIV, serologies of rickettsia, syphilis and toxoplasmosa were negatives. Serologies of CMV and rubella showed an old immunization. The immunological tests such as antinuclear antibodies (ANA), anti-LKM1, anti-mitochondria and anti-smooth muscle antibodies were negatives. Because of the consanguineous family, notion of recurrent infection and the presence of silvery-gray color of the hair and eyebrows, diagnosis of Griscelli Syndrome was evoked directing a light microscopy examination of the hair that showed a large, irregular clumps of pigments characteristic of Griscelli Syndrome. The molecular biology showed mutation in RAB27A gene confirming the diagnosis of a Griscelli syndrome type 2. The first-line therapy in management of Macrophage activation syndrome complicating Griscelli syndrome type 2 was based on the parenteral administration of high doses of corticosteroids (methylprednisolone pulses 1g/0.73m 2 /day for 3 days then 60mg/m 2) with poor response; persisting with involvement of the 3 cell lines in the blood count, elevated ferritin and triglycerides. Given the lack of response to steroids, Etoposide was started for 3 days associated with cyclophosphamide, the evolution was good with improvement of count blood cells, ferritin was lessening while the bone marrow showed decreased numerous of macrophages with low haemophagocytic activity. On the other side the infectious process was good managed by perfusion of Antibiotics such as Ceftazidim in combination with aminoside. The patient was sent home with prednisone and cyclosporine, He was placed on the list of bone marrow transplantation (BMT).

EN100372

The operation was finished by double aspiration drainage.

A 50-years-old woman, hypertensive, hospitalized for a large cervical mass appeared 30 years ago. In the family history, her mother, sisters and cousins underwent a surgery for MNG. Despite of the large volume of the mass, the patient never described signs of cervical compression whatsoever respiratory, digestive, laryngeal, vascular or neurologic signs. She never suffered from thyroid dysfunction. Her neck was deformed by the voluminous formation classified grade III according to the WHO modified classification. The mass took the front and the two sides of the neck. Its surface was embossed and covered by a thin normal skin. There were some veins of the collateral circulation limited to the neck. The goiter measured 18 x 11 cm. The mass was firm, painless, and mobile with the swallowing movements. Lymphadenopathy research was difficult and found no palpable lymph nodes. The laboratory tests (T 3, T 4 and TSH) were normal. Thoracic radiography showed a large cervical opacity roughly round and strewn with microcalcifications associated with a right eccentricity of the trachea. Cervical and chest CT revealed the presence of a partially calcified thyroid mass slightly plunging in the anterior mediastinum. It took heterogeneously the contrast and then evocate a large MNG. The trachea was surrounded by the goiter, slightly narrowed and right deviated as well as the lower part of the larynx. The right and left vascular axes of the neck (carotid artery and jugular vein) were deviated backward. The patient underwent a surgery for her enormous MNG slightly plunging in the mediastinum. Endotracheal intubation was relatively easy by the laryngoscope. The incision performed was a Kocher cervicotomy. There was a multinodular, hypervascularized goiter. Its lower end plunges behind the sternal manubrium. The larynx was deviated towards the right side. The total thyroidectomy was performed in two steps: initially a right lobo-isthmectomy, then the left lobectomy. The retrosternal part of the goiter was released using the finger by the same incision. Both recurrent laryngeal nerves (RLN) were not identified because of the hemorrhage. One parathyroid gland was accidently devascularized and was autotransplanted to the ipsilateral sternocleidomastoid muscle. The operation was finished by double aspiration drainage. In the first hours after surgery, the patient developed a large cervical hematoma. She was readmitted to the operating room, and after evacuation of the hematoma there was no vessels bleeding. The operation was completed with a double suction drainage. In the immediate postoperative period, the patient developed hemodynamic collapse requiring the introduction of dobutamine. After 48 hours of hemodynamic support, the blood pressure stabilized and dobutamine was stopped. Histological study concluded in multinodular colloid goiter. The patient was discharged from the hospital after 20 days in good health.

EN100067

Mr F.B a 70-year-old male patient presented in our institution because of the occurrence of a right epistaxis.

Mr F.B a 70-year-old male patient presented in our institution because of the occurrence of a right epistaxis. First of all, the patient is not smoking and reported an aneurysm of the abdominal aorta surgically treated by prosthesis under plavix. The beginning of the symptomatology goes back to 2 months before with right nasal obstruction of progressive onset, associated with right epistaxis and bloody rhinorrhea with right chronic eye watering. Nasal Endoscopy examination revealed a pinnkish burgeoning mass bleeding on contact and filling the right nostril vestibule. The patient didn't presents neurological signs nor cervical lymph nods. Otherwise, ophthalmologic examination found normal visual acuity and ocular mobility on both sides. Sinonasal computed tomography (CT) found a right nasal process enhanced after injection of contrast product with relatively little washing at the late time. Otherwise, there is no extension involvement at the level of the cavum and the pterygo palate fossa. This finding found also a crenate appearance of the posterior nasal mucosa especially of the middle turbinates and contralateral inferior turbinate with a left deviation of the nasal septum with nasal spur. Nasosinusal MRI shows a progressive increase in size of the tissue mass compared to the previously CT occupying the right nasal fossa on almost all of its height, measuring 4 × 2.8 × 3.5cm without extension within the cavum with probable invasion of the lower part of the nasolacrimal duct and mass effect on both the sinusonasal bone septum and the intersinusonasal septum. This finding shows ethmoidal sinus fluid retention. The appearance, even if it remains unspecific, is compatible with inverted papilloma. The patient underwent complete endoscopic removal of the tumor with ethmoidectomy and right maxillary antrostomy. The extemporaneous examination is in favor of a malignant tumor lesion. Histopathologic analysis shows a tumor proliferation made of polymorphic cells with major anisocytosis, most often located in lobules. There is sometimes a somewhat palisadic seat on the periphery of the tumor mass. The mitotic index is 10 mitoses per 10 high power fields. The immunohistochemical study of the tumor found that the cytokeratin KL1 and CK7 and the neuroendocrine marker Chromogranin A, synaptophysin and CD56 are positive. Proliferation index Ki67 is of the order of 80%. Otherwise there is an absence of expression of PS100, MelanA, HMB45, desmin, p40, CD45 and EBV. The clear positivity of cytokeratins formally excludes the diagnosis of olfactory neuroblastoma and leads to neuroendocrine carcinoma. Given the atypia, the mitotic index, and the Ki67 proliferation index, it is a high-grade large-cell neuroendocrine carcinoma. Extension assessment, comprising a PET SCAN, was negative. Postoperative treatment consisted in adjuvant polychemotherapy, with six cycles (cisplatin and etoposide), Followed by loco regional external radiotherapy. After 6 month follow-up, the patient was in complete clinical and radiological remission.

EN100383

We noticed that the hernia orifice was in a higher than normal position 5 cm from the pubic bone.

Male infant 9.5 month old was examined in the emergency department of our hospital because of an empty scrotum and the ipsilateral testis lying in a high iliac crest position. With the increase in intrabdominal pressure [crying etc.] there was an ever-increasing swelling with spiral course from the groin to ipsilateral iliac crest. On clinical examination, a reducible mass was present in the right ileac crest. The swelling looked like a spigelian hernia [4]. However no abdominal wall defect could be palpated at that area. Moreover the ipsilateral scrotum was empty and the testis could be palpated with difficulty lying in a high inguinal position. Ultrasonography showed a loop of herniated small bowel and an undescended testis on the right. At operation, under general anesthesia, an extended transverse inguinal incision was made. Upon entering the inguinal canal a large hernia sac was identified. This followed a course through the superficial inguinal ring, extended upwards to the anterior abdomninal wall below the subcutaneous plane and was fixed in the area of the right iliac crest. We noticed that the hernia orifice was in a higher than normal position 5 cm from the pubic bone. This bulk was easily reduced back to the abdomen along with an ipsilateral mobile intravaginal testis. After careful isolation of the sac at a length of 3 cm the ipsilateral testis was isolated. The vas and the vessels were intravaginal, the gubernaculum was fixed in an ectopic position and the dorsal inguinal wall was lax. We removed the large sac after separating the vas and vessels and the testis. Moreover we strengthened the dorsal inguinal wall, fixated the testis in a subdartos pouch and closed the wound. Patient stayed for a day in the hospital. No postoperative complications happened. Three years later the boy was operated for left hydrocele. The boy remains in perfect health now five years after the initial operation.

EN100508

Sinonasal computed tomography (CT) found a right nasal process enhanced after injection of contrast product with relatively little washing at the late time.

Mr F.B a 70-year-old male patient presented in our institution because of the occurrence of a right epistaxis. First of all, the patient is not smoking and reported an aneurysm of the abdominal aorta surgically treated by prosthesis under plavix. The beginning of the symptomatology goes back to 2 months before with right nasal obstruction of progressive onset, associated with right epistaxis and bloody rhinorrhea with right chronic eye watering. Nasal Endoscopy examination revealed a pinnkish burgeoning mass bleeding on contact and filling the right nostril vestibule. The patient didn't presents neurological signs nor cervical lymph nods. Otherwise, ophthalmologic examination found normal visual acuity and ocular mobility on both sides. Sinonasal computed tomography (CT) found a right nasal process enhanced after injection of contrast product with relatively little washing at the late time. Otherwise, there is no extension involvement at the level of the cavum and the pterygo palate fossa. This finding found also a crenate appearance of the posterior nasal mucosa especially of the middle turbinates and contralateral inferior turbinate with a left deviation of the nasal septum with nasal spur. Nasosinusal MRI shows a progressive increase in size of the tissue mass compared to the previously CT occupying the right nasal fossa on almost all of its height, measuring 4 × 2.8 × 3.5cm without extension within the cavum with probable invasion of the lower part of the nasolacrimal duct and mass effect on both the sinusonasal bone septum and the intersinusonasal septum. This finding shows ethmoidal sinus fluid retention. The appearance, even if it remains unspecific, is compatible with inverted papilloma. The patient underwent complete endoscopic removal of the tumor with ethmoidectomy and right maxillary antrostomy. The extemporaneous examination is in favor of a malignant tumor lesion. Histopathologic analysis shows a tumor proliferation made of polymorphic cells with major anisocytosis, most often located in lobules. There is sometimes a somewhat palisadic seat on the periphery of the tumor mass. The mitotic index is 10 mitoses per 10 high power fields. The immunohistochemical study of the tumor found that the cytokeratin KL1 and CK7 and the neuroendocrine marker Chromogranin A, synaptophysin and CD56 are positive. Proliferation index Ki67 is of the order of 80%. Otherwise there is an absence of expression of PS100, MelanA, HMB45, desmin, p40, CD45 and EBV. The clear positivity of cytokeratins formally excludes the diagnosis of olfactory neuroblastoma and leads to neuroendocrine carcinoma. Given the atypia, the mitotic index, and the Ki67 proliferation index, it is a high-grade large-cell neuroendocrine carcinoma. Extension assessment, comprising a PET SCAN, was negative. Postoperative treatment consisted in adjuvant polychemotherapy, with six cycles (cisplatin and etoposide), Followed by loco regional external radiotherapy. After 6 month follow-up, the patient was in complete clinical and radiological remission.

EN100383

An MRI done in the same period shows a cure of the patient by a complete disappearance of the cyst.

A 3 years old girl born from non-consanguineous parents, without any neonatal suffering; with good psychomotor development, from a popular district in one of our cities, without any obvious contact with dogs; presented with exophthalmia associated with unilateral blindness evolving rapidly within 3 months. Clinical examination showed a non-pulsatile, painless, axial, irreducible exophthalmia with no sign of conjunctivitis or keratitis, and right monocular blindness, right ptosis; and fundal examination had objectified right papillary oedema. The rest of the clinical examination was normal. Brain and orbital magnetic resonance imaging (MRI) revealed an extra-connal right orbital lesion near the orbital apex, measuring 28 x 18mm, of oval shape, it appeared hypo-signal in T1 and hyper-signal in T2, limited by a thin wall which took contrast product, this lesion compressed the optic nerve towards the nasal region. Chest x-ray and abdominal ultrasound did not reveal any other localization. The patient was operated by performing a right extra-dural frontal approach, a cyst puncture was done in the first intention because the cyst was adherent to the neighbouring structures making its complete removal impossible, then a microscopic extirpation of the cystic membrane, combined with abundant washing by hypertonic serum to sterilize the cystic sit and reduce chances of dissemination. Histological examination was in favour of intra-orbital hydatidosis. Postoperatively, the patient was given an antihelminthic treatment of albendazole at a dose of 10mg per kilogram per day in 3 doses separated by 2 weeks; the evolution was marked by the significant regression of the exophthalmia and the gradual improvement of the visual acuity. The patient however still retains a right unilateral nasal hemianopia after two years of follow-up. An MRI done in the same period shows a cure of the patient by a complete disappearance of the cyst.

EN100453

She was not a case of von Recklinghausen disease.

The patient was a 35-year old female who presented with urinary frequency for 2 months. Her past medical history showed a nonsmoking history and no significant family history. The physical examination was unremarkable. She was treated as urinary tract infection, but there was no improvement in her symptoms. Urine analysis and culture were performed several times that was negative for infection. Other laboratory data were also insignificant. Through a routine evaluation, urinary system ultrasonography (US) revealed a bladder lesion measuring 7 mm. In order to confirm the diagnosis, magnetic resonance imaging (MRI) of the abdomen and pelvic cavity was done; it revealed a lesion in the dome of the bladder. To confirm the diagnosis and for histopathological examination, the patient underwent Transurethral Resection of Bladder Tumor (TURBT) in April 2018. Before starting the procedure, the urethra was dilated till Fr: 28; at first, the tumor location was specified by cystoscopy. Then, it was resected by monopolar cautery from superficial to deeper parts with removal of the muscle layers. Proper bleeders were taken and three way Foley catheter was applied for a continuous irrigation. The patient was transferred to the recovery room with complete stability. The histopathology slides show a well-defined mass composed of bland looking spindle cells. Subsequent immunohistochemistry (IHC) was done which showed diffuse immunoreactivity for S100, but other markers including smooth muscle actin (SMA), desmin, cytokeratin, CD34 and beta catenin were negative. Ki67 was 2-3%, so the diagnosis of schwannoma was confirmed. Then, the neck, abdomen, pelvic and chest computed tomography (CT) scans were done that were normal. Brain and spine MRI and physical examination showed no evidence of other schwannoma or evidence of neurofibromatosis. She had no history of neurofibromatosis in her family. Skin examination was normal and had no café au lait spot. She was not a case of von Recklinghausen disease.

EN100506

This followed a course through the superficial inguinal ring, extended upwards to the anterior abdomninal wall below the subcutaneous plane and was fixed in the area of the right iliac crest.

Male infant 9.5 month old was examined in the emergency department of our hospital because of an empty scrotum and the ipsilateral testis lying in a high iliac crest position. With the increase in intrabdominal pressure [crying etc.] there was an ever-increasing swelling with spiral course from the groin to ipsilateral iliac crest. On clinical examination, a reducible mass was present in the right ileac crest. The swelling looked like a spigelian hernia [4]. However no abdominal wall defect could be palpated at that area. Moreover the ipsilateral scrotum was empty and the testis could be palpated with difficulty lying in a high inguinal position. Ultrasonography showed a loop of herniated small bowel and an undescended testis on the right. At operation, under general anesthesia, an extended transverse inguinal incision was made. Upon entering the inguinal canal a large hernia sac was identified. This followed a course through the superficial inguinal ring, extended upwards to the anterior abdomninal wall below the subcutaneous plane and was fixed in the area of the right iliac crest. We noticed that the hernia orifice was in a higher than normal position 5 cm from the pubic bone. This bulk was easily reduced back to the abdomen along with an ipsilateral mobile intravaginal testis. After careful isolation of the sac at a length of 3 cm the ipsilateral testis was isolated. The vas and the vessels were intravaginal, the gubernaculum was fixed in an ectopic position and the dorsal inguinal wall was lax. We removed the large sac after separating the vas and vessels and the testis. Moreover we strengthened the dorsal inguinal wall, fixated the testis in a subdartos pouch and closed the wound. Patient stayed for a day in the hospital. No postoperative complications happened. Three years later the boy was operated for left hydrocele. The boy remains in perfect health now five years after the initial operation.

EN100508

She was treated as urinary tract infection, but there was no improvement in her symptoms.

The patient was a 35-year old female who presented with urinary frequency for 2 months. Her past medical history showed a nonsmoking history and no significant family history. The physical examination was unremarkable. She was treated as urinary tract infection, but there was no improvement in her symptoms. Urine analysis and culture were performed several times that was negative for infection. Other laboratory data were also insignificant. Through a routine evaluation, urinary system ultrasonography (US) revealed a bladder lesion measuring 7 mm. In order to confirm the diagnosis, magnetic resonance imaging (MRI) of the abdomen and pelvic cavity was done; it revealed a lesion in the dome of the bladder. To confirm the diagnosis and for histopathological examination, the patient underwent Transurethral Resection of Bladder Tumor (TURBT) in April 2018. Before starting the procedure, the urethra was dilated till Fr: 28; at first, the tumor location was specified by cystoscopy. Then, it was resected by monopolar cautery from superficial to deeper parts with removal of the muscle layers. Proper bleeders were taken and three way Foley catheter was applied for a continuous irrigation. The patient was transferred to the recovery room with complete stability. The histopathology slides show a well-defined mass composed of bland looking spindle cells. Subsequent immunohistochemistry (IHC) was done which showed diffuse immunoreactivity for S100, but other markers including smooth muscle actin (SMA), desmin, cytokeratin, CD34 and beta catenin were negative. Ki67 was 2-3%, so the diagnosis of schwannoma was confirmed. Then, the neck, abdomen, pelvic and chest computed tomography (CT) scans were done that were normal. Brain and spine MRI and physical examination showed no evidence of other schwannoma or evidence of neurofibromatosis. She had no history of neurofibromatosis in her family. Skin examination was normal and had no café au lait spot. She was not a case of von Recklinghausen disease.

EN100506

Haematological investigations showed normal serum calcium, phosphorus and parathormone (PTH) levels.

A 12-year-old girl was referred to Department of Oral Surgery, with a complaint of pain and swelling in the left of maxilla that appeared 3 months ago. The swelling was slowly progressive, associated with pain. However, the patient did not refer any motor or sensory deficit. There was no family history of similar swelling. Physical examination revealed a left maxillary swelling. There was no facial palsy. No cervical lymph node enlargement was seen. The oral examination showed a 4 cm x 5 cm, tender, compressible mass in the left maxilla, from the lateral incisor to the second premolar teeth. The tumor surface was smooth and red-purple. Several teeth were involved and displaced in the tumor mass. We noted the absence of canine. Vitality tests proved negative on the central and lateral incisor. Hight mobility of lateral incisor was noted. Orthopantomography and occlusal radiography showed a wide osteolytic area of the anterior left maxilla, extending from the first permanent molar to the central incisor. The canine was impacted. No root resorption was observed. The radiolucency was closely related to maxillary sinus and involving the nasal cavity. Haematological investigations showed normal serum calcium, phosphorus and parathormone (PTH) levels. The provisional diagnosis of benign tumor of the maxilla was made. The patient underwent excision and curettage of the mass with extraction of the canine and lateral incisor tooth. The wound was closed with interrupted sutures. The post-operative histopathological report revealed multinuclear giant cells scattered randomly throughout the cellular and fibrous vascular-rich tissue. New bone formation and granulation tissue rich in mononuclear inflammatory cells was revealed. The giant cells were multinucleated with bland-appearing nuclei, and the background stromal cells displayed no evidence of atypical mitoses. A diagnosis of giant cell tumor was established. During a 1-year serial clinical and radiological follow-up, there was no evidence of recurrence. The facial contour and masticatory function were well-preserved.

EN100600

The rectum was full of faecal material which was of normal colour and consistency.

A 13-year-old boy diagnosed with WHO diagnostic criteria of probable NS was referred from Atanga HC III in Pader district where he was enrolled and undergoing care at the nodding syndrome treatment center; he came with a history of progressive swelling and pain in the right lumbar region. The swelling was associated with a high grade fever which was constant and only partially relieved by analgesics. These symptoms were not associated with vomiting, constipation, yellow eyes, loss of appetite or weight loss. The patient reported a history of falling from a tree during one of the nodding episodes in October 2012 and hit his abdomen onto a tree branch. On further probe on his childhood history, his mother reported that he was born normally at home by a Traditional Birth Attendant (TBA) in one of the Internally Displaced peoples (IDP) camps in 2000. She reported that there was an uneventful pregnancy which was carried to term and delivery by Spontaneous vaginal delivery (SVD). She reported that during her pregnancy, she had exclusive feeding on the relief food provided by WFP (beans, yellow posho and cooking oil) during the IDP camps and denies history of ingestion of herbs or medications which caused adverse events during and after the pregnancy. She reported that her child had a normal physical, cognitive and social childhood development before the onset of nodding which began in May 27th 2011 immediately after returning home from IDP camps. The child was enrolled in Atanga treatment centre and was being managed with Carbamazepine, multivitamins and Ivermectin. She reported that in spite of these medications the child continued to have seizures and nodding at least twice a day and had since dropped out of school. On general examination, he was dehydrated, febrile and moderately wasted. There was a right lumbar region mass, tender, indurated and non-fluctuant. The spleen and liver were not palpable. There was no renal or supra-pubic tenderness. The rectum was full of faecal material which was of normal colour and consistency. The anal tone was normal and there was no blood on examining fingure. Haematological investigations were conducted and showed neutrophilia, lympocytosis, monocytosis, and eosinophilia. There were immature granulocytes and atypical lymphocytes seen on the peripheral film report. Other laboratory results including liver function tests (ALT, AST) were elevated while serum protein levels were low; renal functions tests (serum creatinine, blood urea and nitrogen level), and serum electrolytes (K+, Na+, Cl-, HC03-) which were within normal ranges. Abdominal Ultrasound showed inflamed internal and external oblique muscles of the anterior abdominal wall. The patient underwent incision and drainage at Gulu Hospital and wound left open for 14 days and thereafter secondary wound closure was conducted. He received supplementary food rehabilitation and his seizure medication was changed to Sodium Valproate 200mg once a day under direct observation therapy (DOTS) and close monitoring of the vital signs. The patient continued to have regular follow up in the surgical ward; seizures and nodding stopped completely from the time of intervention in the hospital. With these interventions for over one month the child had no seizures nor nodding and the child returned to normal life. A subsequent review of the haematological and clinical chemistry findings 2 weeks later showed that renal function tests, serum electrolytes were normal except the liver enzymes level were elevated and were still high.

EN100075

She presented with sympathetic signs of pregnancy and the urinary pregnancy test was positive but echography was not yet done.

It is the case of a 29 years old woman, G2P1001, married, at 9 weeks of pregnancy. She consulted at our emergency service for the exacerbation of a left pelvic pain that has been evolving for two weeks prior to admission. A transient lull was observed following a treatment with a progesteron (hydroxyprogesterone caproate) and an antispasmodic (tiemonium methylsulfate) in a community clinic. The resurgence of that lancinating and permanent pain irradiating to the loins and associated with slight vaginal bleeding prompted consultation. She had first menses at 14 and her menstrual cycle is regular with a length of 30 days. She has never practiced contraception. She has been adequately treated for acute pelvic inflammatory disease five years earlier. Her only child is a girl born vaginally seven years ago. Her blood group is B rhesus positive and she has never undergone surgery. She presented with sympathetic signs of pregnancy and the urinary pregnancy test was positive but echography was not yet done. On admission, besides the main complain, the patient had vaginal bleeding and nausea but neither fever, nor vomiting. On physical examination her general condition was good and the blood pressure was 110/70 millimeters of mercury, the respiratory rate: 20 cycles / minute; the pulse rate: 70 pulsations/minute and the temperature: 37.3 degree Celsius. The conjunctivae were pink and the tongue was clean and moist. There were no cervical adenopathies. The breast and cardiopulmonary examination revealed no abnormalities. The abdomen was flat, and mobile with respiration. On palpation there was just a tenderness of the left iliac fossa and the bowel sounds were normal on auscultation. Inspection under speculum revealed normal gravid external cervical os and there was no vaginal discharge. On digital exploration, the cervix was posterior long and closed, the uterus was globular, increased in size and compatible with an eight weeks pregnancy. The left adnexae presented with a tender, smooth and mobile mass of seven centimeters diameter but the right one were normal. The posterior cervico-vaginal fornix was neither tender nor bulging. We suspected a non ruptured extra-uterine pregnancy with the following differentials: heterotopic pregnancy, torsion of ovarian cyst in pregnancy, and intra-cystic ovarian bleeding in pregnancy. Paraclinical investigations revealed: beta human Chorionic Gonadotropin (β hCG) level of 96 702 milli International Units per milliliter of plasma (mIU/ml). Ultrasonography revealed a heterogenous left ovarian mass of 82 millimeters in diameter, an empty uterus and no peritoneal effusion. This comforted our first diagnosis. After a normal pre-operative work up, an emergency laparotomy was done under general anesthesia. The findings were: left ovary containing a gestational sac and the corpus luteum, normal right adnexae, normal left tube and normal uterus. There was no hemoperitoneum. We dissected the ovarian capsule and carried out ablation of the gestational sac, then we did hemostasis. The specimen was analysed by the pathologist who found decidual cells and trophoblastic tissue within the ovarian capsule and thus confirmed the ovarian pregnancy. Post operative course was uneventful and the patient was discharged six days after surgery. The ?hCG level decreased and disappeared 25 days after surgery.

EN100068

Headaches were lasting for hours.

A 21 year old female patient with the diagnosis of SWS suffering from headaches admitted to our clinic. She had a 2 year history of frequent non-pulsating headaches. Her headache was relieving with non-steroidal anti-inflammatory drugs and was not worsening with physical activity. There was no nausea or aura like symptoms accompanying the headache. Headaches were lasting for hours. The pain was bilateral, generalized and pressing in quality. The family history for headache was negative. She had a history of seizures occurring in the fifteenth day of life described as attacks of tonic clonic contractions and that's when she was diagnosed with SWS. At the age of 6 she had a history of callosotomy to control her seizures. At the age of 18 during a laser treatment done in order to get rid of her port wine birthmark she had her first seizure since callosotomy. After that she was prescribed carbamazepine 400 mg at daily dose and never had a seizure since then. According to the story taken from her parents even though she had a normal development at infancy she barely graduated from elementary school and she's hardly literate. There was nothing significant on her family history except for her elder sister's port wine stain on her face. The elder sister had no feature of SWS and no researches were done regarding her stain. She was inscribed daily doses of ketiapin 25 mg for anxiety disorder and venlafaxine 75 mg for both anxiety disorder and the chronic headaches. She was also inscribed NSAID drugs. After the first week of this treatment her headaches were slightly decreased by heaviness but the frequency was the same. At her physical examination a facial nevus -occurred due to choroid angioma-on the right forehead, right eyelid, nasal wing and the cheek was observed. Intra oral examination showed a right sided overgrowth of gingiva. Gingival overgrowth was bright red in color and showed blanching on applying pressure suggesting angiomatous enlargement. On her extremities a mild asymmetry was visible. Her left arm and leg was slightly smaller in portions and showed hemiparesis both in the upper and lower extremities of the same size. On her ophthalmological evaluation she was diagnosed with glaucoma of the right eye. On her psychiatric examination she showed signs of anxiety disorder. Her neurological examination was not remarkable except for her hemiparesis. Cranial CT scans showed diffuse atrophy in the right hemisphere and irregular double-contoured gyriform cortical calcifications in the right occipital area. Gadolinium enhanced brain MRI revealed multiple dilated pial venous vascular structures on right hemisphere also with the diffuse atrophy on the same side. Axial T1 weighted cranial MRI shows right calvarial thickness compared to the left and right hemisphere is asymmetrically smaller than the left. In addition to that, T2 weighted MRI shows extensive venous formations around corpus of right lateral ventricle and at Gallen vein localization and widespread vascular formations are seen at perivascular space, anterior to third ventricle at Willis polygon localization and at right temporooccipital area at quadrigeminal cistern localization. She was performed a proteus intelligence test in which she had 75 points and accepted as mildly mentally retarded. Proteus intelligence test in which she had 75 points and accepted as mildly mentally retarded.

EN100129

In addition to that, T2 weighted MRI shows extensive venous formations around corpus of right lateral ventricle and at Gallen vein localization and widespread vascular formations are seen at perivascular space, anterior to third ventricle at Willis polygon localization and at right temporooccipital area at quadrigeminal cistern localization.

A 21 year old female patient with the diagnosis of SWS suffering from headaches admitted to our clinic. She had a 2 year history of frequent non-pulsating headaches. Her headache was relieving with non-steroidal anti-inflammatory drugs and was not worsening with physical activity. There was no nausea or aura like symptoms accompanying the headache. Headaches were lasting for hours. The pain was bilateral, generalized and pressing in quality. The family history for headache was negative. She had a history of seizures occurring in the fifteenth day of life described as attacks of tonic clonic contractions and that's when she was diagnosed with SWS. At the age of 6 she had a history of callosotomy to control her seizures. At the age of 18 during a laser treatment done in order to get rid of her port wine birthmark she had her first seizure since callosotomy. After that she was prescribed carbamazepine 400 mg at daily dose and never had a seizure since then. According to the story taken from her parents even though she had a normal development at infancy she barely graduated from elementary school and she's hardly literate. There was nothing significant on her family history except for her elder sister's port wine stain on her face. The elder sister had no feature of SWS and no researches were done regarding her stain. She was inscribed daily doses of ketiapin 25 mg for anxiety disorder and venlafaxine 75 mg for both anxiety disorder and the chronic headaches. She was also inscribed NSAID drugs. After the first week of this treatment her headaches were slightly decreased by heaviness but the frequency was the same. At her physical examination a facial nevus -occurred due to choroid angioma-on the right forehead, right eyelid, nasal wing and the cheek was observed. Intra oral examination showed a right sided overgrowth of gingiva. Gingival overgrowth was bright red in color and showed blanching on applying pressure suggesting angiomatous enlargement. On her extremities a mild asymmetry was visible. Her left arm and leg was slightly smaller in portions and showed hemiparesis both in the upper and lower extremities of the same size. On her ophthalmological evaluation she was diagnosed with glaucoma of the right eye. On her psychiatric examination she showed signs of anxiety disorder. Her neurological examination was not remarkable except for her hemiparesis. Cranial CT scans showed diffuse atrophy in the right hemisphere and irregular double-contoured gyriform cortical calcifications in the right occipital area. Gadolinium enhanced brain MRI revealed multiple dilated pial venous vascular structures on right hemisphere also with the diffuse atrophy on the same side. Axial T1 weighted cranial MRI shows right calvarial thickness compared to the left and right hemisphere is asymmetrically smaller than the left. In addition to that, T2 weighted MRI shows extensive venous formations around corpus of right lateral ventricle and at Gallen vein localization and widespread vascular formations are seen at perivascular space, anterior to third ventricle at Willis polygon localization and at right temporooccipital area at quadrigeminal cistern localization. She was performed a proteus intelligence test in which she had 75 points and accepted as mildly mentally retarded. Proteus intelligence test in which she had 75 points and accepted as mildly mentally retarded.

EN100129

There was no leukocytosis, and the serum level of C-reactive protein was normal.

We report a case of a male near-term neonate with gestational age of 35 weeks. He was the third born child to a 27-year-old mother with no significant past history. He was derived from a third degree consanguineous marriage. He was born by means of normal vaginal delivery with a normal extra-uterine adaptation. Meconium-stained amniotic fluid was noted. His birth weight was 3030g (75 th Percentile). The baby had normal physical examination at birth aside transitory respiratory distress. He had been hospitalized for four days in a private clinic, for the suspicion of materno-foetal infection, where he had antibiotics for 48 hours. He had developed no symptoms during his hospitalization. The output's weight was 2400g which signify the loss of 630g. Back home, he received exclusive maternal breastfeeding. On his fifth day of life, he presented multiple yellowish-watery stools (ab 12 times) and refuse feeding with apyrexia context. He had clinical signs of severe dehydration without fever or vomiting. He lost 930g relative to the birth's weight. The abdomen appeared neither tender nor distended, and the rest of his physical examination was normal. The laboratory findings revealed hypernatremia of 152mEq/L, hyperkalemia of 7,8mEq/L and severe metabolic acidosis: blood PH: 7,1, HCO3:4mEq/L. The osmotic gap was 45. There was no leukocytosis, and the serum level of C-reactive protein was normal. The infant was treated with intravenous fluids and electrolytes as well as antibiotics after obtaining blood and stool cultures, assuming an infectious etiology. After 26 hours of rehydration therapy, the dehydration resolved, however, a functional acute renal failure complicated by acidosis and hyperkalemia appeared. The chest radiograph showed "bat wings" pattern and the cardiothoracic index was 0, 45. The renal ultrasound showed discretely hyperechoic kidneys cortex and the absence of renal malformations. On the 11 th day of life, he was transferred to the pediatric intensive care unit. He needed mechanical ventilation for three days and exclusive parenteral feeding by central catheter. The evolution was burdened by cholestasis due to the prolonged parenteral feeding and nosocomial infection by staphylococcus epidermidis. Since his admission, he had developed a slimy diarrhea with 3 to 4 stools per day. He presented a profuse diarrhea during each attempt to introduce enteral feeding by hydrolyzed milk formula. He was discharged after 32 days with output's weight 3250g. The oesophago-gastro-duodenoscopy and duodenal biopsy showed partial villous atrophy associated to the brush border abnormalities. The periodic acid Schiff (PAS) stain revealed abnormalities in the brush border characterized by loss of the linear part of enterocytes with the presence of intra-cytoplasmic PAS + band in the apical pole of enterocytes uneven thickness and sometimes with double contours pattern. In the submucosa, there were numerous Brunner glands. We find the existence of numerous hemorrhagic suffusions. The CD10 immunostain was positive. Thus, a diagnosis hypothesis of MVID was raised. The electron microscopic examination confirmed the diagnosis. Mutation analysis of peripheral blood samples of the neonate revealed a mutation at the homozygous status of MYO5B gene. The baby died, after 97 days of life, of septic shock and multiple organ failure.

EN100376

Signs of fluid accumulation were not found.

A 35-year-old mentally ill man was transferred from a local psychiatric hospital after attempted suicide by fire 3 days before admission. He acquired full-and partial-thickness injury in approximately 38% of his TBSA, including face and neck (5%), both upper extremities (8%), right thigh (4%), left thigh and leg (3%), and most of his anterior and posterior trunk (16%). The patient was hospitalized for 66 days and underwent five debridement surgeries. The psychiatric diagnosis was schizophrenia type ICD 10 F20.3, treated with Haloperidol 1.5 mg and Diazepam 5 mg daily. The patient was occasionally restrained due to rebellious and self-destruction behavior. The nutrition management of this patient was started after a week of hospitalization, which is considered late. In the initial nutrition assessment, the patient was 40 kg weight and 150 cm tall thus the body mass index (BMI) was 17,57 kg/m 2 or underweight. Gastrointestinal symptoms and signs were not found. Information regarding the patient's dietary intake in the last two weeks and weight change within the last 6 months could not be obtained, but his mother claimed that the patient seemed to lose weight because he refused any food that had been offered to him; he only smoked and drank coffee. Albumin level was 3.6 gr/L with imbalanced electrolyte levels. Signs of fluid accumulation were not found. According to the subjective global assessment (SGA) tool, the patient belonged to group C (severely malnourished). Energy and protein were given starting from 30 kcal/kg, increasing gradually to 50 kcal/kg, and 2 gp/kg divided into oral nutrition and oral nutrition supplements (ONS) with a 50:50 ratio. The patient was also given micronutrient supplementations, specifically vitamin A 100,000 units for the first week, vitamin C 100 mg 3 times a day, and zinc 20 mg once a day. During the early weeks of hospitalization, the patient's weight loss continued. At the end of the second week, his body weight was 36 kilograms while his serum albumin level was 2.7 gr/L. On the 28 th day, the patient's body weight was 33 kilograms while his serum albumin level was 2.1 gr/L. Energy and protein intake was maintained, but the ratio of oral intake and ONS was modified to 70:30. During the treatment, the patient's appetite was unstable. The patient was also hardly cooperative with enteral access. The patient often writhed in pain and became more aggressive. The patient did not respond well to analgesics and sedations. Partially parenteral nutrition (PN) enriched with branched-chain amino acid (BCAA) was administered to support protein needs. However, his BMI continued to decline, and on the 42 nd day, his body weight dropped to 32 kilograms with a serum albumin level of 2 gr/L. On the 66 th day, granulation tissues appeared on the wound bed. Considering the mental and nutrition status of the patient, it was decided not to continue with the skin graft surgery. The patient had lost 24% of body weight (BMI 11.96 kg/m 2) and the serum albumin level was 2.5 gr/L. The patient displayed severe muscle wasting and subcutaneous fat loss. However, the general and mental condition was relatively better. The patient was discharged from the hospital.

EN100658

But pain didn't relieve and limitations in range of motion occurred at knee joint.

A seventy two years old male presented to our clinic with swelling, pain, and limitation of movement at knee joint. The patient's history revealed swellings and pain at knee joint for nearly last 20 years. He was prescribed nonsteroidal anti-inflammatory drugs and rest. But pain didn't relieve and limitations in range of motion occurred at knee joint. Physical examination findings included a solid and semi-mobile mass at anterior knee region. Knee joint range of motion was 80 degrees flexion and 0 degrees extension. There wasn't any neurovascular pathology. Antero-posterior and lateral knee X-rays showed degenerative changes and a giant, irregular, calcific mass which started below patella and extended to suprapatellar area. Knee MRI revealed many loose bodies (consistent with synovial chondromatosis) isointense with fatty bone marrow at all sequences at the level of right knee joint. Scalloping was observed at tibial and femoral surfaces. Decision for a surgical intervention was made and after informing the patient, consent was taken. Under spinal anesthesia, a tourniquet was applied to right lower extremity. Right knee was entered with an anterior longitudinal incision. Cutaneous and subcutaneous tissues were passed. Quadriceps mechanism was opened with a medial parapatellar incision and patella was toppled to left side. Intraarticular mass was exposed. The mass was released from surrounding tissues by blunt excision and excised with enucleation. Then synovial tissue was excised with synoviectomy. Surgical area was irrigated using abundant isotonic solution. The excised mass was sent to pathology laboratory. Pathology evaluation showed that the mass was consistent with synovial chondromatosis. Active and passive exercises were started at an early period after surgery. Recurrence was not detected at his last control visit. Knee movements were at full range when compared with the other knee. There was no pain or other complaints.

EN100247

Quadriceps mechanism was opened with a medial parapatellar incision and patella was toppled to left side.

A seventy two years old male presented to our clinic with swelling, pain, and limitation of movement at knee joint. The patient's history revealed swellings and pain at knee joint for nearly last 20 years. He was prescribed nonsteroidal anti-inflammatory drugs and rest. But pain didn't relieve and limitations in range of motion occurred at knee joint. Physical examination findings included a solid and semi-mobile mass at anterior knee region. Knee joint range of motion was 80 degrees flexion and 0 degrees extension. There wasn't any neurovascular pathology. Antero-posterior and lateral knee X-rays showed degenerative changes and a giant, irregular, calcific mass which started below patella and extended to suprapatellar area. Knee MRI revealed many loose bodies (consistent with synovial chondromatosis) isointense with fatty bone marrow at all sequences at the level of right knee joint. Scalloping was observed at tibial and femoral surfaces. Decision for a surgical intervention was made and after informing the patient, consent was taken. Under spinal anesthesia, a tourniquet was applied to right lower extremity. Right knee was entered with an anterior longitudinal incision. Cutaneous and subcutaneous tissues were passed. Quadriceps mechanism was opened with a medial parapatellar incision and patella was toppled to left side. Intraarticular mass was exposed. The mass was released from surrounding tissues by blunt excision and excised with enucleation. Then synovial tissue was excised with synoviectomy. Surgical area was irrigated using abundant isotonic solution. The excised mass was sent to pathology laboratory. Pathology evaluation showed that the mass was consistent with synovial chondromatosis. Active and passive exercises were started at an early period after surgery. Recurrence was not detected at his last control visit. Knee movements were at full range when compared with the other knee. There was no pain or other complaints.

EN100247

His Sp02 fell by 70% on room air after waking.

A 43-year-old non-diabetic Indian male reported to our outpatient department with chief complaints of cough with expectoration, chest pain, reduced appetite, fever with chills, and night sweats for two weeks. He also complained of breathlessness on exertion and had two episodes of blood in his sputum. The patient explained that the cough was continuous and was relieved after taking cough syrup. He also mentioned that the episodes of fever were initially intermittent and then daily for the last two weeks and were relieved after taking Paracetamol. The chest pain was localized to the middle of the chest and was aggravated on exertion. He was a businessman by profession with no history of smoking, alcoholism, or any other substance abuse. Also, there was no history of any contact of TB or COVID-19 in the family or close contacts. And there was no history of foreign travel in the recent past. But he had reported having traveled by a domestic airline about twenty days back. There was no history of weight loss or any other major illness in the past. On examination his vitals were-pulse-108/minute, arterial BP-130/80 mm of Hg, respiratory rate of 30 breaths/minute, Sp02-899% on room air, temperature- 101-degree centigrade. His Sp02 fell by 70% on room air after waking. On auscultation, there was crepitation on the bilateral middle lobes of the lungs. Also, dyspnea on exertion was noted. The rest of the systemic examination was within normal limits. Considering this as a probable case of TB with COVID-19 he was advised a chest radiograph with sputum microscopy (Ziehl Neelsen (ZN) staining for acid-fast bacilli), Cartridge-based nucleic acid amplification test (CBNAAT) of the sputum and other routine investigations. To check for the COVID-19 he was advised qualitative polymerase chain reaction (PCR) test from the oropharyngeal swab. The results were surprising with Mycobacterium tuberculosis detected on sputum fluorescent microscopy and were also confirmed by the CBNAAT. However, there was no resistance to Rifampicin. The results of the PCR were positive for RNA specific to SARS-CoV-2. Besides, the chest radiograph PA-view was suggestive of bilateral consolidations on the middle lobes of lungs with ill-defined borders. The other investigations revealed a low lymphocyte count (1x 10 9 /L) and increased levels of C-reactive protein (CRP) (57 mg/L), lactate dehydrogenase (LDH) (580 U/L), and erythrocyte sedimentation rate (ESR) (70 mm in the 1 st hour). Also, a sample for liquid culture (MGIT BACTEC) was sent to the Intermediate Reference Laboratory (IRL) which revealed the growth of Mycobacterium tuberculosis. Computed tomography was not performed as the diagnosis was established by other cheaper and faster methods and also the patient was unwilling for the same. All the other routine investigations were within normal limits. He was referred to the nearest designated COVID-19 management center, where he was managed as per national guidelines. Besides, he was also started on an antitubercular treatment of four drugs as per the National Tuberculosis Elimination Program (NTEP) guidelines. He was advised follow-up post completion of his stay at the designated COVID-19 center, but he has not yet reported back for follow-up. Written informed consent was obtained from the patient for using clinical data and images for publication in this study.

EN100606

The patient was readmitted into our pediatric department with complains of severe headache, vomiting, pain and bilateral visual loss since three days.

A 12-year-old girl presented an abdominal pain, high persistent fever of (40°C) and severe fatigue. The physical exam revealed pallor, hepatomegaly, and grade III splenomegaly. The investigation revealed an elevated white blood cell count (WBC): 545,000/mm³. PNN: 49,050/mm³, hemoglobin: 11.6g/dl, platelets: 328,000/mm³. The patient's peripheral blood smear revealed marked leukocytosis with a significant number of immature myeloid precursors and 21% blasts. The analysis of the BCR-ABL shows 94% in the peripheral blood. The patient was diagnosed with CML. She received a chemotherapy protocol made of hyperalkalinisation, allopurinol, hydroxyurea and a treatment based on imatinib at the dose of 400mg per day. Patient achieved complete hematological remission at three months and complete cytogenetic remission at 12 months. The patient was readmitted into our pediatric department with complains of severe headache, vomiting, pain and bilateral visual loss since three days. On examination, patient was afebrile and appeared pale. Blood pressure was 100/60mm/hg and the heart rate 98 beat/minute. There was no lymphadenopathy and the nervous system examination was normal. Intraocular muscle testing was normal; however, there was an exophthalmia in both eyes. The pupil was sluggish to react to light. Fundoscopy revealed bilateral stade III disc edema and hemorrhages of the retina. She underwent a brain computed tomography (CT) scan with the suspicion of leukemic involvement or any other intracranial event (cerebral edema); however, nothing was found. A subsequent bone marrow exam showed no morphologic or molecular evidence of CML or acute leukemia. Cytological evaluation of cerebrospinal fluid (CSF) was also negative. The patient had no other known pathology that could be held responsible for retina edema. We suggested that it was a side effect of imatinib so its administration was temporary interrupted. The patient was put on an anti-edema therapy (mannitol, dexamethasone). However, few months later, the patient presented in the emergency room with a headache and severe vomiting. She described a recurrence of the same character of headaches accompanied with weakness of extremities and seizures. Nervous system examination revealed neck rigidity, presence of meningeal signs and lower limb paraplegia. CT of the brain revealed subdural chronic hematoma and a serpenginous gyriform enhancement around the contusion in the right parietal and occipital lobes. Analysis of the CSF collected showed lymphoblasts in a WBC count of 11000cells/ml. Flow cytometry of the CSF showed that blast cells were positive for a cluster of differentiation markers (CD) (CD34, CD19, CD10, CD22 and partially positive for CD45) confirming CNS extramedullary lymphoid blast infiltration. A bone marrow aspiration was done to evaluate the status of her chronic myelogenous leukemia and result was consistent with a chronic phase. Fundus examination showed bilateral optic atrophy. The diagnosis of extramedullary isolated CNS blast crises (lymphoid type) was based on the presence of blasts in CSF (confirmed by flow cytometry). The patient received a high-dose systemic induction chemotherapy and intrathecal therapy (methotrexate, arabinoside and dexamethasone). The imatinib was replaced by dasatinib at the dose of 100 mg per day. She was treated with 24 Gy of whole-brain radiation therapy. Allogenic stem cell transplantation was not feasible. We noticed a rapidly progressive amelioration in her neurological status after finishing systemic chemotherapy and physical therapy; however, the patient's visual loss showed no signs of improvement.

EN100543

And there was no history of foreign travel in the recent past.

A 43-year-old non-diabetic Indian male reported to our outpatient department with chief complaints of cough with expectoration, chest pain, reduced appetite, fever with chills, and night sweats for two weeks. He also complained of breathlessness on exertion and had two episodes of blood in his sputum. The patient explained that the cough was continuous and was relieved after taking cough syrup. He also mentioned that the episodes of fever were initially intermittent and then daily for the last two weeks and were relieved after taking Paracetamol. The chest pain was localized to the middle of the chest and was aggravated on exertion. He was a businessman by profession with no history of smoking, alcoholism, or any other substance abuse. Also, there was no history of any contact of TB or COVID-19 in the family or close contacts. And there was no history of foreign travel in the recent past. But he had reported having traveled by a domestic airline about twenty days back. There was no history of weight loss or any other major illness in the past. On examination his vitals were-pulse-108/minute, arterial BP-130/80 mm of Hg, respiratory rate of 30 breaths/minute, Sp02-899% on room air, temperature- 101-degree centigrade. His Sp02 fell by 70% on room air after waking. On auscultation, there was crepitation on the bilateral middle lobes of the lungs. Also, dyspnea on exertion was noted. The rest of the systemic examination was within normal limits. Considering this as a probable case of TB with COVID-19 he was advised a chest radiograph with sputum microscopy (Ziehl Neelsen (ZN) staining for acid-fast bacilli), Cartridge-based nucleic acid amplification test (CBNAAT) of the sputum and other routine investigations. To check for the COVID-19 he was advised qualitative polymerase chain reaction (PCR) test from the oropharyngeal swab. The results were surprising with Mycobacterium tuberculosis detected on sputum fluorescent microscopy and were also confirmed by the CBNAAT. However, there was no resistance to Rifampicin. The results of the PCR were positive for RNA specific to SARS-CoV-2. Besides, the chest radiograph PA-view was suggestive of bilateral consolidations on the middle lobes of lungs with ill-defined borders. The other investigations revealed a low lymphocyte count (1x 10 9 /L) and increased levels of C-reactive protein (CRP) (57 mg/L), lactate dehydrogenase (LDH) (580 U/L), and erythrocyte sedimentation rate (ESR) (70 mm in the 1 st hour). Also, a sample for liquid culture (MGIT BACTEC) was sent to the Intermediate Reference Laboratory (IRL) which revealed the growth of Mycobacterium tuberculosis. Computed tomography was not performed as the diagnosis was established by other cheaper and faster methods and also the patient was unwilling for the same. All the other routine investigations were within normal limits. He was referred to the nearest designated COVID-19 management center, where he was managed as per national guidelines. Besides, he was also started on an antitubercular treatment of four drugs as per the National Tuberculosis Elimination Program (NTEP) guidelines. He was advised follow-up post completion of his stay at the designated COVID-19 center, but he has not yet reported back for follow-up. Written informed consent was obtained from the patient for using clinical data and images for publication in this study.

EN100606

A chest X-ray was normal while the abdominal ultrasound showed a hepatomegaly and a splenomegaly, free biliary ducts without ascites.

We present here a case of Macrophage activation syndrome associated with Griscelli syndrome type 2 in a 3-years-old male born of consanguineous parentage. His birth histories, his familie social history and developmental milestones were unremarkable. He was born at full term with no antenatal or perinatal complications, He was on exclusively breast-fed, food diversification was started at 6 months old, his weight, length and psychomotor development were within the normal range, the child was described as a good eater, was on a normal diet and was thriving appropriately. Furthermore the boy presented a progressive abdominal distension since birth, progressive pallor and recurrent episodes of fever since 1 year of age. There was history of blood transfusions for last 2 months. On the other side there was no history of jaundice, vomiting, urinary or bowel complaints, bleeding from any site or neurological complaints. On admission, he was very pale with silvery gray scalp hair, white eyelashes, he was hypotonic, tachycardic, fever of 40°C, lymphoadenopathy and hepato-splenomegaly. The blood count showed pancytopenia; 1980 white blood cells/µL (VN 4000-13 500/µL), neutrophils 820/µL, 910 cells/µL, hemoglobin (Hb) 6.3 g/dL (11.5-14 VN, 5 g/dL), platelets 44 000/µL (VN 150 000-400 000/µL), There were no giant cytoplasmic granules in leucocytes. The liver function tests were normal expect low albumin (2.1 mg/dL) and increased alkaline phosphatase (1196 mg/dL) with low fibrinogen (1.7g/l). Serum triglycerides, ferrintin and lactic dehydrogenase were very high; respectively 4.75g/L; 2763µg/L and 597U/L. the C reactive protein (CRP) was elevated at 83mg/l. A chest X-ray was normal while the abdominal ultrasound showed a hepatomegaly and a splenomegaly, free biliary ducts without ascites. Because of hepato-splenomegaly, pancytopenia, hyperferritinemia and hypofibrinogenemia the diagnosis of Macrophage activation syndrome was evoked indicating a bone marrow aspiration that showed numerous morphologically benign macrophages with haemophagocytic activity that comforting the diagnosis of a SAM according to Ravelli and HLH-2004 criteria. Blood culture, urine examination, malarial serology, Kala Azar serology, viral serologies such as EBV, hepatitis A, B, C and human immunodeficiency virus HIV, serologies of rickettsia, syphilis and toxoplasmosa were negatives. Serologies of CMV and rubella showed an old immunization. The immunological tests such as antinuclear antibodies (ANA), anti-LKM1, anti-mitochondria and anti-smooth muscle antibodies were negatives. Because of the consanguineous family, notion of recurrent infection and the presence of silvery-gray color of the hair and eyebrows, diagnosis of Griscelli Syndrome was evoked directing a light microscopy examination of the hair that showed a large, irregular clumps of pigments characteristic of Griscelli Syndrome. The molecular biology showed mutation in RAB27A gene confirming the diagnosis of a Griscelli syndrome type 2. The first-line therapy in management of Macrophage activation syndrome complicating Griscelli syndrome type 2 was based on the parenteral administration of high doses of corticosteroids (methylprednisolone pulses 1g/0.73m 2 /day for 3 days then 60mg/m 2) with poor response; persisting with involvement of the 3 cell lines in the blood count, elevated ferritin and triglycerides. Given the lack of response to steroids, Etoposide was started for 3 days associated with cyclophosphamide, the evolution was good with improvement of count blood cells, ferritin was lessening while the bone marrow showed decreased numerous of macrophages with low haemophagocytic activity. On the other side the infectious process was good managed by perfusion of Antibiotics such as Ceftazidim in combination with aminoside. The patient was sent home with prednisone and cyclosporine, He was placed on the list of bone marrow transplantation (BMT).

EN100372

Laboratory investigations showed: leucopenia with white blood cell count = 3100/mm3, lymphopenia = 840/mm3, hemoglobin = 10.5g/dL ; low serum albumin level = 29g/L, 47;-globulin = 19g/L. blood glucose levels = 180 mg/dl and hemoglobin A1c = 8.6 %.

A 77-year-old Tunisian woman was hospitalized because of massive painful ascites. Her family history did not include any autoimmune disease. She denied a history of hepatitis, jaundice or alcohol use. She had a history of diabetes, hypertension treated by glinide and calcium blocker. She was explored 4 years prior to admission for exudative pleuritis of the right lung without any established diagnosis after multiple explorations including thoracoscopy with biopsies. On admission, blood pressure was 150/70 mmHg, her rate was 80 /mn and body temperature was 37°C. The physical examination showed only abdominal distension related to the important ascites without collateral venous circulation. She had no skin lesions, lymphadenopathy, or hepatosplenomegaly and lower extremities showed no edema. Laboratory investigations showed: leucopenia with white blood cell count = 3100/mm3, lymphopenia = 840/mm3, hemoglobin = 10.5g/dL ; low serum albumin level = 29g/L, 47;-globulin = 19g/L. blood glucose levels = 180 mg/dl and hemoglobin A1c = 8.6 %. Urine analysis showed trace protein (0.03g/24h). Platelet count, activated partial thromboplastin time, liver function tests, blood urea nitrogen, serum creatinine, erythrocyte sediment rate and total cholesterol were within normal limits. Tests for HBsAg and HCV were negative. Abdominal ultrasonography showed ascitic fluid without any sign of bowel loop thickening or enteritis. Neither lymphadenopathy nor liver abnormality was present. A chest X-ray film disclosed bilateral pleural effusion. Ascitic obtained by aspiration contained 170 cells mm3 (67% lymphocytes), 46g/L protein, but no malignant cells. Cultures for bacteria and mycobacteria gave no growth. Tuberculosis investigation including initial tuberculin skin test and the research of Koch bacilli in sputum and urine were negative. Echocardiogram and electrocardiogram were normal. Computed tomographic scan showed massive ascites, no dysmorphic liver, and a mild bilateral pleural effusion. Pelvic examination was normal. Oesogastroduodenoscopy showed hiatal hernia without oesophageal varices. An exploratory laparoscopy was performed, revealing a large amount of ascite, a normal liver appearance, and no granulations suggesting tuberculosis or peritoneal carcinosis. Histological examination of the peritoneum showed non specific chronic inflammation. Systemic lupus erythematosus was suspected based on pleuritis, lymphopenia and leucopenia. An additional serologic survey revealed markedly elevated anti-nuclear antibody (ANA) titer of 1/1600 and a significantly elevated titer of antibody to double-stranded DNA (83 IU/mL ; normal < 30 IU/mL). She had also positive serum antibody against the Smith antigen and low serum level of C3 complement component: 67 mg/dL (serum normal range: 84 – 151). Relying on these findings, the patient was diagnosed with SLE since 4 of the 11 diagnostic criteria of the American College of Rheumatology were met. The SLEDAI score according to Systemic Lupus Erythematosus Disease Activity Index was estimated to be 7 on admission. Hydroxychloroquine 200 mg daily in combination with diuretics was initiated. One month later, there was no detectable ascitic fluid and no pleural effusions. Five months later she remained free from symptoms while continuing to take Hydroxychloroquine

EN100114

Follow up CT brain showed resolving hematoma.

A 20 years old man presented with fever, myalgias, arthralgias, retro-orbital pain, vomiting and gum bleeding during a large dengue outbreak in Lahore, Pakistan. On 7th day of illness patient became afebrile, but he developed severe headaches, unconsciousness followed by altered behavior. On 9th day of illness patient developed painful neck swelling accompanied by fever, tremors, palpitations, hoarseness of voice and odynophagia. Examination revealed acutely swollen, tender thyroid gland along with features of hyperthyroidism. Laboratory evaluation revealed stable hematocrit, thrombocytopenia and leukopenia. Patient had seroconverted for anti-dengue IgM antibodies on the 10th day of illness. A non-contrast Computed Tomogram (CT) of the brain showed right frontal lobe hematoma. Thyroid profile showed increased free T3 and T4 and low TSH. Technetium thyroid scan showed reduced tracer uptake. He was diagnosed as having subacute thyroiditis and treated with oral prednisolone and propranolol. Follow up CT brain showed resolving hematoma. Patient's recovery was uneventful.

EN107021

He needed mechanical ventilation for three days and exclusive parenteral feeding by central catheter.

We report a case of a male near-term neonate with gestational age of 35 weeks. He was the third born child to a 27-year-old mother with no significant past history. He was derived from a third degree consanguineous marriage. He was born by means of normal vaginal delivery with a normal extra-uterine adaptation. Meconium-stained amniotic fluid was noted. His birth weight was 3030g (75 th Percentile). The baby had normal physical examination at birth aside transitory respiratory distress. He had been hospitalized for four days in a private clinic, for the suspicion of materno-foetal infection, where he had antibiotics for 48 hours. He had developed no symptoms during his hospitalization. The output's weight was 2400g which signify the loss of 630g. Back home, he received exclusive maternal breastfeeding. On his fifth day of life, he presented multiple yellowish-watery stools (ab 12 times) and refuse feeding with apyrexia context. He had clinical signs of severe dehydration without fever or vomiting. He lost 930g relative to the birth's weight. The abdomen appeared neither tender nor distended, and the rest of his physical examination was normal. The laboratory findings revealed hypernatremia of 152mEq/L, hyperkalemia of 7,8mEq/L and severe metabolic acidosis: blood PH: 7,1, HCO3:4mEq/L. The osmotic gap was 45. There was no leukocytosis, and the serum level of C-reactive protein was normal. The infant was treated with intravenous fluids and electrolytes as well as antibiotics after obtaining blood and stool cultures, assuming an infectious etiology. After 26 hours of rehydration therapy, the dehydration resolved, however, a functional acute renal failure complicated by acidosis and hyperkalemia appeared. The chest radiograph showed "bat wings" pattern and the cardiothoracic index was 0, 45. The renal ultrasound showed discretely hyperechoic kidneys cortex and the absence of renal malformations. On the 11 th day of life, he was transferred to the pediatric intensive care unit. He needed mechanical ventilation for three days and exclusive parenteral feeding by central catheter. The evolution was burdened by cholestasis due to the prolonged parenteral feeding and nosocomial infection by staphylococcus epidermidis. Since his admission, he had developed a slimy diarrhea with 3 to 4 stools per day. He presented a profuse diarrhea during each attempt to introduce enteral feeding by hydrolyzed milk formula. He was discharged after 32 days with output's weight 3250g. The oesophago-gastro-duodenoscopy and duodenal biopsy showed partial villous atrophy associated to the brush border abnormalities. The periodic acid Schiff (PAS) stain revealed abnormalities in the brush border characterized by loss of the linear part of enterocytes with the presence of intra-cytoplasmic PAS + band in the apical pole of enterocytes uneven thickness and sometimes with double contours pattern. In the submucosa, there were numerous Brunner glands. We find the existence of numerous hemorrhagic suffusions. The CD10 immunostain was positive. Thus, a diagnosis hypothesis of MVID was raised. The electron microscopic examination confirmed the diagnosis. Mutation analysis of peripheral blood samples of the neonate revealed a mutation at the homozygous status of MYO5B gene. The baby died, after 97 days of life, of septic shock and multiple organ failure.

EN100376

The retrosternal part of the goiter was released using the finger by the same incision.

A 50-years-old woman, hypertensive, hospitalized for a large cervical mass appeared 30 years ago. In the family history, her mother, sisters and cousins underwent a surgery for MNG. Despite of the large volume of the mass, the patient never described signs of cervical compression whatsoever respiratory, digestive, laryngeal, vascular or neurologic signs. She never suffered from thyroid dysfunction. Her neck was deformed by the voluminous formation classified grade III according to the WHO modified classification. The mass took the front and the two sides of the neck. Its surface was embossed and covered by a thin normal skin. There were some veins of the collateral circulation limited to the neck. The goiter measured 18 x 11 cm. The mass was firm, painless, and mobile with the swallowing movements. Lymphadenopathy research was difficult and found no palpable lymph nodes. The laboratory tests (T 3, T 4 and TSH) were normal. Thoracic radiography showed a large cervical opacity roughly round and strewn with microcalcifications associated with a right eccentricity of the trachea. Cervical and chest CT revealed the presence of a partially calcified thyroid mass slightly plunging in the anterior mediastinum. It took heterogeneously the contrast and then evocate a large MNG. The trachea was surrounded by the goiter, slightly narrowed and right deviated as well as the lower part of the larynx. The right and left vascular axes of the neck (carotid artery and jugular vein) were deviated backward. The patient underwent a surgery for her enormous MNG slightly plunging in the mediastinum. Endotracheal intubation was relatively easy by the laryngoscope. The incision performed was a Kocher cervicotomy. There was a multinodular, hypervascularized goiter. Its lower end plunges behind the sternal manubrium. The larynx was deviated towards the right side. The total thyroidectomy was performed in two steps: initially a right lobo-isthmectomy, then the left lobectomy. The retrosternal part of the goiter was released using the finger by the same incision. Both recurrent laryngeal nerves (RLN) were not identified because of the hemorrhage. One parathyroid gland was accidently devascularized and was autotransplanted to the ipsilateral sternocleidomastoid muscle. The operation was finished by double aspiration drainage. In the first hours after surgery, the patient developed a large cervical hematoma. She was readmitted to the operating room, and after evacuation of the hematoma there was no vessels bleeding. The operation was completed with a double suction drainage. In the immediate postoperative period, the patient developed hemodynamic collapse requiring the introduction of dobutamine. After 48 hours of hemodynamic support, the blood pressure stabilized and dobutamine was stopped. Histological study concluded in multinodular colloid goiter. The patient was discharged from the hospital after 20 days in good health.

EN100067

The patient underwent complete endoscopic removal of the tumor with ethmoidectomy and right maxillary antrostomy.

Mr F.B a 70-year-old male patient presented in our institution because of the occurrence of a right epistaxis. First of all, the patient is not smoking and reported an aneurysm of the abdominal aorta surgically treated by prosthesis under plavix. The beginning of the symptomatology goes back to 2 months before with right nasal obstruction of progressive onset, associated with right epistaxis and bloody rhinorrhea with right chronic eye watering. Nasal Endoscopy examination revealed a pinnkish burgeoning mass bleeding on contact and filling the right nostril vestibule. The patient didn't presents neurological signs nor cervical lymph nods. Otherwise, ophthalmologic examination found normal visual acuity and ocular mobility on both sides. Sinonasal computed tomography (CT) found a right nasal process enhanced after injection of contrast product with relatively little washing at the late time. Otherwise, there is no extension involvement at the level of the cavum and the pterygo palate fossa. This finding found also a crenate appearance of the posterior nasal mucosa especially of the middle turbinates and contralateral inferior turbinate with a left deviation of the nasal septum with nasal spur. Nasosinusal MRI shows a progressive increase in size of the tissue mass compared to the previously CT occupying the right nasal fossa on almost all of its height, measuring 4 × 2.8 × 3.5cm without extension within the cavum with probable invasion of the lower part of the nasolacrimal duct and mass effect on both the sinusonasal bone septum and the intersinusonasal septum. This finding shows ethmoidal sinus fluid retention. The appearance, even if it remains unspecific, is compatible with inverted papilloma. The patient underwent complete endoscopic removal of the tumor with ethmoidectomy and right maxillary antrostomy. The extemporaneous examination is in favor of a malignant tumor lesion. Histopathologic analysis shows a tumor proliferation made of polymorphic cells with major anisocytosis, most often located in lobules. There is sometimes a somewhat palisadic seat on the periphery of the tumor mass. The mitotic index is 10 mitoses per 10 high power fields. The immunohistochemical study of the tumor found that the cytokeratin KL1 and CK7 and the neuroendocrine marker Chromogranin A, synaptophysin and CD56 are positive. Proliferation index Ki67 is of the order of 80%. Otherwise there is an absence of expression of PS100, MelanA, HMB45, desmin, p40, CD45 and EBV. The clear positivity of cytokeratins formally excludes the diagnosis of olfactory neuroblastoma and leads to neuroendocrine carcinoma. Given the atypia, the mitotic index, and the Ki67 proliferation index, it is a high-grade large-cell neuroendocrine carcinoma. Extension assessment, comprising a PET SCAN, was negative. Postoperative treatment consisted in adjuvant polychemotherapy, with six cycles (cisplatin and etoposide), Followed by loco regional external radiotherapy. After 6 month follow-up, the patient was in complete clinical and radiological remission.

EN100383

Abdominal ultrasonography showed ascitic fluid without any sign of bowel loop thickening or enteritis.

A 77-year-old Tunisian woman was hospitalized because of massive painful ascites. Her family history did not include any autoimmune disease. She denied a history of hepatitis, jaundice or alcohol use. She had a history of diabetes, hypertension treated by glinide and calcium blocker. She was explored 4 years prior to admission for exudative pleuritis of the right lung without any established diagnosis after multiple explorations including thoracoscopy with biopsies. On admission, blood pressure was 150/70 mmHg, her rate was 80 /mn and body temperature was 37°C. The physical examination showed only abdominal distension related to the important ascites without collateral venous circulation. She had no skin lesions, lymphadenopathy, or hepatosplenomegaly and lower extremities showed no edema. Laboratory investigations showed: leucopenia with white blood cell count = 3100/mm3, lymphopenia = 840/mm3, hemoglobin = 10.5g/dL ; low serum albumin level = 29g/L, 47;-globulin = 19g/L. blood glucose levels = 180 mg/dl and hemoglobin A1c = 8.6 %. Urine analysis showed trace protein (0.03g/24h). Platelet count, activated partial thromboplastin time, liver function tests, blood urea nitrogen, serum creatinine, erythrocyte sediment rate and total cholesterol were within normal limits. Tests for HBsAg and HCV were negative. Abdominal ultrasonography showed ascitic fluid without any sign of bowel loop thickening or enteritis. Neither lymphadenopathy nor liver abnormality was present. A chest X-ray film disclosed bilateral pleural effusion. Ascitic obtained by aspiration contained 170 cells mm3 (67% lymphocytes), 46g/L protein, but no malignant cells. Cultures for bacteria and mycobacteria gave no growth. Tuberculosis investigation including initial tuberculin skin test and the research of Koch bacilli in sputum and urine were negative. Echocardiogram and electrocardiogram were normal. Computed tomographic scan showed massive ascites, no dysmorphic liver, and a mild bilateral pleural effusion. Pelvic examination was normal. Oesogastroduodenoscopy showed hiatal hernia without oesophageal varices. An exploratory laparoscopy was performed, revealing a large amount of ascite, a normal liver appearance, and no granulations suggesting tuberculosis or peritoneal carcinosis. Histological examination of the peritoneum showed non specific chronic inflammation. Systemic lupus erythematosus was suspected based on pleuritis, lymphopenia and leucopenia. An additional serologic survey revealed markedly elevated anti-nuclear antibody (ANA) titer of 1/1600 and a significantly elevated titer of antibody to double-stranded DNA (83 IU/mL ; normal < 30 IU/mL). She had also positive serum antibody against the Smith antigen and low serum level of C3 complement component: 67 mg/dL (serum normal range: 84 – 151). Relying on these findings, the patient was diagnosed with SLE since 4 of the 11 diagnostic criteria of the American College of Rheumatology were met. The SLEDAI score according to Systemic Lupus Erythematosus Disease Activity Index was estimated to be 7 on admission. Hydroxychloroquine 200 mg daily in combination with diuretics was initiated. One month later, there was no detectable ascitic fluid and no pleural effusions. Five months later she remained free from symptoms while continuing to take Hydroxychloroquine

EN100114

A 70yrs old Female was admitted in our institution diagnosed with severe bilateral Osteoarthritis.

A 70yrs old Female was admitted in our institution diagnosed with severe bilateral Osteoarthritis. The x-rays showed bone on bone Tricompartment OA Knee with Varus Malalignment. She was posted for Single Stage Bilateral Total Knee Replacement and as planned the Left Knee Was Operated first. After exposure, Proximal Tibial, Distal Femoral Cuts and measurement of extension gaps the synovium from the anterior Femur was removed and sizing was done. The AP cut was then proceeded with. We spotted a small Osteochondral Cyst in the Anterior Femur which was curretted to remove the cystic material, which is when we realised that the cyst was large and communicating with the medulary canal. The remaining Femoral preparations was done keeping in mind the risk of iatrogenic fracture and extension Stem was used in the femur. The defect was then packed cancellous bone graft.

EN106233

Thyroid profile showed increased free T3 and T4 and low TSH.

A 20 years old man presented with fever, myalgias, arthralgias, retro-orbital pain, vomiting and gum bleeding during a large dengue outbreak in Lahore, Pakistan. On 7th day of illness patient became afebrile, but he developed severe headaches, unconsciousness followed by altered behavior. On 9th day of illness patient developed painful neck swelling accompanied by fever, tremors, palpitations, hoarseness of voice and odynophagia. Examination revealed acutely swollen, tender thyroid gland along with features of hyperthyroidism. Laboratory evaluation revealed stable hematocrit, thrombocytopenia and leukopenia. Patient had seroconverted for anti-dengue IgM antibodies on the 10th day of illness. A non-contrast Computed Tomogram (CT) of the brain showed right frontal lobe hematoma. Thyroid profile showed increased free T3 and T4 and low TSH. Technetium thyroid scan showed reduced tracer uptake. He was diagnosed as having subacute thyroiditis and treated with oral prednisolone and propranolol. Follow up CT brain showed resolving hematoma. Patient's recovery was uneventful.

EN107021

The patient underwent upper endoscopy as duodenal biopsy to confirm a celiac disease diagnosis.

We received a boy 3-year-old boy with autistic disorder on hospital of pediatric ward A at university hospital Mohammed VI of Marrakesh. He has no family history of illness or autistic spectrum disorder. The history revealed that at 2 years of age. The child was diagnosed with a ‘‘severe communication disorder,'' with social interaction difficulties and sensory processing delay. A composite follow-up of all previous assessments and investigations was undertaken. Blood work was normal (thyroid-stimulating hormone (TSH), hemoglobin, mean corpuscular volume (MCV), and ferritin). Genetic testing was unremarkable (normal karyotype, negative for fragile X) and the magnetic resonance imaging (MRI) in search of a demyelinating attack of the white matter, electroencephalography (EEG), optometry assessment were also normal. At the conclusion of this composite assessment, the boy was given a primary diagnosis of autistic spectrum disorder. A plan was instituted including speech-language therapy, intensive individualized educational programming, and contact was encouraged with the Autism Society. For that purpose, the parents moved to morocco to be surrounded by family without any huge improvement. Upon direct questioning at initial presentation, the 3-year-old child was reported by the parents to have unexplained fatigue, Gastrointestinal symptoms included bloating, constipation and diarrhea. Psychiatric symptoms included a frequently depressed mood, disproportionate anger, and emotional lability. On exploring his history, the child was born from yemenite father and Moroccan mother in Germany at-term weighing 3500 g with an Apgar score of 9 at 1 minute and 10 at 5 minutes after an uneventful pregnancy and delivery. No concerns were present in the neonatal period. Development in the first 24 months of life appeared fine according to the parents-his motor skills seemed to progress normally and he achieved expected milestones. After 2 years of age, however, his language skills slowly began to regress. He also demonstrated a change in temperament as he started to whine repeatedly and to scream without provocation. Physical examination revealed a height and weight normal for age. He was uncommunicative, restless, and somewhat agitated. General examination was unremarkable other than dark rings around the eyes. There were no dysmorphic features evident. He had difficulty maintaining eye contact, and he appeared disinterested in what was taking place. No abnormality was found on abdominal assessment. Anti-tissue transglutaminase antibodies levels were 76 U (normal < 10). The patient underwent upper endoscopy as duodenal biopsy to confirm a celiac disease diagnosis. It shows a total villous atrophy corresponding to a stage 4 of Marsh classification. Given the positive screen for celiac disease (positive anti-tissue transglutaminase antibodies and results of duodenal biopsy), dietary intervention was immediately commenced. All gluten was eliminated from the boy's diet. Within 1 month, the boy's gastrointestinal symptoms were relieved and his behavior had changed. The mother reported that her boy became progressively more communicative. The child has continued on the gluten-free diet and has progressed well and remained healthy over the following 6 months.

EN100490

Nasal Endoscopy examination revealed a pinnkish burgeoning mass bleeding on contact and filling the right nostril vestibule.

Mr F.B a 70-year-old male patient presented in our institution because of the occurrence of a right epistaxis. First of all, the patient is not smoking and reported an aneurysm of the abdominal aorta surgically treated by prosthesis under plavix. The beginning of the symptomatology goes back to 2 months before with right nasal obstruction of progressive onset, associated with right epistaxis and bloody rhinorrhea with right chronic eye watering. Nasal Endoscopy examination revealed a pinnkish burgeoning mass bleeding on contact and filling the right nostril vestibule. The patient didn't presents neurological signs nor cervical lymph nods. Otherwise, ophthalmologic examination found normal visual acuity and ocular mobility on both sides. Sinonasal computed tomography (CT) found a right nasal process enhanced after injection of contrast product with relatively little washing at the late time. Otherwise, there is no extension involvement at the level of the cavum and the pterygo palate fossa. This finding found also a crenate appearance of the posterior nasal mucosa especially of the middle turbinates and contralateral inferior turbinate with a left deviation of the nasal septum with nasal spur. Nasosinusal MRI shows a progressive increase in size of the tissue mass compared to the previously CT occupying the right nasal fossa on almost all of its height, measuring 4 × 2.8 × 3.5cm without extension within the cavum with probable invasion of the lower part of the nasolacrimal duct and mass effect on both the sinusonasal bone septum and the intersinusonasal septum. This finding shows ethmoidal sinus fluid retention. The appearance, even if it remains unspecific, is compatible with inverted papilloma. The patient underwent complete endoscopic removal of the tumor with ethmoidectomy and right maxillary antrostomy. The extemporaneous examination is in favor of a malignant tumor lesion. Histopathologic analysis shows a tumor proliferation made of polymorphic cells with major anisocytosis, most often located in lobules. There is sometimes a somewhat palisadic seat on the periphery of the tumor mass. The mitotic index is 10 mitoses per 10 high power fields. The immunohistochemical study of the tumor found that the cytokeratin KL1 and CK7 and the neuroendocrine marker Chromogranin A, synaptophysin and CD56 are positive. Proliferation index Ki67 is of the order of 80%. Otherwise there is an absence of expression of PS100, MelanA, HMB45, desmin, p40, CD45 and EBV. The clear positivity of cytokeratins formally excludes the diagnosis of olfactory neuroblastoma and leads to neuroendocrine carcinoma. Given the atypia, the mitotic index, and the Ki67 proliferation index, it is a high-grade large-cell neuroendocrine carcinoma. Extension assessment, comprising a PET SCAN, was negative. Postoperative treatment consisted in adjuvant polychemotherapy, with six cycles (cisplatin and etoposide), Followed by loco regional external radiotherapy. After 6 month follow-up, the patient was in complete clinical and radiological remission.

EN100383

Brain and orbital magnetic resonance imaging (MRI) revealed an extra-connal right orbital lesion near the orbital apex, measuring 28 x 18mm, of oval shape, it appeared hypo-signal in T1 and hyper-signal in T2, limited by a thin wall which took contrast product, this lesion compressed the optic nerve towards the nasal region.

A 3 years old girl born from non-consanguineous parents, without any neonatal suffering; with good psychomotor development, from a popular district in one of our cities, without any obvious contact with dogs; presented with exophthalmia associated with unilateral blindness evolving rapidly within 3 months. Clinical examination showed a non-pulsatile, painless, axial, irreducible exophthalmia with no sign of conjunctivitis or keratitis, and right monocular blindness, right ptosis; and fundal examination had objectified right papillary oedema. The rest of the clinical examination was normal. Brain and orbital magnetic resonance imaging (MRI) revealed an extra-connal right orbital lesion near the orbital apex, measuring 28 x 18mm, of oval shape, it appeared hypo-signal in T1 and hyper-signal in T2, limited by a thin wall which took contrast product, this lesion compressed the optic nerve towards the nasal region. Chest x-ray and abdominal ultrasound did not reveal any other localization. The patient was operated by performing a right extra-dural frontal approach, a cyst puncture was done in the first intention because the cyst was adherent to the neighbouring structures making its complete removal impossible, then a microscopic extirpation of the cystic membrane, combined with abundant washing by hypertonic serum to sterilize the cystic sit and reduce chances of dissemination. Histological examination was in favour of intra-orbital hydatidosis. Postoperatively, the patient was given an antihelminthic treatment of albendazole at a dose of 10mg per kilogram per day in 3 doses separated by 2 weeks; the evolution was marked by the significant regression of the exophthalmia and the gradual improvement of the visual acuity. The patient however still retains a right unilateral nasal hemianopia after two years of follow-up. An MRI done in the same period shows a cure of the patient by a complete disappearance of the cyst.

EN100453

Upon taking medical history the patient revealed that was diagnosed with thymoma MG and on medication including pyridostigmine bromide 60 mg, azathioprine 50 mg and prednisone 20 mg.

A 28-year-old female patient presented to outpatient dental clinics for full mouth rehabilitation. On physical examination, the patient was awake, alert, oriented, and her blood pressure, temperature and heart rate were within normal limits. Upon taking medical history the patient revealed that was diagnosed with thymoma MG and on medication including pyridostigmine bromide 60 mg, azathioprine 50 mg and prednisone 20 mg. Intraoral examination showed fair oral hygiene multiple fixed prosthodontic restorations and multiple carious teeth, multiple remaining roots and lipomatous tongue atrophy. The patient was referred to her neurologist to provide a detailed history about her medical condition and to coordinate with him regarding the steroid dose take to avoid any complication during patient management. A consultation letter had been received from her neurologist explaining the patient medical condition which starts with difficulty in breathing, chewing and swallowing, there was no external ocular weakness, motor weakness, sphincter dysfunction, sensory deficits, muscle wasting and no signs of diplopia, dizziness, double vision, ptosis or any difficulty in maintaining balance. Patient underwent chest CT scan with contrast which show evidence of thymoma benign tumor of the thymus that has been the possible causes of MG. Diagnosis was confirmed with measuring serum level of acetylcholine receptor antibodies, later treatment plan had been formulated including drugs to control the condition and then thymectomy to avoid sever complication to the patient. Before proceeding to the dental management of the case; CBC and blood chemistry had been requested form the patient. Creatinine level was below normal (42.23 umol/l), with normal levels of albumin, urea, uric acid, AST, ALT, cholesterol, triglycerides and bilirubin. Patient had elevated WBC “12.52 x 10 9 /L” and Platelet “453 x 10 9 /L” with reduction in RBC “4.4 x 10 9 /L” and Eosinophil count “0.07”. Consultation with patient neurologist regarding the steroid dose to reduce the susceptibility of developing adrenal crisis.

EN100437

The left adnexae presented with a tender, smooth and mobile mass of seven centimeters diameter but the right one were normal.

It is the case of a 29 years old woman, G2P1001, married, at 9 weeks of pregnancy. She consulted at our emergency service for the exacerbation of a left pelvic pain that has been evolving for two weeks prior to admission. A transient lull was observed following a treatment with a progesteron (hydroxyprogesterone caproate) and an antispasmodic (tiemonium methylsulfate) in a community clinic. The resurgence of that lancinating and permanent pain irradiating to the loins and associated with slight vaginal bleeding prompted consultation. She had first menses at 14 and her menstrual cycle is regular with a length of 30 days. She has never practiced contraception. She has been adequately treated for acute pelvic inflammatory disease five years earlier. Her only child is a girl born vaginally seven years ago. Her blood group is B rhesus positive and she has never undergone surgery. She presented with sympathetic signs of pregnancy and the urinary pregnancy test was positive but echography was not yet done. On admission, besides the main complain, the patient had vaginal bleeding and nausea but neither fever, nor vomiting. On physical examination her general condition was good and the blood pressure was 110/70 millimeters of mercury, the respiratory rate: 20 cycles / minute; the pulse rate: 70 pulsations/minute and the temperature: 37.3 degree Celsius. The conjunctivae were pink and the tongue was clean and moist. There were no cervical adenopathies. The breast and cardiopulmonary examination revealed no abnormalities. The abdomen was flat, and mobile with respiration. On palpation there was just a tenderness of the left iliac fossa and the bowel sounds were normal on auscultation. Inspection under speculum revealed normal gravid external cervical os and there was no vaginal discharge. On digital exploration, the cervix was posterior long and closed, the uterus was globular, increased in size and compatible with an eight weeks pregnancy. The left adnexae presented with a tender, smooth and mobile mass of seven centimeters diameter but the right one were normal. The posterior cervico-vaginal fornix was neither tender nor bulging. We suspected a non ruptured extra-uterine pregnancy with the following differentials: heterotopic pregnancy, torsion of ovarian cyst in pregnancy, and intra-cystic ovarian bleeding in pregnancy. Paraclinical investigations revealed: beta human Chorionic Gonadotropin (β hCG) level of 96 702 milli International Units per milliliter of plasma (mIU/ml). Ultrasonography revealed a heterogenous left ovarian mass of 82 millimeters in diameter, an empty uterus and no peritoneal effusion. This comforted our first diagnosis. After a normal pre-operative work up, an emergency laparotomy was done under general anesthesia. The findings were: left ovary containing a gestational sac and the corpus luteum, normal right adnexae, normal left tube and normal uterus. There was no hemoperitoneum. We dissected the ovarian capsule and carried out ablation of the gestational sac, then we did hemostasis. The specimen was analysed by the pathologist who found decidual cells and trophoblastic tissue within the ovarian capsule and thus confirmed the ovarian pregnancy. Post operative course was uneventful and the patient was discharged six days after surgery. The ?hCG level decreased and disappeared 25 days after surgery.

EN100068

Human immunodeficiency virus and diabetic screen were also negative.

A 23 year old female patient (weight 67kg, height 171cm, BMI 22.91kg/cm 2) presented to emergency department with fever (39.4oC), tachycardia (116 bpm), hypotension (95/45 mmHg), tachypnoea (25 breaths/min), sore throat, neck pain, diffuse pain and tissue oedema in the right-side gluteal region. She was appointed to hospital by a private outpatient surgical center where she had undergone perineal abscess drainage a week before. Recent medical history included also endometrial curettage for ERPC a month before and conservative therapy for hemorrhoids 15 days after. Past medical history included Henoch-Schönlein purpura during childhood. Social history included smoking (11 pack-years), family history was non-contributory. She had no known drug allergies. Complete blood count revealed marked neutrophilic leukocytosis (WBC 23.400 /lt). A CT scan revealed small bilateral pleural effusions, small hepatic enlargement, Gerota's fascia thickening, perineal soft tissue stranding with fascial thickening and small bubbles of air. Fournier's gangrene was set as working diagnosis (calculated FGSI 8, UFGSI 10 and LRINEC 7) and the patient was transported to operation room for immediate radical surgical debridement of necrotic tissue. Blood and surgical material was taken for microbiologic analysis. Due to the extent of the surgical debridement, the patient was admitted postoperatively to the ICU (SOFA score 6, APACHE II score 12); where sedation (midazolam 30 mcg/kg/hr c.iv), analgesia (fentanyl 3 mcg/kg/min c.iv), triple antibiotic regimen with meropenem 2gr q8h i.v., clindamycin 600mg q 6h i.v. and daptomycin 500mg q.d. i.v., gastroprotective and antithrombotic prophylaxis were initiated. During the next 3 days a second CT and an obstetrical consultation were conducted with no significant findings. Human immunodeficiency virus and diabetic screen were also negative. Yet, the patient remained febrile. Microbiology cultures from material taken from the surgical trauma area revealed Candida albicans, Staphylococcus epidermidis and Klebsiella pneumonia ; hence antibiotic regimen was modified and antimycotic treatment (caspofungin 100mg q.d. i.v.) was initiated too. Debridements were repeated every 24 hours but due to the extent of the lesions, on the 10 th day, the patient was transported to operation room for temporary ileostomy till the end of the therapy. Percutaneous tracheotomy was also performed. Hyperbaric oxygen therapy was not available at the setting. One day after the second surgery, patient's condition complicated with MRSA ventilation associated pneumonia. Along with that, a localized oedema appeared in her left upper limb. A palpable mass along the anterior margin of the sternocleidomastoid muscle was also noticed. Vascular ultrasound examination revealed occlusion of left internal jugular vein. Transcranial Doppler examination was normal. Dental consultation and examination of soft palate and peritonsilar tissues showed no abnormalities. Linezolide 600 mg q12h i.v. was added to antibiotic regimen. Since debridements were going on, simultaneous anticoagulation therapy was started with only bemiparine 2500 b.i.d. s.c. Gradually, the patient became afebrile and her clinical condition improved. She was discharged from ICU 13 days later. A follow-up CTA confirmed the disappearance of the left jugular vein thrombosis. About a month after her ICU discharge, an operation was performed for ileostomy closure and reconstruction of the right gluteal region. On a follow-up examination 6 months later, the young woman had returned to her normal everyday life.

EN100123

He was born by means of normal vaginal delivery with a normal extra-uterine adaptation.

We report a case of a male near-term neonate with gestational age of 35 weeks. He was the third born child to a 27-year-old mother with no significant past history. He was derived from a third degree consanguineous marriage. He was born by means of normal vaginal delivery with a normal extra-uterine adaptation. Meconium-stained amniotic fluid was noted. His birth weight was 3030g (75 th Percentile). The baby had normal physical examination at birth aside transitory respiratory distress. He had been hospitalized for four days in a private clinic, for the suspicion of materno-foetal infection, where he had antibiotics for 48 hours. He had developed no symptoms during his hospitalization. The output's weight was 2400g which signify the loss of 630g. Back home, he received exclusive maternal breastfeeding. On his fifth day of life, he presented multiple yellowish-watery stools (ab 12 times) and refuse feeding with apyrexia context. He had clinical signs of severe dehydration without fever or vomiting. He lost 930g relative to the birth's weight. The abdomen appeared neither tender nor distended, and the rest of his physical examination was normal. The laboratory findings revealed hypernatremia of 152mEq/L, hyperkalemia of 7,8mEq/L and severe metabolic acidosis: blood PH: 7,1, HCO3:4mEq/L. The osmotic gap was 45. There was no leukocytosis, and the serum level of C-reactive protein was normal. The infant was treated with intravenous fluids and electrolytes as well as antibiotics after obtaining blood and stool cultures, assuming an infectious etiology. After 26 hours of rehydration therapy, the dehydration resolved, however, a functional acute renal failure complicated by acidosis and hyperkalemia appeared. The chest radiograph showed "bat wings" pattern and the cardiothoracic index was 0, 45. The renal ultrasound showed discretely hyperechoic kidneys cortex and the absence of renal malformations. On the 11 th day of life, he was transferred to the pediatric intensive care unit. He needed mechanical ventilation for three days and exclusive parenteral feeding by central catheter. The evolution was burdened by cholestasis due to the prolonged parenteral feeding and nosocomial infection by staphylococcus epidermidis. Since his admission, he had developed a slimy diarrhea with 3 to 4 stools per day. He presented a profuse diarrhea during each attempt to introduce enteral feeding by hydrolyzed milk formula. He was discharged after 32 days with output's weight 3250g. The oesophago-gastro-duodenoscopy and duodenal biopsy showed partial villous atrophy associated to the brush border abnormalities. The periodic acid Schiff (PAS) stain revealed abnormalities in the brush border characterized by loss of the linear part of enterocytes with the presence of intra-cytoplasmic PAS + band in the apical pole of enterocytes uneven thickness and sometimes with double contours pattern. In the submucosa, there were numerous Brunner glands. We find the existence of numerous hemorrhagic suffusions. The CD10 immunostain was positive. Thus, a diagnosis hypothesis of MVID was raised. The electron microscopic examination confirmed the diagnosis. Mutation analysis of peripheral blood samples of the neonate revealed a mutation at the homozygous status of MYO5B gene. The baby died, after 97 days of life, of septic shock and multiple organ failure.

EN100376

Under spinal anesthesia, a tourniquet was applied to right lower extremity.

A seventy two years old male presented to our clinic with swelling, pain, and limitation of movement at knee joint. The patient's history revealed swellings and pain at knee joint for nearly last 20 years. He was prescribed nonsteroidal anti-inflammatory drugs and rest. But pain didn't relieve and limitations in range of motion occurred at knee joint. Physical examination findings included a solid and semi-mobile mass at anterior knee region. Knee joint range of motion was 80 degrees flexion and 0 degrees extension. There wasn't any neurovascular pathology. Antero-posterior and lateral knee X-rays showed degenerative changes and a giant, irregular, calcific mass which started below patella and extended to suprapatellar area. Knee MRI revealed many loose bodies (consistent with synovial chondromatosis) isointense with fatty bone marrow at all sequences at the level of right knee joint. Scalloping was observed at tibial and femoral surfaces. Decision for a surgical intervention was made and after informing the patient, consent was taken. Under spinal anesthesia, a tourniquet was applied to right lower extremity. Right knee was entered with an anterior longitudinal incision. Cutaneous and subcutaneous tissues were passed. Quadriceps mechanism was opened with a medial parapatellar incision and patella was toppled to left side. Intraarticular mass was exposed. The mass was released from surrounding tissues by blunt excision and excised with enucleation. Then synovial tissue was excised with synoviectomy. Surgical area was irrigated using abundant isotonic solution. The excised mass was sent to pathology laboratory. Pathology evaluation showed that the mass was consistent with synovial chondromatosis. Active and passive exercises were started at an early period after surgery. Recurrence was not detected at his last control visit. Knee movements were at full range when compared with the other knee. There was no pain or other complaints.

EN100247

In the absence of arterial embolization, she was going with ambulatory monitoring.

Mrs B.S. 28 years old multiparous that we had received 02 hours after a vaginal delivery of a newborn alive weighing 2750 grams in a peripheral maternity 3 km away. It was referred for postpartum hemorrhage. His medical history was featureless. On admission, she presented hypovolemic shock with blood pressure 76/44 mmHg, regular tachycardia at 136 beats per minute, polypnea to 39 cycles per minute. The obstetrical examination objectified an involuted uterus, an absence of vaginal and perineal cervical lesions, a fluid and red blood originating of endouterine. Furthermore, there was purpura. We therefore concluded a significant postpartum hemorrhage complicated by coagulopathy. Resuscitation measures were taken and an transfusion 1120 ml whole blood, red cell pellets 350 ml and 860 ml fresh frozen plasma have helped to management coagulopathy and anemia. Blood count at admission highlighted severe thrombocytopenia 23 0000 platelets and hemoglobin levels of 3.9 g/dl. The post natal care was also administered. After seven days of hospitalization, she was going after an amendment to the clinical symptoms and normal laboratory tests. Twenty five days later, she returned to obstetric emergencies for significant bleeding with severe anemia with hemoglobin 9 g/dL without signs of hypovolemic shock. The gynecological examination was normal. The balance sheet of the coagulation was normal including prothrombin to 92% and a normal activated partial thromboplastin time. The treatments were based on oxytocin, tranexamic acid, etamsylate and strict regular monitoring. Transvaginal ultrasound coupled Doppler was normal. In the absence of arterial embolization, she was going with ambulatory monitoring. Two weeks later she presented again rebleeding requiring hospitalization resulting in a total hysterectomy after conditioning of the patient. Histological examination of the surgical specimen objectified in the cervix and uterine body the presence of arterial vessels proliferation homes large and small caliber associated with tissue hemorrhage. The postoperative course was simple.

EN100340

Partially parenteral nutrition (PN) enriched with branched-chain amino acid (BCAA) was administered to support protein needs.

A 35-year-old mentally ill man was transferred from a local psychiatric hospital after attempted suicide by fire 3 days before admission. He acquired full-and partial-thickness injury in approximately 38% of his TBSA, including face and neck (5%), both upper extremities (8%), right thigh (4%), left thigh and leg (3%), and most of his anterior and posterior trunk (16%). The patient was hospitalized for 66 days and underwent five debridement surgeries. The psychiatric diagnosis was schizophrenia type ICD 10 F20.3, treated with Haloperidol 1.5 mg and Diazepam 5 mg daily. The patient was occasionally restrained due to rebellious and self-destruction behavior. The nutrition management of this patient was started after a week of hospitalization, which is considered late. In the initial nutrition assessment, the patient was 40 kg weight and 150 cm tall thus the body mass index (BMI) was 17,57 kg/m 2 or underweight. Gastrointestinal symptoms and signs were not found. Information regarding the patient's dietary intake in the last two weeks and weight change within the last 6 months could not be obtained, but his mother claimed that the patient seemed to lose weight because he refused any food that had been offered to him; he only smoked and drank coffee. Albumin level was 3.6 gr/L with imbalanced electrolyte levels. Signs of fluid accumulation were not found. According to the subjective global assessment (SGA) tool, the patient belonged to group C (severely malnourished). Energy and protein were given starting from 30 kcal/kg, increasing gradually to 50 kcal/kg, and 2 gp/kg divided into oral nutrition and oral nutrition supplements (ONS) with a 50:50 ratio. The patient was also given micronutrient supplementations, specifically vitamin A 100,000 units for the first week, vitamin C 100 mg 3 times a day, and zinc 20 mg once a day. During the early weeks of hospitalization, the patient's weight loss continued. At the end of the second week, his body weight was 36 kilograms while his serum albumin level was 2.7 gr/L. On the 28 th day, the patient's body weight was 33 kilograms while his serum albumin level was 2.1 gr/L. Energy and protein intake was maintained, but the ratio of oral intake and ONS was modified to 70:30. During the treatment, the patient's appetite was unstable. The patient was also hardly cooperative with enteral access. The patient often writhed in pain and became more aggressive. The patient did not respond well to analgesics and sedations. Partially parenteral nutrition (PN) enriched with branched-chain amino acid (BCAA) was administered to support protein needs. However, his BMI continued to decline, and on the 42 nd day, his body weight dropped to 32 kilograms with a serum albumin level of 2 gr/L. On the 66 th day, granulation tissues appeared on the wound bed. Considering the mental and nutrition status of the patient, it was decided not to continue with the skin graft surgery. The patient had lost 24% of body weight (BMI 11.96 kg/m 2) and the serum albumin level was 2.5 gr/L. The patient displayed severe muscle wasting and subcutaneous fat loss. However, the general and mental condition was relatively better. The patient was discharged from the hospital.

EN100658

On palpation there was just a tenderness of the left iliac fossa and the bowel sounds were normal on auscultation.

It is the case of a 29 years old woman, G2P1001, married, at 9 weeks of pregnancy. She consulted at our emergency service for the exacerbation of a left pelvic pain that has been evolving for two weeks prior to admission. A transient lull was observed following a treatment with a progesteron (hydroxyprogesterone caproate) and an antispasmodic (tiemonium methylsulfate) in a community clinic. The resurgence of that lancinating and permanent pain irradiating to the loins and associated with slight vaginal bleeding prompted consultation. She had first menses at 14 and her menstrual cycle is regular with a length of 30 days. She has never practiced contraception. She has been adequately treated for acute pelvic inflammatory disease five years earlier. Her only child is a girl born vaginally seven years ago. Her blood group is B rhesus positive and she has never undergone surgery. She presented with sympathetic signs of pregnancy and the urinary pregnancy test was positive but echography was not yet done. On admission, besides the main complain, the patient had vaginal bleeding and nausea but neither fever, nor vomiting. On physical examination her general condition was good and the blood pressure was 110/70 millimeters of mercury, the respiratory rate: 20 cycles / minute; the pulse rate: 70 pulsations/minute and the temperature: 37.3 degree Celsius. The conjunctivae were pink and the tongue was clean and moist. There were no cervical adenopathies. The breast and cardiopulmonary examination revealed no abnormalities. The abdomen was flat, and mobile with respiration. On palpation there was just a tenderness of the left iliac fossa and the bowel sounds were normal on auscultation. Inspection under speculum revealed normal gravid external cervical os and there was no vaginal discharge. On digital exploration, the cervix was posterior long and closed, the uterus was globular, increased in size and compatible with an eight weeks pregnancy. The left adnexae presented with a tender, smooth and mobile mass of seven centimeters diameter but the right one were normal. The posterior cervico-vaginal fornix was neither tender nor bulging. We suspected a non ruptured extra-uterine pregnancy with the following differentials: heterotopic pregnancy, torsion of ovarian cyst in pregnancy, and intra-cystic ovarian bleeding in pregnancy. Paraclinical investigations revealed: beta human Chorionic Gonadotropin (β hCG) level of 96 702 milli International Units per milliliter of plasma (mIU/ml). Ultrasonography revealed a heterogenous left ovarian mass of 82 millimeters in diameter, an empty uterus and no peritoneal effusion. This comforted our first diagnosis. After a normal pre-operative work up, an emergency laparotomy was done under general anesthesia. The findings were: left ovary containing a gestational sac and the corpus luteum, normal right adnexae, normal left tube and normal uterus. There was no hemoperitoneum. We dissected the ovarian capsule and carried out ablation of the gestational sac, then we did hemostasis. The specimen was analysed by the pathologist who found decidual cells and trophoblastic tissue within the ovarian capsule and thus confirmed the ovarian pregnancy. Post operative course was uneventful and the patient was discharged six days after surgery. The ?hCG level decreased and disappeared 25 days after surgery.

EN100068

Extension assessment, comprising a PET SCAN, was negative.

Mr F.B a 70-year-old male patient presented in our institution because of the occurrence of a right epistaxis. First of all, the patient is not smoking and reported an aneurysm of the abdominal aorta surgically treated by prosthesis under plavix. The beginning of the symptomatology goes back to 2 months before with right nasal obstruction of progressive onset, associated with right epistaxis and bloody rhinorrhea with right chronic eye watering. Nasal Endoscopy examination revealed a pinnkish burgeoning mass bleeding on contact and filling the right nostril vestibule. The patient didn't presents neurological signs nor cervical lymph nods. Otherwise, ophthalmologic examination found normal visual acuity and ocular mobility on both sides. Sinonasal computed tomography (CT) found a right nasal process enhanced after injection of contrast product with relatively little washing at the late time. Otherwise, there is no extension involvement at the level of the cavum and the pterygo palate fossa. This finding found also a crenate appearance of the posterior nasal mucosa especially of the middle turbinates and contralateral inferior turbinate with a left deviation of the nasal septum with nasal spur. Nasosinusal MRI shows a progressive increase in size of the tissue mass compared to the previously CT occupying the right nasal fossa on almost all of its height, measuring 4 × 2.8 × 3.5cm without extension within the cavum with probable invasion of the lower part of the nasolacrimal duct and mass effect on both the sinusonasal bone septum and the intersinusonasal septum. This finding shows ethmoidal sinus fluid retention. The appearance, even if it remains unspecific, is compatible with inverted papilloma. The patient underwent complete endoscopic removal of the tumor with ethmoidectomy and right maxillary antrostomy. The extemporaneous examination is in favor of a malignant tumor lesion. Histopathologic analysis shows a tumor proliferation made of polymorphic cells with major anisocytosis, most often located in lobules. There is sometimes a somewhat palisadic seat on the periphery of the tumor mass. The mitotic index is 10 mitoses per 10 high power fields. The immunohistochemical study of the tumor found that the cytokeratin KL1 and CK7 and the neuroendocrine marker Chromogranin A, synaptophysin and CD56 are positive. Proliferation index Ki67 is of the order of 80%. Otherwise there is an absence of expression of PS100, MelanA, HMB45, desmin, p40, CD45 and EBV. The clear positivity of cytokeratins formally excludes the diagnosis of olfactory neuroblastoma and leads to neuroendocrine carcinoma. Given the atypia, the mitotic index, and the Ki67 proliferation index, it is a high-grade large-cell neuroendocrine carcinoma. Extension assessment, comprising a PET SCAN, was negative. Postoperative treatment consisted in adjuvant polychemotherapy, with six cycles (cisplatin and etoposide), Followed by loco regional external radiotherapy. After 6 month follow-up, the patient was in complete clinical and radiological remission.

EN100383

The goiter measured 18 x 11 cm.

A 50-years-old woman, hypertensive, hospitalized for a large cervical mass appeared 30 years ago. In the family history, her mother, sisters and cousins underwent a surgery for MNG. Despite of the large volume of the mass, the patient never described signs of cervical compression whatsoever respiratory, digestive, laryngeal, vascular or neurologic signs. She never suffered from thyroid dysfunction. Her neck was deformed by the voluminous formation classified grade III according to the WHO modified classification. The mass took the front and the two sides of the neck. Its surface was embossed and covered by a thin normal skin. There were some veins of the collateral circulation limited to the neck. The goiter measured 18 x 11 cm. The mass was firm, painless, and mobile with the swallowing movements. Lymphadenopathy research was difficult and found no palpable lymph nodes. The laboratory tests (T 3, T 4 and TSH) were normal. Thoracic radiography showed a large cervical opacity roughly round and strewn with microcalcifications associated with a right eccentricity of the trachea. Cervical and chest CT revealed the presence of a partially calcified thyroid mass slightly plunging in the anterior mediastinum. It took heterogeneously the contrast and then evocate a large MNG. The trachea was surrounded by the goiter, slightly narrowed and right deviated as well as the lower part of the larynx. The right and left vascular axes of the neck (carotid artery and jugular vein) were deviated backward. The patient underwent a surgery for her enormous MNG slightly plunging in the mediastinum. Endotracheal intubation was relatively easy by the laryngoscope. The incision performed was a Kocher cervicotomy. There was a multinodular, hypervascularized goiter. Its lower end plunges behind the sternal manubrium. The larynx was deviated towards the right side. The total thyroidectomy was performed in two steps: initially a right lobo-isthmectomy, then the left lobectomy. The retrosternal part of the goiter was released using the finger by the same incision. Both recurrent laryngeal nerves (RLN) were not identified because of the hemorrhage. One parathyroid gland was accidently devascularized and was autotransplanted to the ipsilateral sternocleidomastoid muscle. The operation was finished by double aspiration drainage. In the first hours after surgery, the patient developed a large cervical hematoma. She was readmitted to the operating room, and after evacuation of the hematoma there was no vessels bleeding. The operation was completed with a double suction drainage. In the immediate postoperative period, the patient developed hemodynamic collapse requiring the introduction of dobutamine. After 48 hours of hemodynamic support, the blood pressure stabilized and dobutamine was stopped. Histological study concluded in multinodular colloid goiter. The patient was discharged from the hospital after 20 days in good health.

EN100067

A 19-year-old healthy man was admitted to our cardiology department following a syncopal episode.

A 19-year-old healthy man was admitted to our cardiology department following a syncopal episode. His past medical history was unremarkable. He no family history of heart disease or sudden death. Two-days prior the syncope, he developed chest pain and an influenza-like illness consisting of fevers, rhinorrhea, and sore throat. At the physical exam, the patient was febrile at 39°C. He had an inflamed throat, bradycardia at cardiac auscultation and there were no signs of heart failure. The electrocardiogram (EKG) showed grade III atrioventricular (AV) bloc with a ventricular rate of 38 beats per minute. Laboratory studies were remarkable for a HS-troponin level 181ng/L, a white blood cell count of 14270/mm³ with 48% lymphocytes, and elevated C-reactive protein at 90mg/L. Serum electrolytes, kidney and liver functions were within normal limits. A chest X-ray showed an appropriate cardiac size. The initial transthoracic echocardiography (TTE) showed a normal global left ventricular function (left ventricular ejection fraction (LVEF) 60%) and wall motion abnormalities (anteroseptal hypokinesia), with reduced left ventricular global longitudinal strain (LV GLS= -14.1%). Cardiac magnetic resonance (CMR) showed increased signal intensity at the mid-lateral wall on T2-weighted images. Late enhancement revealed a patchy subepicardial hyperenhancement of the medio-distal segments of the anterior and anterolateral walls which confirm the diagnosis of myocarditis. The patient was treated with cefotaxime and doxycycline. All serological tests (chlamydia, coxiella, mycoplasma, rickettsia, lyme and bartonella) obtained during the hospitalization were negative. Five days later, the patient was asymptomatic. The 24 hours ECG monitoring showed a sinus rhythm. The TTE revealed a normal LVEF and an improvement in the LVGLS with segmental reduced values concordant with the CMR abnormalities. At one-month follow-up the patient made a full recovery without any recurring cardiovascular symptoms or abnormal electrocardiographic or echocardiographic findings.

EN100682

On examination his vitals were-pulse-108/minute, arterial BP-130/80 mm of Hg, respiratory rate of 30 breaths/minute, Sp02-899% on room air, temperature- 101-degree centigrade.

A 43-year-old non-diabetic Indian male reported to our outpatient department with chief complaints of cough with expectoration, chest pain, reduced appetite, fever with chills, and night sweats for two weeks. He also complained of breathlessness on exertion and had two episodes of blood in his sputum. The patient explained that the cough was continuous and was relieved after taking cough syrup. He also mentioned that the episodes of fever were initially intermittent and then daily for the last two weeks and were relieved after taking Paracetamol. The chest pain was localized to the middle of the chest and was aggravated on exertion. He was a businessman by profession with no history of smoking, alcoholism, or any other substance abuse. Also, there was no history of any contact of TB or COVID-19 in the family or close contacts. And there was no history of foreign travel in the recent past. But he had reported having traveled by a domestic airline about twenty days back. There was no history of weight loss or any other major illness in the past. On examination his vitals were-pulse-108/minute, arterial BP-130/80 mm of Hg, respiratory rate of 30 breaths/minute, Sp02-899% on room air, temperature- 101-degree centigrade. His Sp02 fell by 70% on room air after waking. On auscultation, there was crepitation on the bilateral middle lobes of the lungs. Also, dyspnea on exertion was noted. The rest of the systemic examination was within normal limits. Considering this as a probable case of TB with COVID-19 he was advised a chest radiograph with sputum microscopy (Ziehl Neelsen (ZN) staining for acid-fast bacilli), Cartridge-based nucleic acid amplification test (CBNAAT) of the sputum and other routine investigations. To check for the COVID-19 he was advised qualitative polymerase chain reaction (PCR) test from the oropharyngeal swab. The results were surprising with Mycobacterium tuberculosis detected on sputum fluorescent microscopy and were also confirmed by the CBNAAT. However, there was no resistance to Rifampicin. The results of the PCR were positive for RNA specific to SARS-CoV-2. Besides, the chest radiograph PA-view was suggestive of bilateral consolidations on the middle lobes of lungs with ill-defined borders. The other investigations revealed a low lymphocyte count (1x 10 9 /L) and increased levels of C-reactive protein (CRP) (57 mg/L), lactate dehydrogenase (LDH) (580 U/L), and erythrocyte sedimentation rate (ESR) (70 mm in the 1 st hour). Also, a sample for liquid culture (MGIT BACTEC) was sent to the Intermediate Reference Laboratory (IRL) which revealed the growth of Mycobacterium tuberculosis. Computed tomography was not performed as the diagnosis was established by other cheaper and faster methods and also the patient was unwilling for the same. All the other routine investigations were within normal limits. He was referred to the nearest designated COVID-19 management center, where he was managed as per national guidelines. Besides, he was also started on an antitubercular treatment of four drugs as per the National Tuberculosis Elimination Program (NTEP) guidelines. He was advised follow-up post completion of his stay at the designated COVID-19 center, but he has not yet reported back for follow-up. Written informed consent was obtained from the patient for using clinical data and images for publication in this study.

EN100606

The patient was hospitalized for 66 days and underwent five debridement surgeries.

A 35-year-old mentally ill man was transferred from a local psychiatric hospital after attempted suicide by fire 3 days before admission. He acquired full-and partial-thickness injury in approximately 38% of his TBSA, including face and neck (5%), both upper extremities (8%), right thigh (4%), left thigh and leg (3%), and most of his anterior and posterior trunk (16%). The patient was hospitalized for 66 days and underwent five debridement surgeries. The psychiatric diagnosis was schizophrenia type ICD 10 F20.3, treated with Haloperidol 1.5 mg and Diazepam 5 mg daily. The patient was occasionally restrained due to rebellious and self-destruction behavior. The nutrition management of this patient was started after a week of hospitalization, which is considered late. In the initial nutrition assessment, the patient was 40 kg weight and 150 cm tall thus the body mass index (BMI) was 17,57 kg/m 2 or underweight. Gastrointestinal symptoms and signs were not found. Information regarding the patient's dietary intake in the last two weeks and weight change within the last 6 months could not be obtained, but his mother claimed that the patient seemed to lose weight because he refused any food that had been offered to him; he only smoked and drank coffee. Albumin level was 3.6 gr/L with imbalanced electrolyte levels. Signs of fluid accumulation were not found. According to the subjective global assessment (SGA) tool, the patient belonged to group C (severely malnourished). Energy and protein were given starting from 30 kcal/kg, increasing gradually to 50 kcal/kg, and 2 gp/kg divided into oral nutrition and oral nutrition supplements (ONS) with a 50:50 ratio. The patient was also given micronutrient supplementations, specifically vitamin A 100,000 units for the first week, vitamin C 100 mg 3 times a day, and zinc 20 mg once a day. During the early weeks of hospitalization, the patient's weight loss continued. At the end of the second week, his body weight was 36 kilograms while his serum albumin level was 2.7 gr/L. On the 28 th day, the patient's body weight was 33 kilograms while his serum albumin level was 2.1 gr/L. Energy and protein intake was maintained, but the ratio of oral intake and ONS was modified to 70:30. During the treatment, the patient's appetite was unstable. The patient was also hardly cooperative with enteral access. The patient often writhed in pain and became more aggressive. The patient did not respond well to analgesics and sedations. Partially parenteral nutrition (PN) enriched with branched-chain amino acid (BCAA) was administered to support protein needs. However, his BMI continued to decline, and on the 42 nd day, his body weight dropped to 32 kilograms with a serum albumin level of 2 gr/L. On the 66 th day, granulation tissues appeared on the wound bed. Considering the mental and nutrition status of the patient, it was decided not to continue with the skin graft surgery. The patient had lost 24% of body weight (BMI 11.96 kg/m 2) and the serum albumin level was 2.5 gr/L. The patient displayed severe muscle wasting and subcutaneous fat loss. However, the general and mental condition was relatively better. The patient was discharged from the hospital.

EN100658

Gastrointestinal symptoms and signs were not found.

A 35-year-old mentally ill man was transferred from a local psychiatric hospital after attempted suicide by fire 3 days before admission. He acquired full-and partial-thickness injury in approximately 38% of his TBSA, including face and neck (5%), both upper extremities (8%), right thigh (4%), left thigh and leg (3%), and most of his anterior and posterior trunk (16%). The patient was hospitalized for 66 days and underwent five debridement surgeries. The psychiatric diagnosis was schizophrenia type ICD 10 F20.3, treated with Haloperidol 1.5 mg and Diazepam 5 mg daily. The patient was occasionally restrained due to rebellious and self-destruction behavior. The nutrition management of this patient was started after a week of hospitalization, which is considered late. In the initial nutrition assessment, the patient was 40 kg weight and 150 cm tall thus the body mass index (BMI) was 17,57 kg/m 2 or underweight. Gastrointestinal symptoms and signs were not found. Information regarding the patient's dietary intake in the last two weeks and weight change within the last 6 months could not be obtained, but his mother claimed that the patient seemed to lose weight because he refused any food that had been offered to him; he only smoked and drank coffee. Albumin level was 3.6 gr/L with imbalanced electrolyte levels. Signs of fluid accumulation were not found. According to the subjective global assessment (SGA) tool, the patient belonged to group C (severely malnourished). Energy and protein were given starting from 30 kcal/kg, increasing gradually to 50 kcal/kg, and 2 gp/kg divided into oral nutrition and oral nutrition supplements (ONS) with a 50:50 ratio. The patient was also given micronutrient supplementations, specifically vitamin A 100,000 units for the first week, vitamin C 100 mg 3 times a day, and zinc 20 mg once a day. During the early weeks of hospitalization, the patient's weight loss continued. At the end of the second week, his body weight was 36 kilograms while his serum albumin level was 2.7 gr/L. On the 28 th day, the patient's body weight was 33 kilograms while his serum albumin level was 2.1 gr/L. Energy and protein intake was maintained, but the ratio of oral intake and ONS was modified to 70:30. During the treatment, the patient's appetite was unstable. The patient was also hardly cooperative with enteral access. The patient often writhed in pain and became more aggressive. The patient did not respond well to analgesics and sedations. Partially parenteral nutrition (PN) enriched with branched-chain amino acid (BCAA) was administered to support protein needs. However, his BMI continued to decline, and on the 42 nd day, his body weight dropped to 32 kilograms with a serum albumin level of 2 gr/L. On the 66 th day, granulation tissues appeared on the wound bed. Considering the mental and nutrition status of the patient, it was decided not to continue with the skin graft surgery. The patient had lost 24% of body weight (BMI 11.96 kg/m 2) and the serum albumin level was 2.5 gr/L. The patient displayed severe muscle wasting and subcutaneous fat loss. However, the general and mental condition was relatively better. The patient was discharged from the hospital.

EN100658

The facial contour and masticatory function were well-preserved.

A 12-year-old girl was referred to Department of Oral Surgery, with a complaint of pain and swelling in the left of maxilla that appeared 3 months ago. The swelling was slowly progressive, associated with pain. However, the patient did not refer any motor or sensory deficit. There was no family history of similar swelling. Physical examination revealed a left maxillary swelling. There was no facial palsy. No cervical lymph node enlargement was seen. The oral examination showed a 4 cm x 5 cm, tender, compressible mass in the left maxilla, from the lateral incisor to the second premolar teeth. The tumor surface was smooth and red-purple. Several teeth were involved and displaced in the tumor mass. We noted the absence of canine. Vitality tests proved negative on the central and lateral incisor. Hight mobility of lateral incisor was noted. Orthopantomography and occlusal radiography showed a wide osteolytic area of the anterior left maxilla, extending from the first permanent molar to the central incisor. The canine was impacted. No root resorption was observed. The radiolucency was closely related to maxillary sinus and involving the nasal cavity. Haematological investigations showed normal serum calcium, phosphorus and parathormone (PTH) levels. The provisional diagnosis of benign tumor of the maxilla was made. The patient underwent excision and curettage of the mass with extraction of the canine and lateral incisor tooth. The wound was closed with interrupted sutures. The post-operative histopathological report revealed multinuclear giant cells scattered randomly throughout the cellular and fibrous vascular-rich tissue. New bone formation and granulation tissue rich in mononuclear inflammatory cells was revealed. The giant cells were multinucleated with bland-appearing nuclei, and the background stromal cells displayed no evidence of atypical mitoses. A diagnosis of giant cell tumor was established. During a 1-year serial clinical and radiological follow-up, there was no evidence of recurrence. The facial contour and masticatory function were well-preserved.

EN100600

We used in silico tools to evaluate the possible impact of p.W221S variant on protein structure and function.

We observed a 29 y.o. female proband with the episode of venous thrombosis at the age of 18 and family history of thrombosis. The antithrombin level in our patient was low, 44-48% (AT deficiency type I). A new genetic variant c.662G > C (p.W221S) in the SERPINC1 gene was detected in proband and affected father but was absent in healthy sister. We used in silico tools to evaluate the possible impact of p.W221S variant on protein structure and function. In mutated SERPINC1 protein a new N-linked glycosylation site is formed, however, it is unclear if the glycosylation at 219-221 site is possible.

EN101318

Hemogram showed mild anemia, normal platelet count and a leukocyte count of 1.2 x 10(11)/L.

A 45 year old Indian female of Nordic origin presented 5 years back with a lump in the right breast and the axilla. She underwent modified radical mastectomy. Histophotomicrograph of the excised breast lesion showed a 2.1 cm duct carcinoma, positive for ER and PR with 1 out of 25 lymph nodes positive for metastasis. She received 6 cycles of chemotherapy with cyclophosphamide, epirubicin, and 5-fluorouracil. This was followed by tamoxifen 20 mg per day for five years. She was doing well on follow up until the completion of fifth year of her disease, when she presented with complaints of mild fever and weakness. Examination revealed generalized lymph node enlargement along with hepatomegaly. Hemogram showed mild anemia, normal platelet count and a leukocyte count of 1.2 x 10(11)/L. Peripheral blood examination revealed medium sized lymphoid cells, constituting almost 75% of total nucleated cell population. Immunophenotying, established a diagnosis of post thymic T-cell prolymphocytic leukemia. Contrast-enhanced computed tomography of the chest and abdomen was done which revealed an anterior mediastinal mass with destruction of sternum along with multiple small nodular shadows in bilateral lung fields suggestive of lung metastasis. Fine needle aspiration cytology of the mass showed atypical ductal cells with nuclear pleomorphism, which were positive for ER, PR and Her2neu protein. This confirmed a co-existent metastatic breast carcinoma. She was started on chemotherapy for T-PLL along with hormonal therapy with aromatase inhibitor. Unfortunately, both her malignancies progressed after an initial stable disease of two months.

EN108139

A 29-year-old man was admitted to our hospital with an unidentified fever.

A 29-year-old man was admitted to our hospital with an unidentified fever. He was diagnosed with malignant lymphoma (extra-nodal NK/T cell lymphoma nasal type), and chemotherapy was administered. After chemotherapy, nodular lung shadows along with new brain, cervical, and myocardial abscesses appeared, despite anti-bacterial/fungal therapy. Gene analysis of the cervical abscess biopsy revealed the presence of Aspergillus fumigatus species, and the transesophageal echocardiogram showed a mobile mural vegetation in the left ventricle (22 × 8 mm). He underwent surgical resection of this mural vegetation. His postoperative course was uneventful. He remains healthy at 28 months after surgery with continued oral antifungal therapy.

EN101114

On a follow-up examination 6 months later, the young woman had returned to her normal everyday life.

A 23 year old female patient (weight 67kg, height 171cm, BMI 22.91kg/cm 2) presented to emergency department with fever (39.4oC), tachycardia (116 bpm), hypotension (95/45 mmHg), tachypnoea (25 breaths/min), sore throat, neck pain, diffuse pain and tissue oedema in the right-side gluteal region. She was appointed to hospital by a private outpatient surgical center where she had undergone perineal abscess drainage a week before. Recent medical history included also endometrial curettage for ERPC a month before and conservative therapy for hemorrhoids 15 days after. Past medical history included Henoch-Schönlein purpura during childhood. Social history included smoking (11 pack-years), family history was non-contributory. She had no known drug allergies. Complete blood count revealed marked neutrophilic leukocytosis (WBC 23.400 /lt). A CT scan revealed small bilateral pleural effusions, small hepatic enlargement, Gerota's fascia thickening, perineal soft tissue stranding with fascial thickening and small bubbles of air. Fournier's gangrene was set as working diagnosis (calculated FGSI 8, UFGSI 10 and LRINEC 7) and the patient was transported to operation room for immediate radical surgical debridement of necrotic tissue. Blood and surgical material was taken for microbiologic analysis. Due to the extent of the surgical debridement, the patient was admitted postoperatively to the ICU (SOFA score 6, APACHE II score 12); where sedation (midazolam 30 mcg/kg/hr c.iv), analgesia (fentanyl 3 mcg/kg/min c.iv), triple antibiotic regimen with meropenem 2gr q8h i.v., clindamycin 600mg q 6h i.v. and daptomycin 500mg q.d. i.v., gastroprotective and antithrombotic prophylaxis were initiated. During the next 3 days a second CT and an obstetrical consultation were conducted with no significant findings. Human immunodeficiency virus and diabetic screen were also negative. Yet, the patient remained febrile. Microbiology cultures from material taken from the surgical trauma area revealed Candida albicans, Staphylococcus epidermidis and Klebsiella pneumonia ; hence antibiotic regimen was modified and antimycotic treatment (caspofungin 100mg q.d. i.v.) was initiated too. Debridements were repeated every 24 hours but due to the extent of the lesions, on the 10 th day, the patient was transported to operation room for temporary ileostomy till the end of the therapy. Percutaneous tracheotomy was also performed. Hyperbaric oxygen therapy was not available at the setting. One day after the second surgery, patient's condition complicated with MRSA ventilation associated pneumonia. Along with that, a localized oedema appeared in her left upper limb. A palpable mass along the anterior margin of the sternocleidomastoid muscle was also noticed. Vascular ultrasound examination revealed occlusion of left internal jugular vein. Transcranial Doppler examination was normal. Dental consultation and examination of soft palate and peritonsilar tissues showed no abnormalities. Linezolide 600 mg q12h i.v. was added to antibiotic regimen. Since debridements were going on, simultaneous anticoagulation therapy was started with only bemiparine 2500 b.i.d. s.c. Gradually, the patient became afebrile and her clinical condition improved. She was discharged from ICU 13 days later. A follow-up CTA confirmed the disappearance of the left jugular vein thrombosis. About a month after her ICU discharge, an operation was performed for ileostomy closure and reconstruction of the right gluteal region. On a follow-up examination 6 months later, the young woman had returned to her normal everyday life.

EN100123

The patient was operated by performing a right extra-dural frontal approach, a cyst puncture was done in the first intention because the cyst was adherent to the neighbouring structures making its complete removal impossible, then a microscopic extirpation of the cystic membrane, combined with abundant washing by hypertonic serum to sterilize the cystic sit and reduce chances of dissemination.

A 3 years old girl born from non-consanguineous parents, without any neonatal suffering; with good psychomotor development, from a popular district in one of our cities, without any obvious contact with dogs; presented with exophthalmia associated with unilateral blindness evolving rapidly within 3 months. Clinical examination showed a non-pulsatile, painless, axial, irreducible exophthalmia with no sign of conjunctivitis or keratitis, and right monocular blindness, right ptosis; and fundal examination had objectified right papillary oedema. The rest of the clinical examination was normal. Brain and orbital magnetic resonance imaging (MRI) revealed an extra-connal right orbital lesion near the orbital apex, measuring 28 x 18mm, of oval shape, it appeared hypo-signal in T1 and hyper-signal in T2, limited by a thin wall which took contrast product, this lesion compressed the optic nerve towards the nasal region. Chest x-ray and abdominal ultrasound did not reveal any other localization. The patient was operated by performing a right extra-dural frontal approach, a cyst puncture was done in the first intention because the cyst was adherent to the neighbouring structures making its complete removal impossible, then a microscopic extirpation of the cystic membrane, combined with abundant washing by hypertonic serum to sterilize the cystic sit and reduce chances of dissemination. Histological examination was in favour of intra-orbital hydatidosis. Postoperatively, the patient was given an antihelminthic treatment of albendazole at a dose of 10mg per kilogram per day in 3 doses separated by 2 weeks; the evolution was marked by the significant regression of the exophthalmia and the gradual improvement of the visual acuity. The patient however still retains a right unilateral nasal hemianopia after two years of follow-up. An MRI done in the same period shows a cure of the patient by a complete disappearance of the cyst.

EN100453

He lost 930g relative to the birth's weight.

We report a case of a male near-term neonate with gestational age of 35 weeks. He was the third born child to a 27-year-old mother with no significant past history. He was derived from a third degree consanguineous marriage. He was born by means of normal vaginal delivery with a normal extra-uterine adaptation. Meconium-stained amniotic fluid was noted. His birth weight was 3030g (75 th Percentile). The baby had normal physical examination at birth aside transitory respiratory distress. He had been hospitalized for four days in a private clinic, for the suspicion of materno-foetal infection, where he had antibiotics for 48 hours. He had developed no symptoms during his hospitalization. The output's weight was 2400g which signify the loss of 630g. Back home, he received exclusive maternal breastfeeding. On his fifth day of life, he presented multiple yellowish-watery stools (ab 12 times) and refuse feeding with apyrexia context. He had clinical signs of severe dehydration without fever or vomiting. He lost 930g relative to the birth's weight. The abdomen appeared neither tender nor distended, and the rest of his physical examination was normal. The laboratory findings revealed hypernatremia of 152mEq/L, hyperkalemia of 7,8mEq/L and severe metabolic acidosis: blood PH: 7,1, HCO3:4mEq/L. The osmotic gap was 45. There was no leukocytosis, and the serum level of C-reactive protein was normal. The infant was treated with intravenous fluids and electrolytes as well as antibiotics after obtaining blood and stool cultures, assuming an infectious etiology. After 26 hours of rehydration therapy, the dehydration resolved, however, a functional acute renal failure complicated by acidosis and hyperkalemia appeared. The chest radiograph showed "bat wings" pattern and the cardiothoracic index was 0, 45. The renal ultrasound showed discretely hyperechoic kidneys cortex and the absence of renal malformations. On the 11 th day of life, he was transferred to the pediatric intensive care unit. He needed mechanical ventilation for three days and exclusive parenteral feeding by central catheter. The evolution was burdened by cholestasis due to the prolonged parenteral feeding and nosocomial infection by staphylococcus epidermidis. Since his admission, he had developed a slimy diarrhea with 3 to 4 stools per day. He presented a profuse diarrhea during each attempt to introduce enteral feeding by hydrolyzed milk formula. He was discharged after 32 days with output's weight 3250g. The oesophago-gastro-duodenoscopy and duodenal biopsy showed partial villous atrophy associated to the brush border abnormalities. The periodic acid Schiff (PAS) stain revealed abnormalities in the brush border characterized by loss of the linear part of enterocytes with the presence of intra-cytoplasmic PAS + band in the apical pole of enterocytes uneven thickness and sometimes with double contours pattern. In the submucosa, there were numerous Brunner glands. We find the existence of numerous hemorrhagic suffusions. The CD10 immunostain was positive. Thus, a diagnosis hypothesis of MVID was raised. The electron microscopic examination confirmed the diagnosis. Mutation analysis of peripheral blood samples of the neonate revealed a mutation at the homozygous status of MYO5B gene. The baby died, after 97 days of life, of septic shock and multiple organ failure.

EN100376

She was performed a proteus intelligence test in which she had 75 points and accepted as mildly mentally retarded.

A 21 year old female patient with the diagnosis of SWS suffering from headaches admitted to our clinic. She had a 2 year history of frequent non-pulsating headaches. Her headache was relieving with non-steroidal anti-inflammatory drugs and was not worsening with physical activity. There was no nausea or aura like symptoms accompanying the headache. Headaches were lasting for hours. The pain was bilateral, generalized and pressing in quality. The family history for headache was negative. She had a history of seizures occurring in the fifteenth day of life described as attacks of tonic clonic contractions and that's when she was diagnosed with SWS. At the age of 6 she had a history of callosotomy to control her seizures. At the age of 18 during a laser treatment done in order to get rid of her port wine birthmark she had her first seizure since callosotomy. After that she was prescribed carbamazepine 400 mg at daily dose and never had a seizure since then. According to the story taken from her parents even though she had a normal development at infancy she barely graduated from elementary school and she's hardly literate. There was nothing significant on her family history except for her elder sister's port wine stain on her face. The elder sister had no feature of SWS and no researches were done regarding her stain. She was inscribed daily doses of ketiapin 25 mg for anxiety disorder and venlafaxine 75 mg for both anxiety disorder and the chronic headaches. She was also inscribed NSAID drugs. After the first week of this treatment her headaches were slightly decreased by heaviness but the frequency was the same. At her physical examination a facial nevus -occurred due to choroid angioma-on the right forehead, right eyelid, nasal wing and the cheek was observed. Intra oral examination showed a right sided overgrowth of gingiva. Gingival overgrowth was bright red in color and showed blanching on applying pressure suggesting angiomatous enlargement. On her extremities a mild asymmetry was visible. Her left arm and leg was slightly smaller in portions and showed hemiparesis both in the upper and lower extremities of the same size. On her ophthalmological evaluation she was diagnosed with glaucoma of the right eye. On her psychiatric examination she showed signs of anxiety disorder. Her neurological examination was not remarkable except for her hemiparesis. Cranial CT scans showed diffuse atrophy in the right hemisphere and irregular double-contoured gyriform cortical calcifications in the right occipital area. Gadolinium enhanced brain MRI revealed multiple dilated pial venous vascular structures on right hemisphere also with the diffuse atrophy on the same side. Axial T1 weighted cranial MRI shows right calvarial thickness compared to the left and right hemisphere is asymmetrically smaller than the left. In addition to that, T2 weighted MRI shows extensive venous formations around corpus of right lateral ventricle and at Gallen vein localization and widespread vascular formations are seen at perivascular space, anterior to third ventricle at Willis polygon localization and at right temporooccipital area at quadrigeminal cistern localization. She was performed a proteus intelligence test in which she had 75 points and accepted as mildly mentally retarded. Proteus intelligence test in which she had 75 points and accepted as mildly mentally retarded.

EN100129

Hight mobility of lateral incisor was noted.

A 12-year-old girl was referred to Department of Oral Surgery, with a complaint of pain and swelling in the left of maxilla that appeared 3 months ago. The swelling was slowly progressive, associated with pain. However, the patient did not refer any motor or sensory deficit. There was no family history of similar swelling. Physical examination revealed a left maxillary swelling. There was no facial palsy. No cervical lymph node enlargement was seen. The oral examination showed a 4 cm x 5 cm, tender, compressible mass in the left maxilla, from the lateral incisor to the second premolar teeth. The tumor surface was smooth and red-purple. Several teeth were involved and displaced in the tumor mass. We noted the absence of canine. Vitality tests proved negative on the central and lateral incisor. Hight mobility of lateral incisor was noted. Orthopantomography and occlusal radiography showed a wide osteolytic area of the anterior left maxilla, extending from the first permanent molar to the central incisor. The canine was impacted. No root resorption was observed. The radiolucency was closely related to maxillary sinus and involving the nasal cavity. Haematological investigations showed normal serum calcium, phosphorus and parathormone (PTH) levels. The provisional diagnosis of benign tumor of the maxilla was made. The patient underwent excision and curettage of the mass with extraction of the canine and lateral incisor tooth. The wound was closed with interrupted sutures. The post-operative histopathological report revealed multinuclear giant cells scattered randomly throughout the cellular and fibrous vascular-rich tissue. New bone formation and granulation tissue rich in mononuclear inflammatory cells was revealed. The giant cells were multinucleated with bland-appearing nuclei, and the background stromal cells displayed no evidence of atypical mitoses. A diagnosis of giant cell tumor was established. During a 1-year serial clinical and radiological follow-up, there was no evidence of recurrence. The facial contour and masticatory function were well-preserved.

EN100600

The radiological results were satisfactory with a restored bilateral symmetry of the articular anatomy.

A 3 years old boy without any particular diseases history, admitted to the emergency room for a bipolar trauma with a left hemicorporal point of impact following a domestic accident. The delay to consult was 5 hours after the incident. The clinical examination at admission had showed a deformed left superior member, painful on palpation with the presence of the humeral pulse and a right inferior member shortened and flexed, the thigh slightly in adduction, internal rotation with the member. A radiological assessment was performed immediately; including a standard X-ray of the pelvis which showed a dislocated head of the right femur, completed secondarily by a CT Scan of the hip confirming the posterior type of the dislocation isolated from any fractures of the head of the femur. The standard radiography of the left shoulder showed a Salter I epiphyseal separation of the humerus. After one hour, we performed an orthopedic reduction of the dislocated hip monitored under scope, with placement of a pelvic splint for an analgesic aim to be kept during three weeks. The radiological results were satisfactory with a restored bilateral symmetry of the articular anatomy. The Salter I detachment of the humerus was orthopedically reduced then immobilized by a thoracoabdominal plaster be kept during a month. The child has been discharged the next day. Revised after a month, showed a stable left hip on the clinical examination. In the decline of 2 years, we haven't noticed any recidivism and the functional results were good; On the radiography we do not have any signs of avascular necrosis no changes of the head of femur, of the acetabulum or the joint space.

EN100315

Intraocular muscle testing was normal; however, there was an exophthalmia in both eyes.

A 12-year-old girl presented an abdominal pain, high persistent fever of (40°C) and severe fatigue. The physical exam revealed pallor, hepatomegaly, and grade III splenomegaly. The investigation revealed an elevated white blood cell count (WBC): 545,000/mm³. PNN: 49,050/mm³, hemoglobin: 11.6g/dl, platelets: 328,000/mm³. The patient's peripheral blood smear revealed marked leukocytosis with a significant number of immature myeloid precursors and 21% blasts. The analysis of the BCR-ABL shows 94% in the peripheral blood. The patient was diagnosed with CML. She received a chemotherapy protocol made of hyperalkalinisation, allopurinol, hydroxyurea and a treatment based on imatinib at the dose of 400mg per day. Patient achieved complete hematological remission at three months and complete cytogenetic remission at 12 months. The patient was readmitted into our pediatric department with complains of severe headache, vomiting, pain and bilateral visual loss since three days. On examination, patient was afebrile and appeared pale. Blood pressure was 100/60mm/hg and the heart rate 98 beat/minute. There was no lymphadenopathy and the nervous system examination was normal. Intraocular muscle testing was normal; however, there was an exophthalmia in both eyes. The pupil was sluggish to react to light. Fundoscopy revealed bilateral stade III disc edema and hemorrhages of the retina. She underwent a brain computed tomography (CT) scan with the suspicion of leukemic involvement or any other intracranial event (cerebral edema); however, nothing was found. A subsequent bone marrow exam showed no morphologic or molecular evidence of CML or acute leukemia. Cytological evaluation of cerebrospinal fluid (CSF) was also negative. The patient had no other known pathology that could be held responsible for retina edema. We suggested that it was a side effect of imatinib so its administration was temporary interrupted. The patient was put on an anti-edema therapy (mannitol, dexamethasone). However, few months later, the patient presented in the emergency room with a headache and severe vomiting. She described a recurrence of the same character of headaches accompanied with weakness of extremities and seizures. Nervous system examination revealed neck rigidity, presence of meningeal signs and lower limb paraplegia. CT of the brain revealed subdural chronic hematoma and a serpenginous gyriform enhancement around the contusion in the right parietal and occipital lobes. Analysis of the CSF collected showed lymphoblasts in a WBC count of 11000cells/ml. Flow cytometry of the CSF showed that blast cells were positive for a cluster of differentiation markers (CD) (CD34, CD19, CD10, CD22 and partially positive for CD45) confirming CNS extramedullary lymphoid blast infiltration. A bone marrow aspiration was done to evaluate the status of her chronic myelogenous leukemia and result was consistent with a chronic phase. Fundus examination showed bilateral optic atrophy. The diagnosis of extramedullary isolated CNS blast crises (lymphoid type) was based on the presence of blasts in CSF (confirmed by flow cytometry). The patient received a high-dose systemic induction chemotherapy and intrathecal therapy (methotrexate, arabinoside and dexamethasone). The imatinib was replaced by dasatinib at the dose of 100 mg per day. She was treated with 24 Gy of whole-brain radiation therapy. Allogenic stem cell transplantation was not feasible. We noticed a rapidly progressive amelioration in her neurological status after finishing systemic chemotherapy and physical therapy; however, the patient's visual loss showed no signs of improvement.

EN100543

On the other side the infectious process was good managed by perfusion of Antibiotics such as Ceftazidim in combination with aminoside.

We present here a case of Macrophage activation syndrome associated with Griscelli syndrome type 2 in a 3-years-old male born of consanguineous parentage. His birth histories, his familie social history and developmental milestones were unremarkable. He was born at full term with no antenatal or perinatal complications, He was on exclusively breast-fed, food diversification was started at 6 months old, his weight, length and psychomotor development were within the normal range, the child was described as a good eater, was on a normal diet and was thriving appropriately. Furthermore the boy presented a progressive abdominal distension since birth, progressive pallor and recurrent episodes of fever since 1 year of age. There was history of blood transfusions for last 2 months. On the other side there was no history of jaundice, vomiting, urinary or bowel complaints, bleeding from any site or neurological complaints. On admission, he was very pale with silvery gray scalp hair, white eyelashes, he was hypotonic, tachycardic, fever of 40°C, lymphoadenopathy and hepato-splenomegaly. The blood count showed pancytopenia; 1980 white blood cells/µL (VN 4000-13 500/µL), neutrophils 820/µL, 910 cells/µL, hemoglobin (Hb) 6.3 g/dL (11.5-14 VN, 5 g/dL), platelets 44 000/µL (VN 150 000-400 000/µL), There were no giant cytoplasmic granules in leucocytes. The liver function tests were normal expect low albumin (2.1 mg/dL) and increased alkaline phosphatase (1196 mg/dL) with low fibrinogen (1.7g/l). Serum triglycerides, ferrintin and lactic dehydrogenase were very high; respectively 4.75g/L; 2763µg/L and 597U/L. the C reactive protein (CRP) was elevated at 83mg/l. A chest X-ray was normal while the abdominal ultrasound showed a hepatomegaly and a splenomegaly, free biliary ducts without ascites. Because of hepato-splenomegaly, pancytopenia, hyperferritinemia and hypofibrinogenemia the diagnosis of Macrophage activation syndrome was evoked indicating a bone marrow aspiration that showed numerous morphologically benign macrophages with haemophagocytic activity that comforting the diagnosis of a SAM according to Ravelli and HLH-2004 criteria. Blood culture, urine examination, malarial serology, Kala Azar serology, viral serologies such as EBV, hepatitis A, B, C and human immunodeficiency virus HIV, serologies of rickettsia, syphilis and toxoplasmosa were negatives. Serologies of CMV and rubella showed an old immunization. The immunological tests such as antinuclear antibodies (ANA), anti-LKM1, anti-mitochondria and anti-smooth muscle antibodies were negatives. Because of the consanguineous family, notion of recurrent infection and the presence of silvery-gray color of the hair and eyebrows, diagnosis of Griscelli Syndrome was evoked directing a light microscopy examination of the hair that showed a large, irregular clumps of pigments characteristic of Griscelli Syndrome. The molecular biology showed mutation in RAB27A gene confirming the diagnosis of a Griscelli syndrome type 2. The first-line therapy in management of Macrophage activation syndrome complicating Griscelli syndrome type 2 was based on the parenteral administration of high doses of corticosteroids (methylprednisolone pulses 1g/0.73m 2 /day for 3 days then 60mg/m 2) with poor response; persisting with involvement of the 3 cell lines in the blood count, elevated ferritin and triglycerides. Given the lack of response to steroids, Etoposide was started for 3 days associated with cyclophosphamide, the evolution was good with improvement of count blood cells, ferritin was lessening while the bone marrow showed decreased numerous of macrophages with low haemophagocytic activity. On the other side the infectious process was good managed by perfusion of Antibiotics such as Ceftazidim in combination with aminoside. The patient was sent home with prednisone and cyclosporine, He was placed on the list of bone marrow transplantation (BMT).

EN100372

On examination patient was pale with non pruritic macular rashes and mild splenomegaly.

A 33-year man working in Gulf, presented with hemetemesis 15 days ago in the month of February this year. This was preceeded by low grade fever, bone pain, generalised bodyache, low back ache and generalised macular skin rashes. There was no history of abdominal pain, jaundice or features of chronic liver disease. He gave history of intake of analgesics for backache. After symptomatic treatment he returned back. He came to our OPD with Complete blood count which showed a haemoglobin of 8g/dl, total count 6700/mm3 platelet count 24000/mm3 and ESR 55mm/hr. On examination patient was pale with non pruritic macular rashes and mild splenomegaly. Residing in Middle East with background history of fever, backache, skin rash, splenomegaly and a low platelet count possibility of atypical infection like Brucellosis with NSAID induced gi bleed was considered. He was admitted for further evaluation and endoscopy done showed a nodular ulcerative lesions in the antrum of stomach and histopathologic examination confirmed adenocarcinoma of stomach. Liver function tests showed a high ALP (1536 U/l), elevated INR (2.15) though bilirubin, transaminases and serum proteins were normal. Brucella agglutination test was negative. Xray Chest was normal. Ultrasound of abdomen showed normal echotexture of liver, no SOL, normal portal vein and no IHBRD. CT scan of the abdomen revealed gross pylorus thickening with regional lymphadenopathy and skeletal metastasis with sclerotic foci involving vertebrae and pelvic bones and lytic areas in bilateral iliac blades with no liver metastasis or ascites. A skeletal survey was done which showed lytic lesions in the iliac and femoral bones. Bone marrow aspirate showed decreased red cells and platelets and marrow biopsy done was reported as metastasis from adenocarcinoma. Coagulation profile done was suggestive of DIC due to the raised levels of FDP (5,170 ng/dl) and a low fibrinogen \(152mg/dl). He was transferred to Medical Oncology department for subsequent management and was treated with chemotherapy. He succumbed to his illness within 10 months of diagnosis.

EN100046

Histophotomicrograph of the excised breast lesion showed a 2.1 cm duct carcinoma, positive for ER and PR with 1 out of 25 lymph nodes positive for metastasis.

A 45 year old Indian female of Nordic origin presented 5 years back with a lump in the right breast and the axilla. She underwent modified radical mastectomy. Histophotomicrograph of the excised breast lesion showed a 2.1 cm duct carcinoma, positive for ER and PR with 1 out of 25 lymph nodes positive for metastasis. She received 6 cycles of chemotherapy with cyclophosphamide, epirubicin, and 5-fluorouracil. This was followed by tamoxifen 20 mg per day for five years. She was doing well on follow up until the completion of fifth year of her disease, when she presented with complaints of mild fever and weakness. Examination revealed generalized lymph node enlargement along with hepatomegaly. Hemogram showed mild anemia, normal platelet count and a leukocyte count of 1.2 x 10(11)/L. Peripheral blood examination revealed medium sized lymphoid cells, constituting almost 75% of total nucleated cell population. Immunophenotying, established a diagnosis of post thymic T-cell prolymphocytic leukemia. Contrast-enhanced computed tomography of the chest and abdomen was done which revealed an anterior mediastinal mass with destruction of sternum along with multiple small nodular shadows in bilateral lung fields suggestive of lung metastasis. Fine needle aspiration cytology of the mass showed atypical ductal cells with nuclear pleomorphism, which were positive for ER, PR and Her2neu protein. This confirmed a co-existent metastatic breast carcinoma. She was started on chemotherapy for T-PLL along with hormonal therapy with aromatase inhibitor. Unfortunately, both her malignancies progressed after an initial stable disease of two months.

EN108139

She had a history of diabetes, hypertension treated by glinide and calcium blocker.

A 77-year-old Tunisian woman was hospitalized because of massive painful ascites. Her family history did not include any autoimmune disease. She denied a history of hepatitis, jaundice or alcohol use. She had a history of diabetes, hypertension treated by glinide and calcium blocker. She was explored 4 years prior to admission for exudative pleuritis of the right lung without any established diagnosis after multiple explorations including thoracoscopy with biopsies. On admission, blood pressure was 150/70 mmHg, her rate was 80 /mn and body temperature was 37°C. The physical examination showed only abdominal distension related to the important ascites without collateral venous circulation. She had no skin lesions, lymphadenopathy, or hepatosplenomegaly and lower extremities showed no edema. Laboratory investigations showed: leucopenia with white blood cell count = 3100/mm3, lymphopenia = 840/mm3, hemoglobin = 10.5g/dL ; low serum albumin level = 29g/L, 47;-globulin = 19g/L. blood glucose levels = 180 mg/dl and hemoglobin A1c = 8.6 %. Urine analysis showed trace protein (0.03g/24h). Platelet count, activated partial thromboplastin time, liver function tests, blood urea nitrogen, serum creatinine, erythrocyte sediment rate and total cholesterol were within normal limits. Tests for HBsAg and HCV were negative. Abdominal ultrasonography showed ascitic fluid without any sign of bowel loop thickening or enteritis. Neither lymphadenopathy nor liver abnormality was present. A chest X-ray film disclosed bilateral pleural effusion. Ascitic obtained by aspiration contained 170 cells mm3 (67% lymphocytes), 46g/L protein, but no malignant cells. Cultures for bacteria and mycobacteria gave no growth. Tuberculosis investigation including initial tuberculin skin test and the research of Koch bacilli in sputum and urine were negative. Echocardiogram and electrocardiogram were normal. Computed tomographic scan showed massive ascites, no dysmorphic liver, and a mild bilateral pleural effusion. Pelvic examination was normal. Oesogastroduodenoscopy showed hiatal hernia without oesophageal varices. An exploratory laparoscopy was performed, revealing a large amount of ascite, a normal liver appearance, and no granulations suggesting tuberculosis or peritoneal carcinosis. Histological examination of the peritoneum showed non specific chronic inflammation. Systemic lupus erythematosus was suspected based on pleuritis, lymphopenia and leucopenia. An additional serologic survey revealed markedly elevated anti-nuclear antibody (ANA) titer of 1/1600 and a significantly elevated titer of antibody to double-stranded DNA (83 IU/mL ; normal < 30 IU/mL). She had also positive serum antibody against the Smith antigen and low serum level of C3 complement component: 67 mg/dL (serum normal range: 84 – 151). Relying on these findings, the patient was diagnosed with SLE since 4 of the 11 diagnostic criteria of the American College of Rheumatology were met. The SLEDAI score according to Systemic Lupus Erythematosus Disease Activity Index was estimated to be 7 on admission. Hydroxychloroquine 200 mg daily in combination with diuretics was initiated. One month later, there was no detectable ascitic fluid and no pleural effusions. Five months later she remained free from symptoms while continuing to take Hydroxychloroquine

EN100114

Thus, a diagnosis hypothesis of MVID was raised.

We report a case of a male near-term neonate with gestational age of 35 weeks. He was the third born child to a 27-year-old mother with no significant past history. He was derived from a third degree consanguineous marriage. He was born by means of normal vaginal delivery with a normal extra-uterine adaptation. Meconium-stained amniotic fluid was noted. His birth weight was 3030g (75 th Percentile). The baby had normal physical examination at birth aside transitory respiratory distress. He had been hospitalized for four days in a private clinic, for the suspicion of materno-foetal infection, where he had antibiotics for 48 hours. He had developed no symptoms during his hospitalization. The output's weight was 2400g which signify the loss of 630g. Back home, he received exclusive maternal breastfeeding. On his fifth day of life, he presented multiple yellowish-watery stools (ab 12 times) and refuse feeding with apyrexia context. He had clinical signs of severe dehydration without fever or vomiting. He lost 930g relative to the birth's weight. The abdomen appeared neither tender nor distended, and the rest of his physical examination was normal. The laboratory findings revealed hypernatremia of 152mEq/L, hyperkalemia of 7,8mEq/L and severe metabolic acidosis: blood PH: 7,1, HCO3:4mEq/L. The osmotic gap was 45. There was no leukocytosis, and the serum level of C-reactive protein was normal. The infant was treated with intravenous fluids and electrolytes as well as antibiotics after obtaining blood and stool cultures, assuming an infectious etiology. After 26 hours of rehydration therapy, the dehydration resolved, however, a functional acute renal failure complicated by acidosis and hyperkalemia appeared. The chest radiograph showed "bat wings" pattern and the cardiothoracic index was 0, 45. The renal ultrasound showed discretely hyperechoic kidneys cortex and the absence of renal malformations. On the 11 th day of life, he was transferred to the pediatric intensive care unit. He needed mechanical ventilation for three days and exclusive parenteral feeding by central catheter. The evolution was burdened by cholestasis due to the prolonged parenteral feeding and nosocomial infection by staphylococcus epidermidis. Since his admission, he had developed a slimy diarrhea with 3 to 4 stools per day. He presented a profuse diarrhea during each attempt to introduce enteral feeding by hydrolyzed milk formula. He was discharged after 32 days with output's weight 3250g. The oesophago-gastro-duodenoscopy and duodenal biopsy showed partial villous atrophy associated to the brush border abnormalities. The periodic acid Schiff (PAS) stain revealed abnormalities in the brush border characterized by loss of the linear part of enterocytes with the presence of intra-cytoplasmic PAS + band in the apical pole of enterocytes uneven thickness and sometimes with double contours pattern. In the submucosa, there were numerous Brunner glands. We find the existence of numerous hemorrhagic suffusions. The CD10 immunostain was positive. Thus, a diagnosis hypothesis of MVID was raised. The electron microscopic examination confirmed the diagnosis. Mutation analysis of peripheral blood samples of the neonate revealed a mutation at the homozygous status of MYO5B gene. The baby died, after 97 days of life, of septic shock and multiple organ failure.

EN100376

A 26-year-old pregnant patient with a history of two previous cesarean deliveries was referred to our tertiary clinic.

A 26-year-old pregnant patient with a history of two previous cesarean deliveries was referred to our tertiary clinic. The patient was having severe right sided abdominal pain with sudden onset. She was in the 18 th week of her pregnancy based on last menstrual period. Obstetric ultrasound measurements revealed a live pregnancy compatible with 18 weeks gestation without any abnormality. However, a 15x12cm in size edematous multicystic adnexal mass occupying the mid and upper right abdomen without vascularity was identified. The mass was suspected to originate from right ovary. It was pushing the pregnant uterus into the left and anterior direction. There was no ultrasonographic evidence of malignancy. Considering the severe sudden pain complaint and adnexal mass finding, explorative laparotomy was planned for the suspected diagnosis of adnexal torsion. A mediolateral (near vertical) skin incision was made originated from infra-umbilical level on the lateral side of right rectus muscle. Specifically, the starting point for this slightly oblique incision was at 3cm lateral to the midline and near to lateral one third of the rectus muscle. The skin and fascia incisions were approximately 4cm and 2.5cm in length, respectively and this difference was due to the thick subcutaneous fat tissue. The oblique paramedian incision of the anterior rectus sheath was approximately 2cm medial to the lateral border of the rectus muscle and it was extended to caudal-lateral direction, down to 1/2cm to the lateral border of rectus muscle. The lateral edge of anterior rectus sheath incision was dissected off from the rectus muscle laterally up to semilunar line of rectus muscle in 1cm width. Then, the rectus muscle was retracted medially to expose the posterior sheath of rectus (below the arcuate line, only the transversalis fascia and parietal peritoneum are exposed). An oblique incision was made on the posterior sheath of rectus and peritoneum behind the rectus muscle, in a projection of the anterior sheath incision. In this manner, the abdominal wall incisions were located at anterior and posterior sheaths of rectus muscle at least 1/2cm medial to the semilunar line and to the lateral border of rectus. In other words, incision was not made from fascial layers' merging point or by muscle splitting dissection. Therefore, traction forces from enlarging uterus in advancing pregnant abdomen could be minimized. A large edematous, necrotic and dark purple torsion of a right adnexal mass was observed. It was pulled out and was partially dissected from its surface layer following a 2cm incision. The cystic structure was aspirated and right salpingo-ooferectomy was performed. The operation was completed by avoiding the manipulation of the pregnant uterus. Frozen section pathology of the removed specimen was reported as benign (necrotic tissues, teratoma) and final pathology was mucinous cystadenoma. Follow up on the pregnant patient was uneventful a month later.

EN100668

She reported that in spite of these medications the child continued to have seizures and nodding at least twice a day and had since dropped out of school.

A 13-year-old boy diagnosed with WHO diagnostic criteria of probable NS was referred from Atanga HC III in Pader district where he was enrolled and undergoing care at the nodding syndrome treatment center; he came with a history of progressive swelling and pain in the right lumbar region. The swelling was associated with a high grade fever which was constant and only partially relieved by analgesics. These symptoms were not associated with vomiting, constipation, yellow eyes, loss of appetite or weight loss. The patient reported a history of falling from a tree during one of the nodding episodes in October 2012 and hit his abdomen onto a tree branch. On further probe on his childhood history, his mother reported that he was born normally at home by a Traditional Birth Attendant (TBA) in one of the Internally Displaced peoples (IDP) camps in 2000. She reported that there was an uneventful pregnancy which was carried to term and delivery by Spontaneous vaginal delivery (SVD). She reported that during her pregnancy, she had exclusive feeding on the relief food provided by WFP (beans, yellow posho and cooking oil) during the IDP camps and denies history of ingestion of herbs or medications which caused adverse events during and after the pregnancy. She reported that her child had a normal physical, cognitive and social childhood development before the onset of nodding which began in May 27th 2011 immediately after returning home from IDP camps. The child was enrolled in Atanga treatment centre and was being managed with Carbamazepine, multivitamins and Ivermectin. She reported that in spite of these medications the child continued to have seizures and nodding at least twice a day and had since dropped out of school. On general examination, he was dehydrated, febrile and moderately wasted. There was a right lumbar region mass, tender, indurated and non-fluctuant. The spleen and liver were not palpable. There was no renal or supra-pubic tenderness. The rectum was full of faecal material which was of normal colour and consistency. The anal tone was normal and there was no blood on examining fingure. Haematological investigations were conducted and showed neutrophilia, lympocytosis, monocytosis, and eosinophilia. There were immature granulocytes and atypical lymphocytes seen on the peripheral film report. Other laboratory results including liver function tests (ALT, AST) were elevated while serum protein levels were low; renal functions tests (serum creatinine, blood urea and nitrogen level), and serum electrolytes (K+, Na+, Cl-, HC03-) which were within normal ranges. Abdominal Ultrasound showed inflamed internal and external oblique muscles of the anterior abdominal wall. The patient underwent incision and drainage at Gulu Hospital and wound left open for 14 days and thereafter secondary wound closure was conducted. He received supplementary food rehabilitation and his seizure medication was changed to Sodium Valproate 200mg once a day under direct observation therapy (DOTS) and close monitoring of the vital signs. The patient continued to have regular follow up in the surgical ward; seizures and nodding stopped completely from the time of intervention in the hospital. With these interventions for over one month the child had no seizures nor nodding and the child returned to normal life. A subsequent review of the haematological and clinical chemistry findings 2 weeks later showed that renal function tests, serum electrolytes were normal except the liver enzymes level were elevated and were still high.

EN100075

Otherwise there is an absence of expression of PS100, MelanA, HMB45, desmin, p40, CD45 and EBV.

Mr F.B a 70-year-old male patient presented in our institution because of the occurrence of a right epistaxis. First of all, the patient is not smoking and reported an aneurysm of the abdominal aorta surgically treated by prosthesis under plavix. The beginning of the symptomatology goes back to 2 months before with right nasal obstruction of progressive onset, associated with right epistaxis and bloody rhinorrhea with right chronic eye watering. Nasal Endoscopy examination revealed a pinnkish burgeoning mass bleeding on contact and filling the right nostril vestibule. The patient didn't presents neurological signs nor cervical lymph nods. Otherwise, ophthalmologic examination found normal visual acuity and ocular mobility on both sides. Sinonasal computed tomography (CT) found a right nasal process enhanced after injection of contrast product with relatively little washing at the late time. Otherwise, there is no extension involvement at the level of the cavum and the pterygo palate fossa. This finding found also a crenate appearance of the posterior nasal mucosa especially of the middle turbinates and contralateral inferior turbinate with a left deviation of the nasal septum with nasal spur. Nasosinusal MRI shows a progressive increase in size of the tissue mass compared to the previously CT occupying the right nasal fossa on almost all of its height, measuring 4 × 2.8 × 3.5cm without extension within the cavum with probable invasion of the lower part of the nasolacrimal duct and mass effect on both the sinusonasal bone septum and the intersinusonasal septum. This finding shows ethmoidal sinus fluid retention. The appearance, even if it remains unspecific, is compatible with inverted papilloma. The patient underwent complete endoscopic removal of the tumor with ethmoidectomy and right maxillary antrostomy. The extemporaneous examination is in favor of a malignant tumor lesion. Histopathologic analysis shows a tumor proliferation made of polymorphic cells with major anisocytosis, most often located in lobules. There is sometimes a somewhat palisadic seat on the periphery of the tumor mass. The mitotic index is 10 mitoses per 10 high power fields. The immunohistochemical study of the tumor found that the cytokeratin KL1 and CK7 and the neuroendocrine marker Chromogranin A, synaptophysin and CD56 are positive. Proliferation index Ki67 is of the order of 80%. Otherwise there is an absence of expression of PS100, MelanA, HMB45, desmin, p40, CD45 and EBV. The clear positivity of cytokeratins formally excludes the diagnosis of olfactory neuroblastoma and leads to neuroendocrine carcinoma. Given the atypia, the mitotic index, and the Ki67 proliferation index, it is a high-grade large-cell neuroendocrine carcinoma. Extension assessment, comprising a PET SCAN, was negative. Postoperative treatment consisted in adjuvant polychemotherapy, with six cycles (cisplatin and etoposide), Followed by loco regional external radiotherapy. After 6 month follow-up, the patient was in complete clinical and radiological remission.

EN100383

Frozen section pathology of the removed specimen was reported as benign (necrotic tissues, teratoma) and final pathology was mucinous cystadenoma.

A 26-year-old pregnant patient with a history of two previous cesarean deliveries was referred to our tertiary clinic. The patient was having severe right sided abdominal pain with sudden onset. She was in the 18 th week of her pregnancy based on last menstrual period. Obstetric ultrasound measurements revealed a live pregnancy compatible with 18 weeks gestation without any abnormality. However, a 15x12cm in size edematous multicystic adnexal mass occupying the mid and upper right abdomen without vascularity was identified. The mass was suspected to originate from right ovary. It was pushing the pregnant uterus into the left and anterior direction. There was no ultrasonographic evidence of malignancy. Considering the severe sudden pain complaint and adnexal mass finding, explorative laparotomy was planned for the suspected diagnosis of adnexal torsion. A mediolateral (near vertical) skin incision was made originated from infra-umbilical level on the lateral side of right rectus muscle. Specifically, the starting point for this slightly oblique incision was at 3cm lateral to the midline and near to lateral one third of the rectus muscle. The skin and fascia incisions were approximately 4cm and 2.5cm in length, respectively and this difference was due to the thick subcutaneous fat tissue. The oblique paramedian incision of the anterior rectus sheath was approximately 2cm medial to the lateral border of the rectus muscle and it was extended to caudal-lateral direction, down to 1/2cm to the lateral border of rectus muscle. The lateral edge of anterior rectus sheath incision was dissected off from the rectus muscle laterally up to semilunar line of rectus muscle in 1cm width. Then, the rectus muscle was retracted medially to expose the posterior sheath of rectus (below the arcuate line, only the transversalis fascia and parietal peritoneum are exposed). An oblique incision was made on the posterior sheath of rectus and peritoneum behind the rectus muscle, in a projection of the anterior sheath incision. In this manner, the abdominal wall incisions were located at anterior and posterior sheaths of rectus muscle at least 1/2cm medial to the semilunar line and to the lateral border of rectus. In other words, incision was not made from fascial layers' merging point or by muscle splitting dissection. Therefore, traction forces from enlarging uterus in advancing pregnant abdomen could be minimized. A large edematous, necrotic and dark purple torsion of a right adnexal mass was observed. It was pulled out and was partially dissected from its surface layer following a 2cm incision. The cystic structure was aspirated and right salpingo-ooferectomy was performed. The operation was completed by avoiding the manipulation of the pregnant uterus. Frozen section pathology of the removed specimen was reported as benign (necrotic tissues, teratoma) and final pathology was mucinous cystadenoma. Follow up on the pregnant patient was uneventful a month later.

EN100668

The patient was referred to her neurologist to provide a detailed history about her medical condition and to coordinate with him regarding the steroid dose take to avoid any complication during patient management.

A 28-year-old female patient presented to outpatient dental clinics for full mouth rehabilitation. On physical examination, the patient was awake, alert, oriented, and her blood pressure, temperature and heart rate were within normal limits. Upon taking medical history the patient revealed that was diagnosed with thymoma MG and on medication including pyridostigmine bromide 60 mg, azathioprine 50 mg and prednisone 20 mg. Intraoral examination showed fair oral hygiene multiple fixed prosthodontic restorations and multiple carious teeth, multiple remaining roots and lipomatous tongue atrophy. The patient was referred to her neurologist to provide a detailed history about her medical condition and to coordinate with him regarding the steroid dose take to avoid any complication during patient management. A consultation letter had been received from her neurologist explaining the patient medical condition which starts with difficulty in breathing, chewing and swallowing, there was no external ocular weakness, motor weakness, sphincter dysfunction, sensory deficits, muscle wasting and no signs of diplopia, dizziness, double vision, ptosis or any difficulty in maintaining balance. Patient underwent chest CT scan with contrast which show evidence of thymoma benign tumor of the thymus that has been the possible causes of MG. Diagnosis was confirmed with measuring serum level of acetylcholine receptor antibodies, later treatment plan had been formulated including drugs to control the condition and then thymectomy to avoid sever complication to the patient. Before proceeding to the dental management of the case; CBC and blood chemistry had been requested form the patient. Creatinine level was below normal (42.23 umol/l), with normal levels of albumin, urea, uric acid, AST, ALT, cholesterol, triglycerides and bilirubin. Patient had elevated WBC “12.52 x 10 9 /L” and Platelet “453 x 10 9 /L” with reduction in RBC “4.4 x 10 9 /L” and Eosinophil count “0.07”. Consultation with patient neurologist regarding the steroid dose to reduce the susceptibility of developing adrenal crisis.

EN100437

A transient lull was observed following a treatment with a progesteron (hydroxyprogesterone caproate) and an antispasmodic (tiemonium methylsulfate) in a community clinic.

It is the case of a 29 years old woman, G2P1001, married, at 9 weeks of pregnancy. She consulted at our emergency service for the exacerbation of a left pelvic pain that has been evolving for two weeks prior to admission. A transient lull was observed following a treatment with a progesteron (hydroxyprogesterone caproate) and an antispasmodic (tiemonium methylsulfate) in a community clinic. The resurgence of that lancinating and permanent pain irradiating to the loins and associated with slight vaginal bleeding prompted consultation. She had first menses at 14 and her menstrual cycle is regular with a length of 30 days. She has never practiced contraception. She has been adequately treated for acute pelvic inflammatory disease five years earlier. Her only child is a girl born vaginally seven years ago. Her blood group is B rhesus positive and she has never undergone surgery. She presented with sympathetic signs of pregnancy and the urinary pregnancy test was positive but echography was not yet done. On admission, besides the main complain, the patient had vaginal bleeding and nausea but neither fever, nor vomiting. On physical examination her general condition was good and the blood pressure was 110/70 millimeters of mercury, the respiratory rate: 20 cycles / minute; the pulse rate: 70 pulsations/minute and the temperature: 37.3 degree Celsius. The conjunctivae were pink and the tongue was clean and moist. There were no cervical adenopathies. The breast and cardiopulmonary examination revealed no abnormalities. The abdomen was flat, and mobile with respiration. On palpation there was just a tenderness of the left iliac fossa and the bowel sounds were normal on auscultation. Inspection under speculum revealed normal gravid external cervical os and there was no vaginal discharge. On digital exploration, the cervix was posterior long and closed, the uterus was globular, increased in size and compatible with an eight weeks pregnancy. The left adnexae presented with a tender, smooth and mobile mass of seven centimeters diameter but the right one were normal. The posterior cervico-vaginal fornix was neither tender nor bulging. We suspected a non ruptured extra-uterine pregnancy with the following differentials: heterotopic pregnancy, torsion of ovarian cyst in pregnancy, and intra-cystic ovarian bleeding in pregnancy. Paraclinical investigations revealed: beta human Chorionic Gonadotropin (β hCG) level of 96 702 milli International Units per milliliter of plasma (mIU/ml). Ultrasonography revealed a heterogenous left ovarian mass of 82 millimeters in diameter, an empty uterus and no peritoneal effusion. This comforted our first diagnosis. After a normal pre-operative work up, an emergency laparotomy was done under general anesthesia. The findings were: left ovary containing a gestational sac and the corpus luteum, normal right adnexae, normal left tube and normal uterus. There was no hemoperitoneum. We dissected the ovarian capsule and carried out ablation of the gestational sac, then we did hemostasis. The specimen was analysed by the pathologist who found decidual cells and trophoblastic tissue within the ovarian capsule and thus confirmed the ovarian pregnancy. Post operative course was uneventful and the patient was discharged six days after surgery. The ?hCG level decreased and disappeared 25 days after surgery.

EN100068

Anatomical pathology result through macroscopic observation showed visible network of approximately 0.3 cm and 0.4 cm in diameter with a grayish-white color, while the microscopic observations showed fibromuscular tissue at high cylindrical epithelium with polyps shaped vacuolar, and inflammatory granulomatic and aplastic cells were not found.

A 33-year-old woman came to Department of Fertility, Endocrinology and Reproduction at Saiful Anwar public Hospital and consulted that she has not menstruated since 5 years ago (28 years old). Her initial menarche was at the age of 14 years and it is in accordance with the normal growth of a child at her age. The patient had a regular menstrual period of 5-7 days, she replaced the pads for 2-3 times / day, and had no menstrual pain. The patient has a history of injective contraceptive that was done every once a month in 2010, and after the injection, she menstruated for two months and then it stopped. Based on clinical examination and a physical examination, the patient was diagnosed with secondary amenorrhea. Investigations that were used are ultrasonography, thorax X-ray and CT Scan Head. Ultrasonography and thorax X-Ray showed that patient is suspected to have right pleural effusion. CT-Scan examination showed that bilateral ethmoidal sinusitis and pituitary gland was within normal limits. The treatment plan in this patient is P test therapy with Prothyra 1x10 mg for 7 days, with control on 2 weeks after the use. P test showed a negative result, because after 14 days of progesterone consumption the prothyra is not bleeding. The treatment is followed by a E+P test (Estrogen+Progesterone) with Estrogen 1x0.625 mg for 21 days and an addition of Progesterone 1x10 mg on 12th -21st days, while the control was performed one week after the drug runs out. Based on the E+P test, the obtained results were FSH: 8.71 MIU/mL, LH: 3.1 IU/L, Prolactin: 319.4 ng/mL. The E+P test showed a negative result, so it was necessary to do hysteroscopy and curettage for the uterus evaluation. Hysteroscopy results showed that there were grade 4 adhesions in the uterine cavum, with a pale colored connective tissue in the uterine cavum, which refers to the diagnosis of secondary amenorrhea suspect tuberculosis endometritis, while the result of tissue curettage was examined on the Anatomical Pathology department. Results of anatomical pathology hysteroscopy showed endometrial tissue with stromal looks granulomas with lymphocytes, histiocytes, epithelioid and Langhans multinucleated giant cells and no malignancy was found in this preparation, which showed that patient's diagnosis is secondary amenorrhea suspected tuberculosis endometritis. Based on the Anatomical Pathology hysteroscopy result, treatment was done by using anti-tuberculosis drug category I (FDC 1X3 tab) for 6 months. Anatomical pathology result through macroscopic observation showed visible network of approximately 0.3 cm and 0.4 cm in diameter with a grayish-white color, while the microscopic observations showed fibromuscular tissue at high cylindrical epithelium with polyps shaped vacuolar, and inflammatory granulomatic and aplastic cells were not found.

EN100275

However, the general and mental condition was relatively better.

A 35-year-old mentally ill man was transferred from a local psychiatric hospital after attempted suicide by fire 3 days before admission. He acquired full-and partial-thickness injury in approximately 38% of his TBSA, including face and neck (5%), both upper extremities (8%), right thigh (4%), left thigh and leg (3%), and most of his anterior and posterior trunk (16%). The patient was hospitalized for 66 days and underwent five debridement surgeries. The psychiatric diagnosis was schizophrenia type ICD 10 F20.3, treated with Haloperidol 1.5 mg and Diazepam 5 mg daily. The patient was occasionally restrained due to rebellious and self-destruction behavior. The nutrition management of this patient was started after a week of hospitalization, which is considered late. In the initial nutrition assessment, the patient was 40 kg weight and 150 cm tall thus the body mass index (BMI) was 17,57 kg/m 2 or underweight. Gastrointestinal symptoms and signs were not found. Information regarding the patient's dietary intake in the last two weeks and weight change within the last 6 months could not be obtained, but his mother claimed that the patient seemed to lose weight because he refused any food that had been offered to him; he only smoked and drank coffee. Albumin level was 3.6 gr/L with imbalanced electrolyte levels. Signs of fluid accumulation were not found. According to the subjective global assessment (SGA) tool, the patient belonged to group C (severely malnourished). Energy and protein were given starting from 30 kcal/kg, increasing gradually to 50 kcal/kg, and 2 gp/kg divided into oral nutrition and oral nutrition supplements (ONS) with a 50:50 ratio. The patient was also given micronutrient supplementations, specifically vitamin A 100,000 units for the first week, vitamin C 100 mg 3 times a day, and zinc 20 mg once a day. During the early weeks of hospitalization, the patient's weight loss continued. At the end of the second week, his body weight was 36 kilograms while his serum albumin level was 2.7 gr/L. On the 28 th day, the patient's body weight was 33 kilograms while his serum albumin level was 2.1 gr/L. Energy and protein intake was maintained, but the ratio of oral intake and ONS was modified to 70:30. During the treatment, the patient's appetite was unstable. The patient was also hardly cooperative with enteral access. The patient often writhed in pain and became more aggressive. The patient did not respond well to analgesics and sedations. Partially parenteral nutrition (PN) enriched with branched-chain amino acid (BCAA) was administered to support protein needs. However, his BMI continued to decline, and on the 42 nd day, his body weight dropped to 32 kilograms with a serum albumin level of 2 gr/L. On the 66 th day, granulation tissues appeared on the wound bed. Considering the mental and nutrition status of the patient, it was decided not to continue with the skin graft surgery. The patient had lost 24% of body weight (BMI 11.96 kg/m 2) and the serum albumin level was 2.5 gr/L. The patient displayed severe muscle wasting and subcutaneous fat loss. However, the general and mental condition was relatively better. The patient was discharged from the hospital.

EN100658

On pathology, the renal mass was classified as a papillary renal neoplasm consistent with a translocation carcinoma.

A 45 year old female with history of sickle cell disease presented with abdominal pain. A CT scan revealed an exophytic renal mass measuring 2.9cm x 2.0cm x 2.1cm located on the antero-lateral aspect of her right kidney and an unremarkable spleen. She was evaluated by urology and a right partial nephrectomy was planned, however on the day of the procedure, the patient's preoperative pregnancy test was positive. The procedure was post postponed until after she delivered and interval ultrasounds were obtained throughout her pregnancy to monitor the renal mass. The lesion increased minimally in size. Two months after caesarian section delivery, patient underwent an unremarkable right laparoscopic partial nephrectomy with individual artery and vein vascular occlusion at the level of the renal hilum. A superficial liver laceration caused by the Veress needle was noted at the beginning of the procedure and effectively managed using bipolar cautery. The patient's post-operative course was notable for marked thrombocytosis with her platelet count increasing for her baseline of 300,000 to a zenith of 1.3 million. She was started on aspirin therapy and splenomegaly was noted on abdomen ultrasound. Her platelet count normalized to 334,000. On pathology, the renal mass was classified as a papillary renal neoplasm consistent with a translocation carcinoma. There was no splenic tissue in the specimen. It was noted that the complete lack of staining of any keratins or epithelial membrane antigens was not consistent with a usual renal cell carcinoma and hence the diagnosis of a translocation tumor was made. The patient was lost to follow up presenting 2 years later and a follow-up CT scan noted marked splenic atrophy with several splenules. The patient was completely asymptomatic and at the time received all the post-splenectomy vaccination and her platelet count remained within normal limits.

EN100385

He had difficulty maintaining eye contact, and he appeared disinterested in what was taking place.

We received a boy 3-year-old boy with autistic disorder on hospital of pediatric ward A at university hospital Mohammed VI of Marrakesh. He has no family history of illness or autistic spectrum disorder. The history revealed that at 2 years of age. The child was diagnosed with a ‘‘severe communication disorder,'' with social interaction difficulties and sensory processing delay. A composite follow-up of all previous assessments and investigations was undertaken. Blood work was normal (thyroid-stimulating hormone (TSH), hemoglobin, mean corpuscular volume (MCV), and ferritin). Genetic testing was unremarkable (normal karyotype, negative for fragile X) and the magnetic resonance imaging (MRI) in search of a demyelinating attack of the white matter, electroencephalography (EEG), optometry assessment were also normal. At the conclusion of this composite assessment, the boy was given a primary diagnosis of autistic spectrum disorder. A plan was instituted including speech-language therapy, intensive individualized educational programming, and contact was encouraged with the Autism Society. For that purpose, the parents moved to morocco to be surrounded by family without any huge improvement. Upon direct questioning at initial presentation, the 3-year-old child was reported by the parents to have unexplained fatigue, Gastrointestinal symptoms included bloating, constipation and diarrhea. Psychiatric symptoms included a frequently depressed mood, disproportionate anger, and emotional lability. On exploring his history, the child was born from yemenite father and Moroccan mother in Germany at-term weighing 3500 g with an Apgar score of 9 at 1 minute and 10 at 5 minutes after an uneventful pregnancy and delivery. No concerns were present in the neonatal period. Development in the first 24 months of life appeared fine according to the parents-his motor skills seemed to progress normally and he achieved expected milestones. After 2 years of age, however, his language skills slowly began to regress. He also demonstrated a change in temperament as he started to whine repeatedly and to scream without provocation. Physical examination revealed a height and weight normal for age. He was uncommunicative, restless, and somewhat agitated. General examination was unremarkable other than dark rings around the eyes. There were no dysmorphic features evident. He had difficulty maintaining eye contact, and he appeared disinterested in what was taking place. No abnormality was found on abdominal assessment. Anti-tissue transglutaminase antibodies levels were 76 U (normal < 10). The patient underwent upper endoscopy as duodenal biopsy to confirm a celiac disease diagnosis. It shows a total villous atrophy corresponding to a stage 4 of Marsh classification. Given the positive screen for celiac disease (positive anti-tissue transglutaminase antibodies and results of duodenal biopsy), dietary intervention was immediately commenced. All gluten was eliminated from the boy's diet. Within 1 month, the boy's gastrointestinal symptoms were relieved and his behavior had changed. The mother reported that her boy became progressively more communicative. The child has continued on the gluten-free diet and has progressed well and remained healthy over the following 6 months.

EN100490

There were immature granulocytes and atypical lymphocytes seen on the peripheral film report.

A 13-year-old boy diagnosed with WHO diagnostic criteria of probable NS was referred from Atanga HC III in Pader district where he was enrolled and undergoing care at the nodding syndrome treatment center; he came with a history of progressive swelling and pain in the right lumbar region. The swelling was associated with a high grade fever which was constant and only partially relieved by analgesics. These symptoms were not associated with vomiting, constipation, yellow eyes, loss of appetite or weight loss. The patient reported a history of falling from a tree during one of the nodding episodes in October 2012 and hit his abdomen onto a tree branch. On further probe on his childhood history, his mother reported that he was born normally at home by a Traditional Birth Attendant (TBA) in one of the Internally Displaced peoples (IDP) camps in 2000. She reported that there was an uneventful pregnancy which was carried to term and delivery by Spontaneous vaginal delivery (SVD). She reported that during her pregnancy, she had exclusive feeding on the relief food provided by WFP (beans, yellow posho and cooking oil) during the IDP camps and denies history of ingestion of herbs or medications which caused adverse events during and after the pregnancy. She reported that her child had a normal physical, cognitive and social childhood development before the onset of nodding which began in May 27th 2011 immediately after returning home from IDP camps. The child was enrolled in Atanga treatment centre and was being managed with Carbamazepine, multivitamins and Ivermectin. She reported that in spite of these medications the child continued to have seizures and nodding at least twice a day and had since dropped out of school. On general examination, he was dehydrated, febrile and moderately wasted. There was a right lumbar region mass, tender, indurated and non-fluctuant. The spleen and liver were not palpable. There was no renal or supra-pubic tenderness. The rectum was full of faecal material which was of normal colour and consistency. The anal tone was normal and there was no blood on examining fingure. Haematological investigations were conducted and showed neutrophilia, lympocytosis, monocytosis, and eosinophilia. There were immature granulocytes and atypical lymphocytes seen on the peripheral film report. Other laboratory results including liver function tests (ALT, AST) were elevated while serum protein levels were low; renal functions tests (serum creatinine, blood urea and nitrogen level), and serum electrolytes (K+, Na+, Cl-, HC03-) which were within normal ranges. Abdominal Ultrasound showed inflamed internal and external oblique muscles of the anterior abdominal wall. The patient underwent incision and drainage at Gulu Hospital and wound left open for 14 days and thereafter secondary wound closure was conducted. He received supplementary food rehabilitation and his seizure medication was changed to Sodium Valproate 200mg once a day under direct observation therapy (DOTS) and close monitoring of the vital signs. The patient continued to have regular follow up in the surgical ward; seizures and nodding stopped completely from the time of intervention in the hospital. With these interventions for over one month the child had no seizures nor nodding and the child returned to normal life. A subsequent review of the haematological and clinical chemistry findings 2 weeks later showed that renal function tests, serum electrolytes were normal except the liver enzymes level were elevated and were still high.

EN100075

A 45 year old female with history of sickle cell disease presented with abdominal pain.

A 45 year old female with history of sickle cell disease presented with abdominal pain. A CT scan revealed an exophytic renal mass measuring 2.9cm x 2.0cm x 2.1cm located on the antero-lateral aspect of her right kidney and an unremarkable spleen. She was evaluated by urology and a right partial nephrectomy was planned, however on the day of the procedure, the patient's preoperative pregnancy test was positive. The procedure was post postponed until after she delivered and interval ultrasounds were obtained throughout her pregnancy to monitor the renal mass. The lesion increased minimally in size. Two months after caesarian section delivery, patient underwent an unremarkable right laparoscopic partial nephrectomy with individual artery and vein vascular occlusion at the level of the renal hilum. A superficial liver laceration caused by the Veress needle was noted at the beginning of the procedure and effectively managed using bipolar cautery. The patient's post-operative course was notable for marked thrombocytosis with her platelet count increasing for her baseline of 300,000 to a zenith of 1.3 million. She was started on aspirin therapy and splenomegaly was noted on abdomen ultrasound. Her platelet count normalized to 334,000. On pathology, the renal mass was classified as a papillary renal neoplasm consistent with a translocation carcinoma. There was no splenic tissue in the specimen. It was noted that the complete lack of staining of any keratins or epithelial membrane antigens was not consistent with a usual renal cell carcinoma and hence the diagnosis of a translocation tumor was made. The patient was lost to follow up presenting 2 years later and a follow-up CT scan noted marked splenic atrophy with several splenules. The patient was completely asymptomatic and at the time received all the post-splenectomy vaccination and her platelet count remained within normal limits.

EN100385

Urine analysis and culture were performed several times that was negative for infection.

The patient was a 35-year old female who presented with urinary frequency for 2 months. Her past medical history showed a nonsmoking history and no significant family history. The physical examination was unremarkable. She was treated as urinary tract infection, but there was no improvement in her symptoms. Urine analysis and culture were performed several times that was negative for infection. Other laboratory data were also insignificant. Through a routine evaluation, urinary system ultrasonography (US) revealed a bladder lesion measuring 7 mm. In order to confirm the diagnosis, magnetic resonance imaging (MRI) of the abdomen and pelvic cavity was done; it revealed a lesion in the dome of the bladder. To confirm the diagnosis and for histopathological examination, the patient underwent Transurethral Resection of Bladder Tumor (TURBT) in April 2018. Before starting the procedure, the urethra was dilated till Fr: 28; at first, the tumor location was specified by cystoscopy. Then, it was resected by monopolar cautery from superficial to deeper parts with removal of the muscle layers. Proper bleeders were taken and three way Foley catheter was applied for a continuous irrigation. The patient was transferred to the recovery room with complete stability. The histopathology slides show a well-defined mass composed of bland looking spindle cells. Subsequent immunohistochemistry (IHC) was done which showed diffuse immunoreactivity for S100, but other markers including smooth muscle actin (SMA), desmin, cytokeratin, CD34 and beta catenin were negative. Ki67 was 2-3%, so the diagnosis of schwannoma was confirmed. Then, the neck, abdomen, pelvic and chest computed tomography (CT) scans were done that were normal. Brain and spine MRI and physical examination showed no evidence of other schwannoma or evidence of neurofibromatosis. She had no history of neurofibromatosis in her family. Skin examination was normal and had no café au lait spot. She was not a case of von Recklinghausen disease.

EN100506

At operation, under general anesthesia, an extended transverse inguinal incision was made.

Male infant 9.5 month old was examined in the emergency department of our hospital because of an empty scrotum and the ipsilateral testis lying in a high iliac crest position. With the increase in intrabdominal pressure [crying etc.] there was an ever-increasing swelling with spiral course from the groin to ipsilateral iliac crest. On clinical examination, a reducible mass was present in the right ileac crest. The swelling looked like a spigelian hernia [4]. However no abdominal wall defect could be palpated at that area. Moreover the ipsilateral scrotum was empty and the testis could be palpated with difficulty lying in a high inguinal position. Ultrasonography showed a loop of herniated small bowel and an undescended testis on the right. At operation, under general anesthesia, an extended transverse inguinal incision was made. Upon entering the inguinal canal a large hernia sac was identified. This followed a course through the superficial inguinal ring, extended upwards to the anterior abdomninal wall below the subcutaneous plane and was fixed in the area of the right iliac crest. We noticed that the hernia orifice was in a higher than normal position 5 cm from the pubic bone. This bulk was easily reduced back to the abdomen along with an ipsilateral mobile intravaginal testis. After careful isolation of the sac at a length of 3 cm the ipsilateral testis was isolated. The vas and the vessels were intravaginal, the gubernaculum was fixed in an ectopic position and the dorsal inguinal wall was lax. We removed the large sac after separating the vas and vessels and the testis. Moreover we strengthened the dorsal inguinal wall, fixated the testis in a subdartos pouch and closed the wound. Patient stayed for a day in the hospital. No postoperative complications happened. Three years later the boy was operated for left hydrocele. The boy remains in perfect health now five years after the initial operation.

EN100508

The three lesser metacarpals and the distal fragment of the hamatum were medially displaced.

A 20-year-old right-handed female factory worker with no medical history is consulting in the emergency ward with an open trauma of the left hand resulting from an occupational accident. Her hand was crushed under a heavy object. Physical examination found an important swelling and obvious distortion at the dorsum of the hand associated with wounds of the ulnar and radial edges of the left hand. The neurovascular examination was normal; in particular no sensitive deficit in the median nerve area was noted. Plain radiographs with front and lateral views showed a dislocation of the three lesser fingers associated with fractures of the hamatum and the second metacarpal. The three lesser metacarpals and the distal fragment of the hamatum were medially displaced. The patient was immediately brought to the operating theatre. The treatment consisted of a reduction and stabilisation using a multiple carpo-metacarpal and cross inter-metacarpal pinning. The wound was cleaned up trimmed and sutured. The had and the wrist were immobilized with a splint for six weeks. The patient had an intense and regular rehabilitation program. At one year follow-up, the outcome is good: the patient is painless with good bone consolidation in right position, strictly normal range of motion (metacarpophalangeal 90°, proximal interphalagienne 100° and distal interphalageal 90°) and a 80% grip strength compared to the right side.

EN100345

Skeletal survey and bone scan did not reveal any other lesion in the body.

Forty year old male presented with a huge mass in this left arm. The same patient apparently was admitted three years back for excision of the arm swelling which was relatively small that time. However the patient did not undergo surgery and was takin treatment from local bone setters during the last three years and tumor grew to a larger size during this period. The current dimensions of tumor was 32x28 cms with circumference of 87 cms. X-ray showed complete destruction of the upper humerus with central calcification. Biopsy releveled a chondrosarcoma. Skeletal survey and bone scan did not reveal any other lesion in the body. A forequarter amputation was done and a 16 kg tumor mass was excised. At three years follow up, the patient has no complains with no recurrence of the tumor.

EN104179

The oblique paramedian incision of the anterior rectus sheath was approximately 2cm medial to the lateral border of the rectus muscle and it was extended to caudal-lateral direction, down to 1/2cm to the lateral border of rectus muscle.

A 26-year-old pregnant patient with a history of two previous cesarean deliveries was referred to our tertiary clinic. The patient was having severe right sided abdominal pain with sudden onset. She was in the 18 th week of her pregnancy based on last menstrual period. Obstetric ultrasound measurements revealed a live pregnancy compatible with 18 weeks gestation without any abnormality. However, a 15x12cm in size edematous multicystic adnexal mass occupying the mid and upper right abdomen without vascularity was identified. The mass was suspected to originate from right ovary. It was pushing the pregnant uterus into the left and anterior direction. There was no ultrasonographic evidence of malignancy. Considering the severe sudden pain complaint and adnexal mass finding, explorative laparotomy was planned for the suspected diagnosis of adnexal torsion. A mediolateral (near vertical) skin incision was made originated from infra-umbilical level on the lateral side of right rectus muscle. Specifically, the starting point for this slightly oblique incision was at 3cm lateral to the midline and near to lateral one third of the rectus muscle. The skin and fascia incisions were approximately 4cm and 2.5cm in length, respectively and this difference was due to the thick subcutaneous fat tissue. The oblique paramedian incision of the anterior rectus sheath was approximately 2cm medial to the lateral border of the rectus muscle and it was extended to caudal-lateral direction, down to 1/2cm to the lateral border of rectus muscle. The lateral edge of anterior rectus sheath incision was dissected off from the rectus muscle laterally up to semilunar line of rectus muscle in 1cm width. Then, the rectus muscle was retracted medially to expose the posterior sheath of rectus (below the arcuate line, only the transversalis fascia and parietal peritoneum are exposed). An oblique incision was made on the posterior sheath of rectus and peritoneum behind the rectus muscle, in a projection of the anterior sheath incision. In this manner, the abdominal wall incisions were located at anterior and posterior sheaths of rectus muscle at least 1/2cm medial to the semilunar line and to the lateral border of rectus. In other words, incision was not made from fascial layers' merging point or by muscle splitting dissection. Therefore, traction forces from enlarging uterus in advancing pregnant abdomen could be minimized. A large edematous, necrotic and dark purple torsion of a right adnexal mass was observed. It was pulled out and was partially dissected from its surface layer following a 2cm incision. The cystic structure was aspirated and right salpingo-ooferectomy was performed. The operation was completed by avoiding the manipulation of the pregnant uterus. Frozen section pathology of the removed specimen was reported as benign (necrotic tissues, teratoma) and final pathology was mucinous cystadenoma. Follow up on the pregnant patient was uneventful a month later.

EN100668

The liver function tests were normal expect low albumin (2.1 mg/dL) and increased alkaline phosphatase (1196 mg/dL) with low fibrinogen (1.7g/l).

We present here a case of Macrophage activation syndrome associated with Griscelli syndrome type 2 in a 3-years-old male born of consanguineous parentage. His birth histories, his familie social history and developmental milestones were unremarkable. He was born at full term with no antenatal or perinatal complications, He was on exclusively breast-fed, food diversification was started at 6 months old, his weight, length and psychomotor development were within the normal range, the child was described as a good eater, was on a normal diet and was thriving appropriately. Furthermore the boy presented a progressive abdominal distension since birth, progressive pallor and recurrent episodes of fever since 1 year of age. There was history of blood transfusions for last 2 months. On the other side there was no history of jaundice, vomiting, urinary or bowel complaints, bleeding from any site or neurological complaints. On admission, he was very pale with silvery gray scalp hair, white eyelashes, he was hypotonic, tachycardic, fever of 40°C, lymphoadenopathy and hepato-splenomegaly. The blood count showed pancytopenia; 1980 white blood cells/µL (VN 4000-13 500/µL), neutrophils 820/µL, 910 cells/µL, hemoglobin (Hb) 6.3 g/dL (11.5-14 VN, 5 g/dL), platelets 44 000/µL (VN 150 000-400 000/µL), There were no giant cytoplasmic granules in leucocytes. The liver function tests were normal expect low albumin (2.1 mg/dL) and increased alkaline phosphatase (1196 mg/dL) with low fibrinogen (1.7g/l). Serum triglycerides, ferrintin and lactic dehydrogenase were very high; respectively 4.75g/L; 2763µg/L and 597U/L. the C reactive protein (CRP) was elevated at 83mg/l. A chest X-ray was normal while the abdominal ultrasound showed a hepatomegaly and a splenomegaly, free biliary ducts without ascites. Because of hepato-splenomegaly, pancytopenia, hyperferritinemia and hypofibrinogenemia the diagnosis of Macrophage activation syndrome was evoked indicating a bone marrow aspiration that showed numerous morphologically benign macrophages with haemophagocytic activity that comforting the diagnosis of a SAM according to Ravelli and HLH-2004 criteria. Blood culture, urine examination, malarial serology, Kala Azar serology, viral serologies such as EBV, hepatitis A, B, C and human immunodeficiency virus HIV, serologies of rickettsia, syphilis and toxoplasmosa were negatives. Serologies of CMV and rubella showed an old immunization. The immunological tests such as antinuclear antibodies (ANA), anti-LKM1, anti-mitochondria and anti-smooth muscle antibodies were negatives. Because of the consanguineous family, notion of recurrent infection and the presence of silvery-gray color of the hair and eyebrows, diagnosis of Griscelli Syndrome was evoked directing a light microscopy examination of the hair that showed a large, irregular clumps of pigments characteristic of Griscelli Syndrome. The molecular biology showed mutation in RAB27A gene confirming the diagnosis of a Griscelli syndrome type 2. The first-line therapy in management of Macrophage activation syndrome complicating Griscelli syndrome type 2 was based on the parenteral administration of high doses of corticosteroids (methylprednisolone pulses 1g/0.73m 2 /day for 3 days then 60mg/m 2) with poor response; persisting with involvement of the 3 cell lines in the blood count, elevated ferritin and triglycerides. Given the lack of response to steroids, Etoposide was started for 3 days associated with cyclophosphamide, the evolution was good with improvement of count blood cells, ferritin was lessening while the bone marrow showed decreased numerous of macrophages with low haemophagocytic activity. On the other side the infectious process was good managed by perfusion of Antibiotics such as Ceftazidim in combination with aminoside. The patient was sent home with prednisone and cyclosporine, He was placed on the list of bone marrow transplantation (BMT).

EN100372

The patient was also hardly cooperative with enteral access.

A 35-year-old mentally ill man was transferred from a local psychiatric hospital after attempted suicide by fire 3 days before admission. He acquired full-and partial-thickness injury in approximately 38% of his TBSA, including face and neck (5%), both upper extremities (8%), right thigh (4%), left thigh and leg (3%), and most of his anterior and posterior trunk (16%). The patient was hospitalized for 66 days and underwent five debridement surgeries. The psychiatric diagnosis was schizophrenia type ICD 10 F20.3, treated with Haloperidol 1.5 mg and Diazepam 5 mg daily. The patient was occasionally restrained due to rebellious and self-destruction behavior. The nutrition management of this patient was started after a week of hospitalization, which is considered late. In the initial nutrition assessment, the patient was 40 kg weight and 150 cm tall thus the body mass index (BMI) was 17,57 kg/m 2 or underweight. Gastrointestinal symptoms and signs were not found. Information regarding the patient's dietary intake in the last two weeks and weight change within the last 6 months could not be obtained, but his mother claimed that the patient seemed to lose weight because he refused any food that had been offered to him; he only smoked and drank coffee. Albumin level was 3.6 gr/L with imbalanced electrolyte levels. Signs of fluid accumulation were not found. According to the subjective global assessment (SGA) tool, the patient belonged to group C (severely malnourished). Energy and protein were given starting from 30 kcal/kg, increasing gradually to 50 kcal/kg, and 2 gp/kg divided into oral nutrition and oral nutrition supplements (ONS) with a 50:50 ratio. The patient was also given micronutrient supplementations, specifically vitamin A 100,000 units for the first week, vitamin C 100 mg 3 times a day, and zinc 20 mg once a day. During the early weeks of hospitalization, the patient's weight loss continued. At the end of the second week, his body weight was 36 kilograms while his serum albumin level was 2.7 gr/L. On the 28 th day, the patient's body weight was 33 kilograms while his serum albumin level was 2.1 gr/L. Energy and protein intake was maintained, but the ratio of oral intake and ONS was modified to 70:30. During the treatment, the patient's appetite was unstable. The patient was also hardly cooperative with enteral access. The patient often writhed in pain and became more aggressive. The patient did not respond well to analgesics and sedations. Partially parenteral nutrition (PN) enriched with branched-chain amino acid (BCAA) was administered to support protein needs. However, his BMI continued to decline, and on the 42 nd day, his body weight dropped to 32 kilograms with a serum albumin level of 2 gr/L. On the 66 th day, granulation tissues appeared on the wound bed. Considering the mental and nutrition status of the patient, it was decided not to continue with the skin graft surgery. The patient had lost 24% of body weight (BMI 11.96 kg/m 2) and the serum albumin level was 2.5 gr/L. The patient displayed severe muscle wasting and subcutaneous fat loss. However, the general and mental condition was relatively better. The patient was discharged from the hospital.

EN100658

Based on this microscopical finding, the final diagnosis was periodontal disease associated with a proliferative non-neoplastic lesion.

A male, 63 years old, complaining about a "wound in the mouth" for 6 months, without any pain or spontaneous bleeding. His medical history was free of disease. The patient was a smoker for 33 years. Intraoral examination revealed a destructive ulcerative lesion around the upper left first and second molars that resembled an oral squamous cell carcinoma. Biopsy of the ulcerative lesion was performed and the microscopic features showed an inflammatory infiltrate rich in plasma cells. Based on this microscopical finding, the final diagnosis was periodontal disease associated with a proliferative non-neoplastic lesion. The patient was referred to a specialized dental surgeon and underwent periodontal therapy including surgical procedures. After that, according to follow-up with the patient, there were no signs of disease remission. The lesion increased in size, although the patient did not complain of any symptoms. A second biopsy was performed and the microscopic features again showed a rich inflammatory infiltrate with mononuclear cells and histiocytic cells, characterized by pale histiocytes with lobed nuclei, resembling a bean. A varying number of eosinophils also were observed, without any evidence of atypical cells present in this infiltrate. An immunohistochemical staining panel was done to determine the nature of this inflammatory infiltrate by using antibodies S-100, CD1a, CD-68 and CD45RO that were positive. These immunohistochemical findings were fundamental for the final diagnosis of LCH. The treatment included surgical extraction of all superior teeth, radiation and systemic corticoid therapies. After 8 years of treatment, the patient is free of disease.

EN105114

Percutaneous tracheotomy was also performed.

A 23 year old female patient (weight 67kg, height 171cm, BMI 22.91kg/cm 2) presented to emergency department with fever (39.4oC), tachycardia (116 bpm), hypotension (95/45 mmHg), tachypnoea (25 breaths/min), sore throat, neck pain, diffuse pain and tissue oedema in the right-side gluteal region. She was appointed to hospital by a private outpatient surgical center where she had undergone perineal abscess drainage a week before. Recent medical history included also endometrial curettage for ERPC a month before and conservative therapy for hemorrhoids 15 days after. Past medical history included Henoch-Schönlein purpura during childhood. Social history included smoking (11 pack-years), family history was non-contributory. She had no known drug allergies. Complete blood count revealed marked neutrophilic leukocytosis (WBC 23.400 /lt). A CT scan revealed small bilateral pleural effusions, small hepatic enlargement, Gerota's fascia thickening, perineal soft tissue stranding with fascial thickening and small bubbles of air. Fournier's gangrene was set as working diagnosis (calculated FGSI 8, UFGSI 10 and LRINEC 7) and the patient was transported to operation room for immediate radical surgical debridement of necrotic tissue. Blood and surgical material was taken for microbiologic analysis. Due to the extent of the surgical debridement, the patient was admitted postoperatively to the ICU (SOFA score 6, APACHE II score 12); where sedation (midazolam 30 mcg/kg/hr c.iv), analgesia (fentanyl 3 mcg/kg/min c.iv), triple antibiotic regimen with meropenem 2gr q8h i.v., clindamycin 600mg q 6h i.v. and daptomycin 500mg q.d. i.v., gastroprotective and antithrombotic prophylaxis were initiated. During the next 3 days a second CT and an obstetrical consultation were conducted with no significant findings. Human immunodeficiency virus and diabetic screen were also negative. Yet, the patient remained febrile. Microbiology cultures from material taken from the surgical trauma area revealed Candida albicans, Staphylococcus epidermidis and Klebsiella pneumonia ; hence antibiotic regimen was modified and antimycotic treatment (caspofungin 100mg q.d. i.v.) was initiated too. Debridements were repeated every 24 hours but due to the extent of the lesions, on the 10 th day, the patient was transported to operation room for temporary ileostomy till the end of the therapy. Percutaneous tracheotomy was also performed. Hyperbaric oxygen therapy was not available at the setting. One day after the second surgery, patient's condition complicated with MRSA ventilation associated pneumonia. Along with that, a localized oedema appeared in her left upper limb. A palpable mass along the anterior margin of the sternocleidomastoid muscle was also noticed. Vascular ultrasound examination revealed occlusion of left internal jugular vein. Transcranial Doppler examination was normal. Dental consultation and examination of soft palate and peritonsilar tissues showed no abnormalities. Linezolide 600 mg q12h i.v. was added to antibiotic regimen. Since debridements were going on, simultaneous anticoagulation therapy was started with only bemiparine 2500 b.i.d. s.c. Gradually, the patient became afebrile and her clinical condition improved. She was discharged from ICU 13 days later. A follow-up CTA confirmed the disappearance of the left jugular vein thrombosis. About a month after her ICU discharge, an operation was performed for ileostomy closure and reconstruction of the right gluteal region. On a follow-up examination 6 months later, the young woman had returned to her normal everyday life.

EN100123