OpenMed/multicare-articles huggingface dataset:
Transitioning Between Prostanoid Therapies in Pulmonary Arterial Hypertension
Authors: Irene Z Pan, Jessica R Carey, Joshua A Jacobs, John Dechand, Joshua J Sessions, Teshia Sorensen, Brittany A Penn, Jennalyn D Mayeux, Nathan D Hatton, John J Ryan
Journal: Front Med (Lausanne) (2020)
Keywords: pharmacology, prostacyclin, prostaglandin, pulmonary hypertension, right heart failure, therapeutics
MeSH terms: Case Reports

Background: New oral prostacyclin therapies and prostacyclin agonists have become available for the treatment of pulmonary arterial hypertension (PAH). However, methods for transitioning between oral, inhaled, and parenteral formulations are not well-established, except in the form of case reports and case series. Collectively, these emphasize the lack of a standardized process and approach in transitioning patients between PAH prostanoid therapies. In this case series, we report our experience at an accredited Pulmonary Hypertension center in transitioning between various oral, inhaled, and parenteral prostanoids to offer additional guidance on safe transitions in therapy. Methods: All cases of prostanoid transitions at an accredited Pulmonary Hypertension center from March 2018 to September 2019 were included in this report. The transition approach for each case was developed through a review of the literature, extrapolation of available pharmacokinetic data, and collaboration between pharmacists and clinicians. Results: This case series describes the transition of 3 patients from selexipag to parenteral treprostinil; 1 patient transitioning from parenteral treprostinil to selexipag; 1 patient transitioning from oral treprostinil to parenteral treprostinil; and 1 patient transitioning from inhaled treprostinil to selexipag. Four of the 6 patients presented here were transitioned to an alternate prostanoid on account of clinical worsening, while the remaining 2 patients transitioned due to intolerance of parenteral therapy and poor medication adherence. This case series includes patients with various etiologies of PAH including idiopathic PAH, methamphetamine-associated PAH, and scleroderma-associated PAH. All patients successfully completed each transition without serious adverse events. Conclusions: With the increasing utilization and availability of prostanoids, there is a critical need for a standardized approach in transitioning safely between different formulations without compromising treatment efficacy. In this case series, we present our clinical experiences, guided by available pharmacokinetic data, in transitioning between various prostanoid formulations.
Transoral endoscopic thyroidectomy via vestibular approach: First case in Saudi Arabia
Authors: Hassan M Al Bisher, Alaa M Khidr, Badria H Alkhudair, Fatema S Alammadi, Arwa H Ibrahim
Journal: Int J Surg Case Rep (2020)
Keywords: endoscopic thyroidectomy, scar-less thyroidectomy, toetva, transoral thyroidectomy, vestibular approach
MeSH terms: Case Reports

Introduction:
There has been increased interest in applying the principles of minimally invasive surgery in thyroid surgery which was promoted by Miccoli and his colleagues in 1999. Different surgical techniques since then were introduced, transcervical and extracervical approaches.
Presentation of case:
A 33-year-old Saudi female presented with a swelling over the left side of her neck and dysphagia for three years. The patient underwent transoral endoscopic left thyroid lobectomy, isthmusectomy and sampling of the central compartment via vestibular approach (TOETVA). To the best of our knowledge, this is the first case underwent TOETVA successfully in Saudi Arabia and gulf cooperation council countries.
Discussion:
TOETVA is a novel promising procedure, an adaptation of the concept of natural orifice transluminal endoscopic surgery (NOTES) with advantages of scar-less result in excellent cosmesis while retaining the advantages of minimally invasive surgery and the potential value of the procedure outside the enhanced cosmesis continuous to be defined. The cosmetic superiority in avoiding visible scarring must be balanced against operative time, post-operative hospital stays, increased expense, surgical training, and steep learning curve required. The technique is novel, some thyroid surgeons opted for longer postoperative observation to exclude complications. As experience and confidence in the procedure increase, the length of stay should decrease dramatically.
Conclusion:
This procedure is feasible and safe with excellent cosmetic result. It is in its initial stage in Saudi Arabia, gulf cooperation council countries and it has a potential to be performed more frequently in near future.
A rare case report of infant ileal atresia with double appendix
Authors: Pinak Pani Dhar, Upasana Mohanty
Journal: Int J Surg Case Rep (2020)
Keywords: case report, double appendix, ileal atresia, male infants
MeSH terms: Case Reports

Introduction:
Small bowel atresia has quite an incidence in infants particularly in males. It is usually seen to be the most common cause of bowel obstruction in neonates since birth. It may be associated with other congenital anomalies.
Presentation of the case:
A 4 day old neonate presented with abdominal distension, billous vomiting & non passage of stools since birth. On further examination, it was seen that the abdominal distension was mostly central & there was hyperperistaltic bowel sounds on auscultation. The infant also had scoliosis. There was decreased appetite & features of dehydration. Diagnosed by USG(W/A), infantogram & X-Ray plain picture abdomen.
Discussion:
The mortality from the atresia of ileum is greater owing to the incidence of early perforation. Infants may present with features discussed above. They usually present during the 1st week of life but majority of the babies presenting late have significant mortality. Early surgical intervention with excision of the dilated bowel segment with anastomosis of the proximal (normal caliber bowel) to the distal part of the bowel has been seen to have been associated with decreased morbidity & mortality.
Conclusion:
It has been observed that one of the many causes for neonatal intestinal obstruction is attributed to small bowel atresia. This has been associated with successful outcome depending on the presentation of the child to the hospital to the timing of surgical intervention along with resuscitation & starting of enteral feeds. Small bowel atresia can be associated with antenatal morbidity in the mother as cited here.
Feasibility of Mediastinal Radiation Use in HeartMate 3 LVAD Recipients with Cancer
Authors: Nausharwan Butt, Farooq H Sheikh
Journal: Methodist Debakey Cardiovasc J (2023)
Keywords: heartmate lvad, mediastinal cancer, radiation exposure, stereotactic bone radiation therapy
MeSH terms: Heart Failure / therapy, Heart-Assist Devices, Neoplasms, Case Reports, Feasibility Studies, Humans, Retrospective Studies

The HeartMate 3 left ventricular assist device possesses the unique feature of having its electronics and software located within the housing of the pump, which may predispose it to malfunction from radiation exposure during cancer treatment. We investigated this association in a case series of two patients at our institution.
PTHrP-related Hypercalcaemia in Infancy and Congenital Anomalies of the Kidney and Urinary Tract (CAKUT)
Authors: Nardeen Kodous, Guido Filler, Ajay Parkash Sharma, Tamara Angela Van Hooren
Journal: Can J Kidney Health Dis (2015)
Keywords: chronic kidney disease, failure to thrive, hypercalcemia, infant, treatment

Background:
Severe hypercalcaemia is a rare but clinically significant condition in infancy and childhood. Parathyroid hormone-related peptide (PTHrP)-mediated hypercalcaemia resulting from a malignancy is rare and only a handful of case reports have outlined its incidence alongside a benign condition.
Objectives:
To describe the diagnostic workup and management of an infant with hypercalcaemia, renal dysplasia, and elevated PTHrP levels.
Design:
Case report.
Setting:
The Victoria Hospital campus of the London Health Sciences Centre in London, Ontario, Canada.
Patients:
A child with congenital anomalies of the kidneys and urinary tract (CAKUT), stage 2 chronic kidney disease (CKD), and renal dysplasia who presented with severe hypercalcaemia.
Measurements:
Weight, renal ultrasound, creatinine, cystatin C, eGFR, calcium, urea, bicarbonate, serum sodium, fractional sodium excretion, urine calcium to creatinine ratio, PTH, TSH, Free T4, AM cortisol, HMA, VMA, 25-vitamin D, 1,25 dihydroxy-vitamin D, calcitriol, vitamin A, ACE levels, skull and chest x-rays, alkaline phosphatase, CBC, tumour lysis profile, catecholamine breakdown, whole-body MRI, PTHrP.
Methods:
Full diagnostic workup and patient management. Patient treated with intravenous hydration, furosemide, calcitonin and CalciLo.
Results:
PTHrP was elevated and no evidence of a malignancy was found. Treatment consisting of a low-calcium CalciLo diet (in place of breast milk) adequately controlled the patient's hypercalcaemia. Hypercalcaemia associated with CAKUT in infancy is not all that uncommon and was reported in 15/99 infants in another study, most of whom had a suppressed PTH similar to that of our patient. PTHrP was not measured in these cases and may have also been elevated.
Limitations:
The study is limited in that it is a description of a single patient case. Future measurement of PTHrP in similar patients is necessary to confirm our results.
Conclusions:
The possibility of elevated PTHrP levels must be considered in patients with known renal dysplasia who are differentially diagnosed with hypercalcaemia.
A case report: Delayed gallstone abscess formation 10 years post-cholecystectomy
Authors: Erina Quinn, James Capanegro, Joseph Hartigan
Journal: Int J Surg Case Rep (2020)
Keywords: abscess, cholecystectomy, gallstones, laparscopic surgery
MeSH terms: Case Reports

Introduction:
Retained gallstones post-cholecystectomy act as a nidus for abscess formation. It is unusual for intraabdominal abscesses to remain asymptomatic due to its propensity to cause inflammation and irritation to the peritoneum.
Presentation of case:
A 73-year-old female presented with acute onset of right-sided abdominal pain and fever. Her past surgical history was significant for a cholecystectomy in 2010, hysterectomy, and partial nephrectomy. She was diagnosed with an intraabdominal abscess secondary to a retained gallstone post-cholecystectomy. She underwent laparoscopic surgery to drain and remove the abscess. The patient's abdominal pain improved, remains afebrile, and is passing stool regularly.
Discussion:
Gallbladder perforation is common and is dependent on the integrity of the gallbladder and surrounding structures. It is unusual for an intra-abdominal abscess to develop so late following gallstone spillage. This example brings to light the potential long-term sequelae of gallbladder perforation and future complications.
Conclusion:
This case highlights the importance of irrigation of the peritoneal cavity and retrieval any spilled gallstones during surgery in the event of gallbladder perforation.
Case Report: Three Rare Cases of Ectopic ACTH Syndrome Caused by Adrenal Medullary Hyperplasia
Authors: Yu Cheng, Jie Li, Jingtao Dou, Jianming Ba, Jin Du, Saichun Zhang, Yiming Mu, Zhaohui Lv, Weijun Gu
Journal: Front Endocrinol (Lausanne) (2021)
Keywords: acth-dependent cushing’s syndrome, adrenal medullary hyperplasia, bilateral adrenalectomy, ectopic acth syndrome, immunohistochemical staining
MeSH terms: ACTH Syndrome, Ectopic / etiology, Adrenal Medulla / pathology, Adrenocorticotropic Hormone / blood, Case Reports, ACTH Syndrome, Ectopic / blood, ACTH Syndrome, Ectopic / diagnostic imaging, Adrenal Medulla / diagnostic imaging, Adult, Female, Humans, Hyperplasia / blood, Hyperplasia / complications, Hyperplasia / diagnostic imaging, Magnetic Resonance Imaging, Male, Tomography, X-Ray Computed, Young Adult, Adrenocorticotropic Hormone

Ectopic ACTH syndrome (EAS) accounts for 10-20% of endogenous Cushing's syndrome (CS). Hardly any cases of adrenal medullary hyperplasia have been reported to ectopically secrete adrenocorticotropic hormone (ACTH). Here we describe a series of three patients with hypercortisolism secondary to ectopic production of ACTH from adrenal medulla. Cushingoid features were absent in case 1 but evident in the other two cases. Marked hypokalemia was found in all three patients, but hyperglycemia and osteoporosis were present only in case 2. All three patients showed significantly elevated serum cortisol and 24-h urinary cortisol levels. The ACTH levels ranged from 19.8 to 103.0pmol/L, favoring ACTH-dependent Cushing's syndrome. Results of bilateral inferior petrosal sinus sampling (BIPSS) for case 1 and case 3 confirmed ectopic origin of ACTH. The extremely high level of ACTH and failure to suppress cortisol with high dose dexamethasone suppression test (HDDST) suggested EAS for patient 2. However, image studies failed to identify the source of ACTH secretion. Bilateral adrenalectomy was performed for rapid control of hypercortisolism. After surgery, cushingoid features gradually disappeared for case 2 and case 3. Blood pressure, blood glucose and potassium levels returned to normal ranges without medication for case 2. The level of serum potassium also normalized without any supplementation for case 1 and case 3. The ACTH levels of all three patients significantly decreased 3-6 months after surgery. Histopathology revealed bilateral adrenal medullary hyperplasia and immunostaining showed positive ACTH staining located in adrenal medulla cells. In summary, our case series reveals the adrenal medulla to be a site of ectopic ACTH secretion. Adrenal medulla-originated EAS makes the differential diagnosis of ACTH-dependent Cushing's syndrome much more difficult. Control of the hypercortisolism is mandatory for such patients.
Staged, open, no-ischemia nephron-sparing surgery for bilateral-multiple kidney tumors in a patient with birt-hogg-dubé syndrome
Authors: Ahmet Tefekli, Ayşe Deniz Akkaya, Kamil Peker, Terman Gümüş, Metin Vural, Fatin Cezayirli, Ahmet Musaoglu, Tarık Esen
Journal: Case Rep Med (2012)
MeSH terms: Case Reports

Hereditary kidney cancer patients with bilateral multiple kidney tumors represent challenges in the era of rapidly growing minimal invasive treatment techniques. Birt-Hogg-Dubé Syndrome (BHDS) is an autosomal dominant genodermatosis characterized by a triad of benign skin tumors (fibrofolliculomas, trichodiscomas, acrochordons) together with an increased risk of developing malignant renal tumors and pulmonary disease such as pneumothoraces and multiple lung cysts. The morbidity and mortality of the affected patients is determined by the presence of the kidney tumors, which tend to be multifocal and bilateral, as observed in other hereditary kidney cancer syndromes like von Hippel-Lindau disease, familial leiomyomatosis, and hereditary papillary renal cell carcinoma. Herein, a patient with BHDS, presenting with synchronous bilateral multiple kidney tumors, is reported. The report describes the management of kidney tumors with two-stage open nephron-sparing surgery in which the nonvascular clamping technique was utilized.
Juvenile lupus, cytomegalovirus infection and cardiac tamponade: case report
Authors: Levi Coelho Maia Barros, Matheus Eugênio de Sousa Lima, Roseny Marinho Mesquita Pereira, Lia Arcanjo Alves Vasconcelos, Willenne Campelo Rabelo
Journal: Rev Paul Pediatr (2021)
MeSH terms: Cardiac Tamponade / diagnosis, Cardiac Tamponade / etiology, Cardiac Tamponade / surgery, Cytomegalovirus Infections / complications, Cytomegalovirus Infections / diagnosis, Lupus Erythematosus, Systemic / complications, Lupus Erythematosus, Systemic / diagnosis, Pericardial Effusion, Case Reports, Child, Female, Humans, Pericardiocentesis

Objective:
To describe a rare case of cardiac tamponade in a pediatric patient with systemic lupus erythematosus (SLE) and cytomegalovirus (CMV) infection, and to discuss the relationship between these morbidities, the diagnostic approach, and the possible treatments.
Case description:
A 9-year-old girl presented to the emergency department with severe dyspnea, muffled heart sounds, jugular vein distention, hemodynamic instability, and intense pallor. She had previously been followed up at the outpatient clinic for a six-month history of mild respiratory distress, polyarthritis, fever, and various cutaneous manifestations. Doppler echocardiogram revealed pericardial effusion. The patient was submitted to pericardiocentesis followed by water seal pleuropericardial drainage, with no complications. The investigation continued, with fulfillment of clinical and laboratory SLE criteria plus CMV antigenemia of 15/200,000 cells. Medications to control CMV infection and SLE were then initiated, with good clinical and laboratory response.
Comments:
Pediatric SLE commonly manifests in a more severe form, accounting for high morbimortality. Cardiac tamponade could be one of the first manifestations of SLE, which can also be precipitated by infectious agents, such as CMV, leading to diagnostic confusion and misleading the treatment. Changes in therapeutics must also be considered in the presence of both conditions. This study presents a juvenile SLE case aggravated by a CMV infection with the unusual manifestation of cardiac tamponade.
Pancreaticoduodenal Artery Aneurysm Associated with Celiac Trunk Stenosis: Case Illustration and Literature Review
Authors: Jad A Degheili, Alissar El Chediak, Mohamad Yasser R Dergham, Aghiad Al-Kutoubi, Ali H Hallal
Journal: Case Rep Radiol (2017)
MeSH terms: Case Reports

Pancreaticoduodenal artery aneurysms (PDA) are rare visceral aneurysms. Celiac trunk stenosis represents a common attributable aetiology for those aneurysms. Therefore, an alternative treatment approach, which differs from those isolated aneurysms, is recommended. We hereby present a 77-year-old male patient who was admitted with sudden onset of severe abdominal pain and significant drop in haemoglobin, occurring within a 24-hour interval. Contrast-enhanced computed tomography revealed a ruptured visceral aneurysm arising from the anterior branch of the inferior pancreaticoduodenal artery. A severe stenosis was also noted at the take-off of the celiac trunk. Selective catheterization of the supplying branch of the superior mesenteric artery, followed by coil embolization of the aneurysm, was performed, resulting in cessation of flow within the aneurysm, with preservation of the posterior branch, supplying the celiac territory. PDAs are usually asymptomatic and discovered incidentally at rupture. The risk of rupture is independent of the aneurysmal size and is associated with a 50% mortality rate. The consensus on coping with aneurysms is to treat them whenever they are discovered. Selective angiography followed by coil embolization represents a less invasive, and frequently definitive, approach than surgery. The risk for ischemia mandates that the celiac territory must not be compromised after embolization.
Thromboelastometry Identified Alteration of Clot Stabilization and Factor XIII Supplementation Need in a Patient with Decompensated Liver Disease Undergoing Liver Biopsy
Authors: Tomaz Crochemore, Felicio Aragão Savioli
Journal: Case Rep Gastrointest Med (2018)
MeSH terms: Case Reports

Liver disease has been considered the prototype of hemorrhagic disease. Disorder in any component of coagulation system can lead to hemorrhage. Deficiency of factor XIII may impair clot strength and clot stabilization and can be accessed by thromboelastometry. We report a case of a patient with a rapid evolution of liver disease who underwent a liver biopsy. Thromboelastometry was performed, evidencing impairment of clot stability. This clotting disorder was corrected with factor XIII concentrate after unsuccessful administration of antifibrinolytic drugs and hepatic biopsy was performed without hemorrhagic complications. Case Presentation. We report the case of a previously healthy 38-year-old man, who presented to our emergency department with clinical signs of rapid progression of acute liver failure. The laboratory tests revealed platelets of 142x103/mm3, plasma fibrinogen concentration of 221 mg/dl, increased international nationalized ratio (INR 1.9), total bilirubin of 3.9mg/dl, direct bilirubin of 2.3mg/dl, ALT 751U/l, and AST 540U/l without acute bleeding. A liver biopsy was indicated. Based on the results of the thromboelastometry, Tranexamic Acid was administered to correct hyperfibrinolysis followed by factor XIII concentrate to correct factor XIII deficiency. Thromboelastometry was normal despite conventional coagulation tests were still altered. So, liver biopsy was performed with no signs of bleeding and without need of further transfusion. Conclusion. Thromboelastometry may be considered a useful, feasible, and safe tool to monitor and manage coagulopathy in patients with liver disease, with the potential advantage of helping avoid unnecessary transfusion in such patients.
Anti-NMDAR Encephalitis: Higher Suspicious Needed for Earlier Diagnosis (Case Report, Literature Review and Diagnostic Criteria)
Authors: Chanaka Amugoda, Noushin Chini Foroush, Hamed Akhlaghi
Journal: Case Rep Neurol Med (2019)
MeSH terms: Case Reports

Background:
Auto-immune mediated anti-NMDA receptor encephalitis is a very common delayed diagnosed encephalitis which predominately affecting young population.
Objectives:
This encephalitis is relatively unknown amongst emergency physicians and a majority of patients are admitted to psychiatric wards before their diagnosis is confirmed and appropriate treatments are commenced. We reported a case of a 22-year-old female presented to our emergency department with acute psychiatric symptoms. She was initially diagnosed with first presentation of acute psychosis and was hospitalised under mental health act. further assessment in the emergency department identified possible an organic cause for her acute psychosis and she was later admitted under medical team after her mental health assessment order was revoke. Several days later, her CSF result was positive with anti-NMDA receptor anti-bodies. Appropriate treatments were instituted leading to her full recovery.
Conclusion:
This case was the first confirmed anti-NMDA receptor encephalitis in our emergency department. It highlights the importance of thorough assessment of psychiatric presentations to emergency departments and consideration of auto-immune medicated encephalitis as one of the differential diagnosis in young patients presenting with first acute psychotic episode.