OpenMed/multicare-articles huggingface dataset:
Follicular lymphoma in situ presenting as dermatopathic lymphadenopathy
Authors: Orah Nnamdi Obumneme, Igbokwe Uche, Banjo Adekunbiola, Akinde Ralph, Irurhe Nicholas
Journal: Case Rep Pathol (2013)

Introduction. Follicular lymphoma in situ (FLIS) is a recently described entity with few cases recognized worldwide. To our knowledge, this is the first FLIS reported from West Africa. Case Presentation. We present the case of a 48-year-old civil servant with axillary lymphadenopathy discovered on routine mammography. On histology, a predominant reactive change with aggregates of melanophages was seen prompting a diagnosis of reactive lymphadenopathy. Immunohistochemistry done in our laboratory showed CD10 and Bcl-6 positive germinal centres with a small population of Bcl-2 positive germinal centre centre cells that were limited to some of the germinal centres. Conclusion. This highlights the use of immunohistochemistry in lymph node pathology-a resource which is very limited in our environment.
Multiple FNH-Like Lesions in a Patient with Chronic Budd-Chiari Syndrome: Gd-EOB-Enhanced MRI and BR1 CEUS Findings
Authors: Caroline Newerla, Fabienne Schaeffer, Luigi Terracciano, Joachim Hohmann
Journal: Case Rep Radiol (2012)
MeSH terms: Case Reports

A-26-year old female patient with chronic Budd-Chiari syndrome due to different underlying blood disorders applied for a two-year followup of the liver with Gadolinium-ethoxybenzyl-diethylenetriaminepentaacetic-acid-(Gd-EOB-DTPA-) enhanced MRI. The liver function tests were raised. Besides showing a progressive hepatosplenomegaly and a cirrhotic liver alteration, the MRI revealed multiple new nodular lesions in all liver segments. These lesions showed typical patterns in the precontrast images, while there was an arterial and a persistent portal venous enhancement. In the hepatobiliary liver-specific late phase, a central "washout" and a persistent rim enhancement were observed (target sign). The additionally performed contrast-enhanced ultrasonography showed a strong zentrifugal arterial enhancement of the lesions followed by an isoechoic enhancement in the portal venous and delayed liver phase. Histologically these lesions turned out as focal nodular hyperplasias (FNH) or FNH-like lesions, also known as large regenerative nodules (LRNs). Differentiation between regenerative nodules like LRN and hepatocellular carcinoma (HCC) in cirrhotic livers is crucial, and the target sign in the hepatobiliary phase of Gd-EOB-DTPA as well as the centrifugal arterial enhancement followed by an isoenhancement during a CEUS might be useful for establishing the correct diagnosis of such hypervascular lesions with proliferated and likely aberrant bile ducts.
Primary Retroperitoneal Malignant Melanoma with Involvement of Iliac Artery and Vein
Authors: Aislyn Oulee, Ling Xu, Scott Worswick
Journal: Case Rep Med (2021)
MeSH terms: Case Reports

Primary retroperitoneal melanoma is a very rare extracutaneous presentation of melanoma. In this case report, we present a 65-year-old female with unilateral lower extremity edema secondary to occlusion of iliac vessels by a primary retroperitoneal melanoma tumor. We also review the findings in other cases previously described in the literature.
Basal encephalocele in an adult patient presenting with minor anomalies: a case report
Authors: Naoyuki Harada, Masaaki Nemoto, Chikao Miyazaki, Kosuke Kondo, Hiroyuki Masuda, Jun Nomoto, Nobuo Sugo, Takao Kuroki
Journal: J Med Case Rep (2014)

Introduction:
Basal encephalocele is rare in adults. Congenital and acquired cases have been reported with regard to the developmental mechanism, and the pathology has not been elucidated in detail.
Case presentation:
We encountered an adult with basal encephalocele strongly suggesting congenital development because of the presence of minor anomalies: strabismus and ocular hypertelorism. The disease manifested as persistent spontaneous cerebrospinal fluid rhinorrhea and repeated meningitis in a 66-year-old Japanese man. On computed tomography, brain tissue protruded through a part of the ethmoid bone of his right anterior skull base, and it was diagnosed as transethmoidal-type basal encephalocele. Regarding his facial form, the distance between his bilateral eyeballs was large compared to his facial width, and his canthal index (defined as inner to outer inter canthal ratio × 100) was calculated as 38.5, based on which it was judged as ocular hypertelorism. In addition, his right eyeball showed strabismus. A right frontotemporal craniotomy was performed for spontaneous cerebrospinal fluid rhinorrhea, and the defective dura mater region was patched with temporal fascia.
Conclusions:
Mild minor anomalies that require no treatment are overlooked in adults, but the presence of several anomalies increases the possibility of congenital disease. Therefore, it may be necessary to examine minor anomalies in cases of adult basal encephalocele when considering the possibility that the disease may be congenital.
Malignant Transformation of Endometriosis in the Ischioanal Fossa
Authors: Jordan S Klebanoff, Shivani K Shah, Mark G Cadungog
Journal: Case Rep Obstet Gynecol (2018)
MeSH terms: Case Reports

We present the case of a 28-year-old nulliparous female with malignant transformation of an ectopic focus of endometriosis in the right ischioanal fossa. A 28-year-old nulliparous patient with a past medical history of polycystic ovarian syndrome (PCOS) was diagnosed with endometrioid adenocarcinoma in her right ischioanal fossa. Initially, patient presented to an emergency department and underwent a CT scan of the appendix to rule out appendicitis. A multiloculated cystic lesion adjacent to the right obturator internus muscle was found. She underwent surgical resection of the mass, which confirmed FIGO grade 2 endometrioid adenocarcinoma, followed by localized radiation therapy. Malignancy arising in endometriosis is rare, and the influence of PCOS on the rate of malignant transformation is not well established.
Healthcare resource use and costs of managing children and adults with lysosomal acid lipase deficiency at a tertiary referral centre in the United Kingdom
Authors: Julian F Guest, Andy Ingram, Nadia Ayoub, Christian J Hendriksz, Elaine Murphy, Yusof Rahman, Patrick McKiernan, Helen Mundy, Patrick Deegan
Journal: PLoS One (2018)
MeSH terms: Cost of Illness, Health Care Rationing, Referral and Consultation, Wolman Disease / therapy, Research Support, Non-U.S. Gov't, Adolescent, Adult, Child, Female, Humans, Retrospective Studies, State Medicine, United Kingdom, Wolman Disease / economics, Young Adult, Lysosomal acid lipase deficiency

Objective:
To estimate clinical progression and resource utilisation together with the associated costs of managing children and adults with LAL Deficiency, at a tertiary referral centre in the UK.
Methods:
A retrospective chart review was undertaken of patients in the UK with a confirmed diagnosis of LAL Deficiency who were managed at a LAL Deficiency tertiary referral treatment centre. Patients' pathways, treatment patterns, health outcomes and resource use were quantified over differing lengths of time for each patient enabling the NHS cost of patient management in tertiary care to be estimated.
Results:
The study population comprised 19 patients of whom 58% were male. Mean age at the time of initial presentation was 15.5 years and the mean age at diagnosis was 18.0 years. 63%, 53% and 42% of patients had hepatomegaly, abnormal lipid storage and splenomegaly at a mean age of presentation of 17.8, 17.1 and 20.9 years, respectively. Over a period of 50 years there were a mean of 48.5 clinician visits and 3.4 hospital admissions per patient. The mean NHS cost of patient management at a LAL Deficiency tertiary referral treatment centre, spanning a period of over 50 years was £61,454 per patient.
Conclusion:
This study provides important insights into a number of aspects of the disease that are difficult to ascertain from published case reports. Additionally, it provides the best estimate available of NHS resource use and costs with which to inform policy and budgetary decisions pertaining to managing this ultra-orphan disease.
Imageless Navigation Improves Intraoperative Monitoring of Leg Length Changes during Total Hip Arthroplasty for Legg-Calve-Perthes Disease: Two Case Reports
Authors: Ritesh R Shah, Varsha Gobin, Jeffrey M Muir
Journal: Case Rep Orthop (2018)
MeSH terms: Case Reports

Legg-Calve-Perthes disease is a rare condition characterized by avascular necrosis and malformation of the femoral head. For many patients, total hip arthroplasty (THA) is the only viable treatment option; however, there are challenges associated with THA in this population, primarily the equalization of leg lengths. Here, we present two cases of Legg-Calve-Perthes disease treated via total hip arthroplasty with the assistance of an imageless, computer-assisted navigation device. In each case, the device provided intraoperative data on leg length in real time, allowing for improved accuracy of component placement. Postoperative leg lengths were confirmed to be equalized in each case using radiographs. These cases are, to our knowledge, the first such cases using imageless navigation during THA and demonstrate the benefits of such assistive technologies in challenging cases such as Legg-Calve-Perthes disease.
Two Cases of Leigh Syndrome in One Family: Diagnostic Challenges and Clinical Management Experience in Latvia
Authors: Arta Katkevica, Madara Kreile, Ieva Grinfelde, Gita Taurina, Ieva Micule, Iveta Dzivite-Krisane, Arta Smite-Laguna, Ieva Malniece
Journal: Case Rep Med (2021)
MeSH terms: Case Reports

Leigh syndrome is a neurodegenerative disorder with an incidence of 1 : 40,000 live births. The clinical presentation of LS is highly variable with heterogeneity in the disease-associated symptoms of cerebellar, motor, and extrapyramidal dysfunction and common infections. There is no effective treatment for this condition; as such, the prognosis of this condition is very poor with death occurring within the first few years of life. In this study, we report the first LS case in Latvia with SURF1 pathogenic variants in two siblings. The difficulties encountered establishing a diagnosis for the first proband and the effective prenatal diagnosis for the second offspring that led to termination of the pregnancy are discussed.
Extensive Cutaneous Scalp Angiosarcoma
Authors: Zabeer Bhatti, Rameez Bhatti, Sharon Brangman, Kerry Whiting, Amit Dhamoon
Journal: Case Rep Dermatol Med (2018)
MeSH terms: Case Reports

Angiosarcoma is a cancer that is derived from endothelial cells that line blood vessels and lymphatic channels. Cutaneous angiosarcoma can appear anywhere on the skin and the clinical presentation is highly variable. Most cases appear on the scalp and face de novo. Our case describes a 91-year-old female with cutaneous scalp angiosarcoma. Our case serves to remind physicians that an abnormal skin finding in older adults should raise their index of suspicion for angiosarcoma and an early biopsy should be performed.
Severe skin disease in lupus associated with hemophagocytic lymphohistiocytosis: case reports and review of the literature
Authors: Christina S Thornton, Parham Minoo, Michelle Schneider, Aurore Fifi-Mah
Journal: BMC Rheumatol (2019)
MeSH terms: Case Reports

Background:
Hemophagocytic lymphohistiocytosis (HLH) is a severe clinical entity associated with high mortality in the adult population. HLH has been associated with infections, malignancy and autoimmune conditions such as Systemic Lupus Erythematosus (SLE), however this is often in the context of a disease flare. Currently, there are limited reports of inaugural SLE manifesting as HLH with a lack of consensus on treatment and management of these patients.
Case presentation:
Here, we present two rare case reports of severe cutaneous manifestation of lupus associated with HLH. Both patients presented with sinister clinical courses with primarily rheumatologic complaints including malaise, arthralgia, and myalgia with biochemical abnormalities. Both patients were diagnosed with HLH as a result of first presentation from cutaneous lupus. A comprehensive literature review using the PubMed database with cases comprising keywords of HLH and SLE up to September 2017 was conducted, with an emphasis on inaugural cutaneous SLE cases.
Conclusions:
Ultimately, we highlight that a keen clinical acumen is required as misdiagnosis may lead to insufficient treatment with adverse clinical outcomes with the unique presentation of HLH from inaugural cases of SLE.
Laparoscopic retrieval of sharp foreign body: An innovative approach
Authors: P O Igwe, N E Brownson, S L Harcourt, N Ejindu
Journal: Int J Surg Case Rep (2020)
Keywords: innovative approach, laparoscopy, sharp foreign body
MeSH terms: Case Reports

Background:
Laparoscopy is a relatively new surgical approach in developing countries. Migrated foreign bodies into the body cavities could be very distressing to patients, relations and caregivers. There are paucity of migrated Steinmann pin and laparoscopic retrieval methods in literature.
Aim:
An innovative approach of laparoscopic retrieval of migrated sharp foreign body (Steinmann pin).
Methods:
A two-port technique was used to retrieve the pin. These ports consisted of optical supra-umbilical port (10 mm) and left iliac fossa port of 10 mm. They were aligned in the direction of sharp end of the foreign body, to aid retrieval of the Steinmann pin. This procedure was performed on a 34-year-old man who presented with migration of Steinmann pin into abdominal cavity. He had open reduction of old unreduced left posterior hip dislocation. A Steinmann pin with a diameter of 4.5 mm and length of 140 mm was passed through the greater trochanter to the acetabular roof. The protruding end of the pin ought to have been bent. Three weeks post-surgery, the pin migrated. Physical examination revealed vague tenderness over supra pubic region. A hard pointed object was palpable in the right iliac fossa. Abdominal X-ray and ultrasound were suggestive but not definite for pin location.
Results:
A sharp Steinman pin was retrieved from the abdominal cavity using laparoscopy.
Conclusion:
Migration of Steinmann pin is unusual. Laparoscopy is an innovative approach to foreign body retrieval. This could help reduce the morbidity that may be accompanied by other modalities such as open approach.
Possible abscopal effect in urothelial carcinoma of the upper urinary tract after treatment with immune checkpoint inhibitors
Authors: Yudai Ishiyama, Toshio Takagi, Kazuhiko Yoshida, Junpei Iizuka, Yoichi Kakuta, Masayoshi Okumi, Hideki Ishida, Kazunari Tanabe
Journal: IJU Case Rep (2019)
Keywords: immunotherapy, lymph nodes, neoplasm metastasis, radiotherapy, urologic neoplasms
MeSH terms: Case Reports

Introduction:
Regression of non-irradiated metastatic lesions after radiation therapy is known as the abscopal effect. We report a case of urothelial carcinoma in which the abscopal effect was possibly observed after immune checkpoint inhibitor administration.
Case presentation:
A 68-year-old woman diagnosed with left renal pelvic cancer underwent total nephroureterectomy and regional lymph node dissection. Eight months later, imaging studies detected local recurrence and paraaortic lymph node metastasis. The tumor progressed despite cisplatin + gemcitabine, pembrolizumab, and gemcitabine + docetaxel therapy. Radiation therapy was administered to a painful back lesion, which resulted in dramatic symptom relief. Computed tomography 2 months after radiation therapy indicated reduced size of the irradiated lesion and some non-irradiated lymph nodes.
Conclusion:
Combined radiation therapy and immune checkpoint inhibitors can provide additional benefits for certain cancers, possibly due to negative immunomodulatory response blockade. Thus, this combined therapy may be a new metastatic urothelial carcinoma treatment strategy.
Renal arteriovenous fistula after renal biopsy: a case report and literature review
Authors: Sergio Quilici Belczak, Guilherme Delicato Pedroso, Luis Felipe Atihe, Ana Beatriz Furlan Vilela, Raquel Silas Melice, Cicero Benedito, Gustavo Garcia Marques
Journal: J Vasc Bras (2019)
Keywords: arteriovenous fistula, coil embolization, needle biopsy, post-biopsy hematuria, renal doppler, therapeutic embolization
MeSH terms: Case Reports

Acquired renal arteriovenous fistulas (AVF) are rare conditions in which an anomalous connection arises between the arterial and venous systems. Renal AVFs can be classified into three main groups: idiopathic, congenital, and acquired, the last of which are the most common. Incidence has been increasing, due to the growing number of renal biopsies. Although the renal biopsy procedure is relatively safe nowadays, one possible complication is formation of an AVF in the renal vascular territory. Treatment of renal AVF is widely discussed in the literature and a variety of treatment methods can be employed. We report a case of arteriovenous fistula after renal biopsy that was successfully treated with endovascular coil embolization.
Treatment of Aggressive NK-Cell Leukemia: A Case Report and Review of the Literature
Authors: Anders Kindberg Boysen, Paw Jensen, Preben Johansen, Karen Dybkær, Mette Nyegaard
Journal: Case Rep Hematol (2011)
MeSH terms: Case Reports

Aggressive NK-cell leukemia is a rare malignancy with neoplastic proliferation of natural killer cells. It often presents with constitutional symptoms, a rapid declining clinical course, and a poor prognosis with a median survival of a few months. The disease is usually resistant to cytotoxic agents, and no treatment has emerged as the standard of care for these patients. We report a case of an 18-year-old male who obtains complete remission following two lines of combination chemotherapy. We describe in details our regimens for induction chemotherapy and perform a review of existing literature concerning treatment of aggressive NK-cell leukemia.
Youth perceptions toward managing elderly care among low-income household families using the My-Elderly-Care-Skills Module
Authors: Nur Syuhada Mokhzan, Rosnah Sutan, Ruhizan Mohammad Yasin, Hamidah Yamat
Journal: Front Public Health (2023)
Keywords: aged care, elderly care, perception, training, youth
MeSH terms: COVID-19, Pandemics, Research Support, Non-U.S. Gov't, Adolescent, Adult, Aged, Caregivers, Humans, Qualitative Research, Young Adult

Background:
Caregivers of elderly people need the right education and empowering skills to manage their own health needs and the elderly people they care for.
Objective:
The study aimed to explore youth perceptions of the My-Elderly-Care-Skills Module intervention and its perceived feasibility.
Methods:
This study involved youth respondents (18-30 years old) from low-income households who are accountable to providing care for independent older people (60 years or above) living in the same house. A qualitative study using a case study design was used to assess youth perceptions based on the content of the My-Elderly-Care-Skills module, by focusing on its implementation usage and usefulness for the care of the elderly. A total of 30 youths voluntarily participated in the online training workshop during the COVID-19 pandemic movement restriction order period. There were multiple sources of data, such as video recorded on reflection of care given at home, text messages in a WhatsApp group, and in-depth interviews during small group online meetings. Data were recorded and transcribed verbatim for common themes before a theme analysis was conducted. Inductive content analysis was performed after the saturation point was met.
Results:
Thematic analysis derived two domains of feasibility: operational and technical feasibility. There were three themes under operational practicality (improving awareness, addressing the caregiving skills needs, and seeking resources for knowledge) and three themes for technical practicality (easily used and informative, skill in effective communication, and program fulfillment).
Conclusion:
It was verified that it is feasible for young caregivers of the elderly to participate in the My-Elderly-Care-Skills training intervention as it helps in improving knowledge and skills performance in managing and caring for the elderly.
Vitamin D Intoxication and Nephrocalcinosis in a Young Breastfed Infant
Authors: Abdullah Al-Kandari, Hussain Sadeq, Rita Alfattal, Maryam AlRashid, Mayra Alsaeid
Journal: Case Rep Endocrinol (2021)
MeSH terms: Case Reports

Numerous studies were performed assessing the benefits and side effects of vitamin D. Vitamin D helps in regulating the calcium and phosphate metabolism leading to a healthy mineral and bone development. Vitamin D intoxication is an uncommon event that leads to hypercalcemia which can be associated with both immediate and late morbidities that can cause severe renal complications. Here, we present a case of a 4-month-old girl with a history of decreased feed and activity due hypercalcemia and high vitamin D level, which led to nephrocalcinosis. The patient received IV fluids, IV diuretics, methylprednisolone, and bisphosphonate in order to normalize the calcium level in blood. With clear verbal and written instructions for the dosage and administration of vitamin D supplements, as well as clear warnings of the potential risks of overdose, vitamin D intoxication could be an easily avoidable condition.
Management of unilateral temporomandibular joint ankylosis & orthomorphic correction in a patient with Marfan syndrome: A rare case report
Authors: B M Rudagi, Jain Rishabh, Merchant Arif, Chourasia Namrata, Naikwade Shahbaaz, Bhavar Gaurav
Journal: Int J Surg Case Rep (2020)
Keywords: ankylosis, case report, marfan syndrome, obstructive sleep apnea, orthomorphic, temporomandibular joint
MeSH terms: Case Reports

Introduction:
Marfan syndrome (MFS) is a disorder of the connective tissue that is inherited in an autosomal dominant fashion and is caused by mutations in the gene coding for fibrillin-1 (FBN1). This condition commonly affects the skeletal system, pulmonary system, ocular system and the cardiovascular system. Surgical intervention is often needed to correct the various deformities affecting the patients to restore the function, improving the patient's aesthetic appearance and quality of life.
Presentation of case:
It's a rare case report of a young male patient suffering through unilateral temporomandibular joint ankylosis, obstructive sleep apnea and unaesthetic appearance while being a case of Marfan syndrome.
Discussion:
This paper highlights the various surgical procedures undertaken to correct the deformities affecting the individual and improving the overall health of the patient.
Conclusion:
Our patient's management shows the necessity of a multidisciplinary, multi factorial and multi-faceted approach with early visual identification and diagnosis.
Hypopharyngeal Dedifferentiated Liposarcoma in the MDM2 Era: A Case Report and Short Review
Authors: Peter Wanes, David A Nolte, Ghassan A Tranesh
Journal: Case Rep Pathol (2020)
MeSH terms: Case Reports

Pharyngeal liposarcomas are very rare; still more rare are dedifferentiated liposarcomas in the pharynx. An 82-year-old man presented with dysphagia, voice changes, weight loss, nasal regurgitation of liquids, and coughing spells. A 3.5 cm mass was identified in the hypopharynx. The mass was biopsied and diagnosed as a benign fibroepithelial polyp. Continued symptoms and airway obstruction prompted a pharyngectomy, and the mass was then diagnosed as dedifferentiated liposarcoma. Due to infrequency and subtle histological findings, liposarcomas of the head and neck can be misdiagnosed and recur.