OpenMed/multicare-articles huggingface dataset:
Papillon-Lefèvre Syndrome: Diagnosis, Dental Management, and a Case Report
Authors: Jean-Claude Abou Chedid, Michel Salameh, Abbass El-Outa, Ziad E F Noujeim
Journal: Case Rep Dent (2019)
MeSH terms: Case Reports

Aim:
This paper revisits Papillon-Lefèvre syndrome (PLS), addresses its diagnostic update and dental management, and reports a case of a 5-year-old Lebanese patient with consanguineously married parents.
Background:
PLS, also known as "keratoris palmoplantaris with periodontopathia" and "hyperkeratosis palmoplantaris with periodontosis," is an extremely rare autosomal-recessive trait that combines a diffuse palmoplantar hyperkeratosis and a severe generalized, progressive prepubertal form of a precocious form of juvenile, aggressive periodontitis.
Case description:
We are reporting a 5-year-old boy that sustained a spontaneous loss of all his primary teeth. At consultation, he was under treatment for hyperkeratosis of his palms and soles. Detailed family history of the child revealed that the patient's parents, grandparents, and relatives were consanguineously married and two of his cousins displayed similar clinical signs (palmoplantar hyperkeratosis and premature loss of deciduous and most of the permanent teeth).
Conclusion:
PLS is an extremely rare disorder that usually becomes apparent from approximately 1-5 years of age. Genetic counseling should always be suggested to parents of affected children, informing them of chances of their offspring having the inherited disease.
Do food donation tax credits for farmers address food loss/waste and food insecurity? A case study from Ontario
Authors: Lesia Kinach, Kate Parizeau, Evan D G Fraser
Journal: Agric Human Values (2020)
Keywords: food donations, food insecurity, food loss, food waste, tax credits

To increase donations of nutritious food, Ontario introduced a tax credit for farmers who donate agricultural products to food banks in 2013. This research seeks to investigate the role of Ontario's Food Donation Tax Credit for Farmers in addressing both food loss and waste (FLW) and food insecurity through a case study of fresh produce rescue in Windsor-Essex, Ontario. This research also documents the challenges associated with rescuing fresh produce from farms, as well as alternatives to donating. Interviews with food banks, producers and key informants revealed that perceptions of the tax credit, and the credit's ability to address FLW and food insecurity, contrasted greatly with the initial perceptions of the policymakers who created the tax credit. In particular, the legislators did not anticipate the logistical challenges associated with incentivizing this type of donation, nor the limitations of a donation-based intervention to provide food insecure Ontarians with access to fresh, nutritious food.
Tracheostomy wound myiasis in a child: case report and review of the literature
Authors: J Blejter
Journal: Case Rep Pediatr (2012)
MeSH terms: Case Reports

An 8-year-old tracheostomized, gastrostomized, and with cerebral palsy boy was admitted for tracheostomy wound myiasis and cellulitis. Ether was applied in the wound, and then all the accessible larvae were removed. Antibiotic therapy was initiated. The procedure had to be repeated three more times to extract all the larvae. An airway endoscopy was performed and ruled out the presence of larvae in the airway, as well as any damage to the wall of the trachea. The patient recovered uneventfully and was discharged.
Cardiovascular and Cerebral Responses During a Vasovagal Reaction Without Syncope
Authors: Mathias R Aebi, Nicolas Bourdillon, Hadj B Meziane, Edward Nicol, Jérôme Barral, Grégoire P Millet, Denis Bron
Journal: Front Neurosci (2019)
Keywords: eeg flattening and slowing, cerebral hypoperfusion, hypotension and bradycardia, pre-syncope symptoms, vasovagal mechanism

This clinical case report presents synchronous physiological data from an individual in whom a spontaneous vasovagal reaction occurred without syncope. The physiological data are presented for three main phases: Baseline (0-200 s), vasovagal reaction (200-600 s), and recovery period (600-1200 s). The first physiological changes occurred at around 200 s, with a decrease in blood pressure, peak in heart rate and vastus lateralis tissue oxygenation, and a drop in alpha power. The vasovagal reaction was associated with a progressive decrease in blood pressure, heart rate and cerebral oxygenation, whilst the mean middle cerebral artery blood flow velocity and blood oxygen saturation remained unchanged. Heart rate variability parameters indicated significant parasympathetic activation with a decrease in sympathetic tone and increased baroreflex sensitivity. The total blood volume and tissue oxygenation index (TOI) dropped in the brain but slightly increased in the vastus lateralis, suggesting cerebral hypoperfusion with blood volume pooling in the lower body part. Cerebral hypoperfusion during the vasovagal reaction was associated with electroencephalography (EEG) flattening (i.e., decreased power in beta and theta activity) followed by an EEG high-amplitude "slow" phase (i.e., increased power in theta activity). The subject developed signs and symptoms of pre-syncope with EEG flattening and slowing during prolonged periods of symptomatic hypotension, but did not lose consciousness.
Metastasis of endometrial cancer to right hemidiaphragm: A case report
Authors: Ivan Sergeevich Gruzdev, Ivan Andreevich Blokhin, Aleksey Nikolaevich Lednev, Aleksey Aleksandrovich Pechetov, Maksim Aleksandrovich Makov, Yuriy Sergeevich Esakov, Artem Garrievich Arevin, Andrey Vyacheslavovich Glotov, Grigory Grigorievich Kаrmаzаnovsky
Journal: Int J Surg Case Rep (2019)
Keywords: ct, diaphragmotomy, endometrial cancer, metastasis to the diaphragm

Introduction:
Tumors of the diaphragm are uncommon. The overwhelming number of cases is metastatic combined with metastases to the liver, lungs and other organs. Only a minority of cases are described as solitary lesions.
Case presentation:
Fifty-five years old female with a history of radical curative surgery for pT3N0M0 endometrial cancer eight years ago was admitted to the Department of Thoracic Surgery with a feeling of discomfort in the right hypochondrium. The contrast-enhanced MDCT revealed a large, well-circumscribed lesion of the right hemidiaphragm deforming upper contour of the liver. A clear boundary between the lesion and the liver suggested former's diaphragmatic origin. PET-CT did not show any distant metastasis. Intraoperative revision revealed a tumor growing from the dome of the diaphragm with well-defined contours and without any signs of lung involvement. The diaphragmotomy was performed. The morphological study with immunohistochemistry showed an endometrial carcinoma metastasis to the diaphragm.
Conclusion:
The diaphragm lesions can have various etiology, but a probability of tumor metastasis after a previous radical surgery should not be excluded. Preoperative differential diagnostics can be difficult, leaving surgical treatment followed by a pathology study as the most informative diagnostic method of tumor morphology.
Rare case of mixed epithelial and stromal tumor (MEST) of the kidney and its diagnostic and therapeutic approach: A case report
Authors: Wajiha Arshad, Asad Amir, Maria Naseer Malik, Shahzaib Maqbool, Muhammad Idrees Anwar, Ka Yiu Lee
Journal: Int J Surg Case Rep (2023)
Keywords: cystic lesion, mixed epithelial stromal tumor (mest), radical nephrectomy
MeSH terms: Case Reports

Introduction and importance:
Mix epithelial and stromal tumor (MEST) is a benign biphasic renal lesion composed of solid as well as cystic components lining tubular and cystic spaces of kidney. There are very few cases of such variety have been reported with perspective to renal involvement. Herein we have reported a rare case of MEST involving left renal tissue and sparing surrounding tissues.
Case presentation:
A 20 years old female presented to surgical outpatient department with complaint of amenorrhea and left flank pain as well as heaviness for 1 year. Patient was vitally stable and cooperative. On physical examination left flank mass was palpated and ultrasound and CT scan imaging was also showing left renal mass confined to upper, middle and lower portion of the kidney while renal capsule, adrenal gland and ureter were spared. On histological examination showed multi-cystic structures with variably sized simple cysts lined by hobnailed epithelium with clear cells. Septa show ovarian type fibrous stroma with variable inflammation and immature nephrogenic elements. A final diagnosis of MEST was made. Therefore, radical nephrectomy with trans-peritoneal approach was done.
Clinical discussion:
MEST is a benign tumor of renal tissue that is confined to the renal parenchyma rather than involvement of surrounding structures as occurred in our case. Due to benign nature of the disease involvement of renal capsule and adrenal gland is less likely. The choice of treatment is radical nephrectomy through transperitoneal approach.
Conclusion:
MEST is a rare diagnosis thought case now start reporting since last decade, however, it's still a rare entity to be reported. USG and CT scan are investigating modalities along with histopathological correlation to reach the diagnosis.
Primary Splenic Angiosarcoma Presenting as Idiopathic Thrombocytopenic Purpura: A Case Report and Review of the Literature
Authors: S Christopher N Frontario, Anna Goldenberg-Sandau, Darshan Roy, Roy Sandau
Journal: Case Rep Surg (2016)

Angiosarcoma of the spleen is a rare malignancy that arises from vascular endothelial origin. This neoplasm is highly malignant and diagnosis is often delayed due to the vague presentation of clinical symptoms. A case report and concise review of the current diagnostic criteria and surgical treatment are provided to aid in the detection and treatment of this malignancy. We present a case of a 56-year-old female who presented with massive splenomegaly secondary to angiosarcoma of the spleen. The patient suffered from longstanding symptomatic anemia and thrombocytopenia. Diagnosis of a splenic angiosarcoma can be difficult due to the vague presentation and lack of concrete risk factors. Early identification and splenectomy are paramount. However, it is an aggressive malignancy with poor prognosis. We reviewed the literature of the current diagnostic and surgical treatment of primary splenic angiosarcoma.
Case report: Percutaneous electrical neural field stimulation in two cases of sympathetically-mediated pain
Authors: Lynn Fraser, Anna Woodbury
Journal: F1000Res (2017)
Keywords: percutaneous electrical neural stimulation, vagal stimulation, fibromyalgia, complex regional pain syndrome, central pain

Background: Fibromyalgia and complex regional pain syndrome (CRPS) are both chronic pain syndromes with pathophysiologic mechanisms related to autonomic nervous system dysregulation and central sensitization. Both syndromes are considered difficult to treat with conventional pain therapies. Case presentations: Here we describe a female veteran with fibromyalgia and a male veteran with CRPS, both of whom failed multiple pharmacologic, physical and psychological therapies for pain, but responded to percutaneous electrical neural field stimulation (PENFS) targeted at the auricular branches of the cranial nerves. Discussion: While PENFS applied to the body has been previously described for treatment of localized pain, PENFS effects on cranial nerve branches of the ear is not well-known, particularly when used for regional and full-body pain syndromes such as those described here. PENFS of the ear is a minimally-invasive, non-pharmacologic therapy that could lead to improved quality of life and decreased reliance on medication. However, further research is needed to guide clinical application, particularly in complex pain patients.
Clear cell unicystic ameloblastoma
Authors: Mb Radhika, Lalita J Thambiah, K Paremala, M Sudhakara
Journal: J Oral Maxillofac Pathol (2011)
Keywords: ameloblastoma, clear cell, unicystic variant
MeSH terms: Case Reports

Clear cell differentiation in unicystic ameloblastoma with inclusion of many other histologic variants in the same tumor is a very rare occurrence. Here, we report a case of a well-circumscribed large mandibular swelling in a 22 - year old female. The lesion was histopathologically diagnosed as unicystic ameloblastoma which showed multiple histologic patterns and clear cell differentiation. The tumor was treated with surgical enucleation and chemical cauterization. A follow up of 20 months has shown no recurrence after initial surgery.
Abdominoscrotal Hydrocele: An Uncommon Cause of Abdominoscrotal Cystic Swelling
Authors: Manish Swarnkar, Pathan Tanveer Khan
Journal: Case Rep Urol (2021)
MeSH terms: Case Reports

Abdominoscrotal hydrocele (ASH) consists of fluid-filled intercommunicating inguinoscrotal and abdominal sac with a characteristic hourglass-like picture on CECT, which usually affects single testis and a rare cause of abdominoscrotal cystic swelling. The precise etiology of ASH is not known. Ultrasonography is the initial diagnostic modality of choice as it demonstrates the intercommunication between the two sacs and also identifies any abnormality of the testis and genitourinary tract. We are reporting a case of a 27-year-old patient presented in the General Surgery OPD of Acharya Vinoba Bhave Hospital in 2019 with bilateral scrotal and abdominal swelling. On examination, cross fluctuation was positive between left hydrocele and abdominal swelling, raising suspicion of ASH, which was confirmed on CECT. The patient underwent excision of sac through left inguinoscrotal approach and an uneventful postoperative course.
Relapse after Prolonged Remission in Philadelphia-Like Acute Lymphoblastic Leukemia
Authors: Gaurav Shah, Fady M Mikhail, Andrew J Carroll 3rd, Matthew Kutny, Nikolaos Papadantonakis
Journal: Case Rep Hematol (2019)
MeSH terms: Case Reports

We describe a case of late relapse of Philadelphia-like acute lymphoblastic leukemia. The patient relapsed several years from diagnosis and responded to second salvage treatment. The case highlights the open questions regarding management of Philadelphia-like acute lymphoblastic leukemia.
Prenatal Identification and Molecular Characterization of Two Simultaneous De Novo Interstitial Duplications of Chromosomal Regions 7p22.1p21.1 and 15q24.1
Authors: Sabrina C Burn, Kali Swift, Maria Palmquist
Journal: Case Rep Genet (2018)
MeSH terms: Case Reports

The occurrence of simultaneous de novo chromosomal aberrations is extremely rare. Here, we describe two, previously unreported, simultaneous de novo interstitial duplications of chromosomes 7p and 15q. Amniocentesis was completed for a healthy gravida 4 para 3 woman due to her advanced maternal age and concurrent ultrasound findings of partial vermian agenesis, choroid-plexus cysts, and hypoplastic nasal bone. Cytogenetic analysis of cultured amniocytes by conventional chromosome analysis, comparative genomic hybridization, and fluorescence in situ hybridization revealed two interstitial duplications of the chromosomal regions 7p22.1p21.1 and 15q24.1, leading to partial trisomy of 7p and 15q and karyotype 46,XY,dup(7)(p22.1-p21.1),dup (15)(q24.1). Parental chromosomal analysis did not identify any heritable changes, suggesting both mutations were de novo in nature. Postnatal examination of the neonate was significant for low set ears, thick helices, flat nasal bridge, ankyloglossia, and aberrant head shape and size concerning for craniosynostosis. Postnatal MRI was consistent with Dandy-Walker variant showing hypogenesis of the inferior cerebellar vermis. To our knowledge, there are no prenatal or postnatal reports of comparable duplications involving these two regions simultaneously. Continued observation of the neonate may reveal further phenotypic consequences of these two simultaneous de novo interstitial duplications.
Prolapsed Uterine Fibroid: Value of MRI in Case of Massive Bleeding
Authors: Houda Azzouzi, Vasiliki Perlepe, Latifa Fellah
Journal: J Belg Soc Radiol (2023)
Keywords: broccoli sign, mri, prolapsed uterine mass
MeSH terms: Case Reports

Teaching Point: In case of acute bleeding caused by a mass located in the vagina, it may be difficult to assess the origin of the mass and determine whether it is benign or malignant; MRI is a useful tool for mass detection, diagnosis, and treatment decision.
Neurogenic Pulmonary Edema Following a Seizure: A Case Report and Literature Review
Authors: Mostafa Suhail Najim, Riyadh Ali Mohammed Hammamy, Sreethish Sasi
Journal: Case Rep Neurol Med (2019)
MeSH terms: Case Reports

Acute pulmonary edema is one of the frequent causes of dyspnea encountered in everyday practice. It is broadly attributed to be either cardiogenic or noncardiogenic. It is usually treated with diuretics in addition to other medications depending on the underlying pathology. Here, we report a case of a female patient who presented with shortness of breath after developing a seizure. Further investigations excluded cardiogenic etiology and showed critically low phenytoin level. It improved within 48 h of supportive care without giving diuretics favoring the diagnosis of neurogenic pulmonary edema as the primary pathology. The goal of our case report is to keep neurogenic pulmonary edema in mind, and hence provide the appropriate management, when dealing with similar cases.
Chronic idiopathic systemic capillary leak syndrome: a case report
Authors: Ahmed M Alkhunaizi, Abdullah H Kabbani, Mohamed A ElTigani
Journal: Allergy Asthma Clin Immunol (2019)
Keywords: capillary leak syndrome, clarkson disease
MeSH terms: Case Reports

Background:
Idiopathic systemic capillary leak syndrome (ISCLS), is a rare disorder characterized by recurrent attacks of varying severity of hypovolemic shock and generalized edema in association with hemoconcentration and hypoalbuminemia in the absence of albuminuria. The chronic form of ISCLS is extremely rare with only a few cases reported in the literature.
Case presentation:
Here we report the case of a young woman who developed chronic ISCLS characterized by massive, generalized and persistent edema. The work up confirmed the presence of monoclonal gammopathy. She was treated with several agents with no response.
Conclusion:
Chronic ISCLS is a very rare disease of unknown etiology and results in devastating complications. The medical community should be aware of this disease with the hope that targeted therapy will become available in the future.
Case report and literature review: double jeopardy - Exophiala dermatitidis and Mycobacterium canariasense central line-associated bloodstream infection in a patient
Authors: Afrinash Ahamad, Bushra Tehreem, Maaz Farooqi, Bennadette Maramara
Journal: Access Microbiol (2022)
Keywords: exophiala dermatitidis, maldi-tof- ms, mycobacterium canariasense, blood culture, catheter-associated infection (clabsi), irritable bowel syndrome
MeSH terms: Case Reports

Central line-associated bloodstream infection (CLABSI) is the most common nosocomial-acquired infection, affecting 38 000 patients in the USA annually. Approximately 8-10 % of inserted catheters lead to bloodstream infections, and ~25-30 % of infections are associated with mortality. Although proper line maintenance is essential to prevent infection, it is quite a challenge to avoid infection in patients with a long-term catheter. We present a case of a female in her 40s with a previous history of irritable bowel syndrome (IBS) who has had a central line for total parenteral nutrition for the past 2 years. The patient recently visited the emergency room with fever and generalized fatigue. Blood cultures sent to microbiology were positive for black mould, Exophiala dermatitidis. However, after a few days, microbiology reported an additional micro-organism, Mycobacterium canariasense , a pathogen rarely associated with bacteraemia. The patient was administered voriconazole and moxifloxacin for black mould and mycobacterium infection, respectively. We present an unusual case of rare opportunistic organisms causing bacteraemia and fungaemia in a patient with a long-term catheter. CLABSI remains a serious challenge for clinical facilities. Implementation and monitoring of effective strategies can prevent catheter-related bloodstream infections in patients with long-term catheters and can reduce the morbidity and mortality associated with CLABSI.
Gadolinium-Induced Acute Graft Pancreatitis in a Simultaneous Pancreas-Kidney Transplant Recipient
Authors: Chiang Sheng Lee, Rachel Yi Ping Tan, Nitesh N Rao
Journal: Case Rep Nephrol (2022)
MeSH terms: Case Reports

Gadolinium-induced acute pancreatitis is a rare phenomenon associated with the administration of gadolinium-based contrast agents. Only five cases of gadolinium-induced acute pancreatitis have been reported worldwide in patients with native pancreas and none with a pancreatic graft. We present a 32-year-old woman with prior history of simultaneous pancreas-kidney transplant who presented with generalized abdominal pain associated with systemic inflammatory response syndrome requiring admission to the intensive care unit. This occurred within 48 hours after having a magnetic resonance imaging (MRI) with gadolinium for investigation of subacute left optic atrophy. She was noted to have a marked rise in serum lipase, and the computed tomography findings were consistent with acute graft pancreatitis. Other causes of pancreatitis were ruled out, and she was managed conservatively with aggressive hydration, bowel rest, and analgesia with good recovery. This is the first reported case of gadolinium-induced acute graft pancreatitis occurring in a simultaneous pancreas-kidney transplant recipient. Clinicians should consider this rare differential diagnosis as a cause of graft pancreatitis in patients who have received gadolinium-based contrast agents.