OpenMed/multicare-articles huggingface dataset:
Double direct hernia, triple indirect hernia, double Pantaloon hernia (Jammu, Kashmir and Ladakh Hernia) with anomalous inferior epigastric artey: Case report
Authors: Imtiaz Wani
Journal: Int J Surg Case Rep (2019)
Keywords: direct, epigastric, hernia, indirect, inferior, pantaloon, romberg’s, saddle

Introduction:
Double direct and triple indirect type hernia in unilateral inguinal hernia is unique. Anomaly of pathway traversed by inferior epigastric artery is always of surgical importance. Meticulous exploration of the groin is mandatory for diagnosis of rare hernia.
Case report:
A 46 year old male presented with the right groin swelling. An inguinal hernia having two direct types separated by anamalous inferior epigastric artery and three indirect types of hernia were present. There was one bubonocele and two funicular types of indirect type of hernia present on the single cord. Anamalous inferior epigastric artery lying superficially at midpoint of inguinal canal was buttresed in the posterior wall to prevent its entrapping in mesh. Individual ligation of the indirect sacs and Lichtenstein repair was done. This is first kind of unique case report with the new type of hernia reported in the English literature after searching Pubmed, Medline, Google Scholar and Embase database. This new hernia, Double Pantaloon hernia, as a mark of respect and unity, is named after its State of origin, "Jammu, Kashmir and Ladakh" so called as "Jammu Kashmir and Ladakh Hernia".
Conclusion:
Double direct and triple indirect type hernia in a unilateral inguinal hernia is unique. Presence of multiple hernia sacs in the inguinal hernia is a risk for recurrence, if not detected. Inferior epigastric artery in inguinal canal may traverse an aberrant course.
Effects of the Chinese herbal medicine Hong Huang decoction, on myocardial injury in breast cancer patients who underwent anthracycline-based chemotherapy
Authors: Sihan Cao, Jingxian Xue, Lu Chen, Yun Hao, Meijuan Lu, Ming Feng, Huanhuan Wang, Jun Zhou, Chang Yao
Journal: Front Cardiovasc Med (2022)
Keywords: gls, hong huang decoction, anthracycline, breast cancer, chemotherapy
MeSH terms: Case Reports

Objective:
To assess the effects of Hong Huang Decoction (HHD), a Chinese herbal medicine, on myocardial injury in breast cancer patients who underwent anthracycline (ANT)-based chemotherapy.
Methods:
A total of 51 patients with breast cancer who underwent an ANT-based chemotherapy program and met the inclusion/exclusion criteria were allocated to the treatment or placebo groups using a random number generation process. Patients in the treatment group received liquid HHD twice a day. Treatment was given from 1 day prior to chemotherapy up to the end of chemotherapy (after 6 months). Participants in the placebo group received a placebo over the same schedule. Left ventricular ejection fraction (LVEF), global longitudinal strain (GLS), diagnostic markers of acute myocardial infarction [e.g., lactate dehydrogenase (LDH), creatine kinase-MB (CK-MB), and B-type natriuretic peptide (BNP)], nitric oxide (NO), superoxide dismutase (SOD), as well as pro-inflammatory cytokines [e.g., tumor necrosis factor-α (TNF-α), interleukin-6 (IL-6), and human C-reactive protein (CRP)], and anti-inflammatory cytokine interleukin-10 (IL-10), were outcome measures assessed before chemotherapy, 3 and 6 months after chemotherapy.
Results:
Compared to the placebo group, the GLS value was significantly higher in the treatment group (19.95 ± 1.16 vs. 19.06 ± 1.64, P ≤ 0.001). Significant differences were also noted for levels of SOD (689.71 ± 203.60 vs. 807.88 ± 182.10, P < 0.05), IL-6 (58.04 ± 22.06 vs. 194.20 ± 40.14, P ≤ 0.001), IL-10 (237.90 ± 94.98 vs. 68.81 ± 32.92, P ≤ 0.001), NO (75.05 ± 26.39 vs. 55.83 ± 19.37, P ≤ 0.005), and TNF-α (301.80 ± 134.20 vs. 680.30 ± 199.60, P ≤ 0.001) in the patients before chemotherapy compared to 6 months after initiating chemotherapy.
Conclusion:
HHD regulated the levels of IL-6, IL-10, SOD, NO, and TNF-α. The results demonstrated that GLS is a better indicator of early myocardial injury compared to LVEF, and HHD could modulate oxidative stress to protect against ANT cardio toxicity.
Clinical trial registration:
Chinese Clinical Trial Registry, identifier ChiCTR1900022394. Date of registration: 2019-04-09.
A meaningful exploration of ofatumumab in refractory NMOSD: a case report
Authors: Yibo Zhan, Min Zhao, Xiaojun Li, Huiying Ouyang, Chenghao Du, Guixian Chen, Zhenzhen Lou, Haoxuan Chen, Yuanqi Zhao, Haoyou Xu
Journal: Front Immunol (2023)
Keywords: b cell depletion therapy, neuromyelitis optica spectrum disorder, ofatumumab, refractory, relapse
MeSH terms: Neuromyelitis Optica / diagnosis, Neuromyelitis Optica / drug therapy, Case Reports, Adult, Azathioprine / adverse effects, Female, Humans, Immunosuppressive Agents / therapeutic use, Treatment Outcome, ofatumumab, Immunosuppressive Agents, Azathioprine

Objective:
To report the case of a patient with refractory neuromyelitis optica spectrum disorder (NMOSD), who, despite showing poor response or intolerance to multiple immunosuppressants, was successfully treated with Ofatumumab.
Case presentation:
A 42-year-old female was diagnosed with NMOSD in the first episode of the disease. Despite treatment with intravenous methylprednisolone, immunoglobulin, rituximab and immunoadsorption, together with oral steroids, azathioprine, mycophenolate mofetil and tacrolimus, she underwent various adverse events, such as abnormal liver function, repeated infections, fever, rashes, hemorrhagic shock, etc., and experienced five relapses over the ensuing four years. Finally, clinicians decided to initiate Ofatumumab to control the disease. The patient received 9 doses of Ofatumumab over the next 10 months at customized intervals. Her symptoms were stable and there was no recurrence or any adverse events.
Conclusion:
Ofatumumab might serve as an effective and safe alternative for NMOSD patients who are resistant to other current immunotherapies.
Acquired A amyloidosis from injection drug use presenting with atraumatic splenic rupture in a hospitalized patient: a case report
Authors: Garrett R Roll, Andrew Y Lee, Kayvan Royaie, Brendan Visser, Douglas K Hanks, Margaret M Knudson, Frederick J Roll
Journal: J Med Case Rep (2011)

Introduction:
Little is known about splenic rupture in patients who develop systemic acquired A amyloidosis. This is the first report of a case of atraumatic splenic rupture in a patient with acquired A amyloidosis from chronic injection drug use.
Case presentation:
A 58-year-old Caucasian man with a long history of injection drug use, hospitalized for infective endocarditis, experienced atraumatic splenic rupture and underwent splenectomy. Histopathological and microbiological analyses of the splenic tissue were consistent with systemic acquired A amyloidosis, most likely from injection drug use, that led to splenic rupture without any recognized trauma or evidence of bacterial embolization to the spleen.
Conclusion:
In patients with chronic inflammatory conditions, including the use of injection drugs, who experience acute onset of left upper quadrant pain, the diagnosis of atraumatic splenic rupture must be considered.
Esophageal low-grade intraepithelial neoplasia overlying multiple leiomyomas: A case report and review of the literature
Authors: Wen Pan, Junchao Wu, Chao Liu, Yanjun He, Jinlin Yang
Journal: Front Oncol (2022)
Keywords: case report, endoscape, esophageal low-grade intraepithelial neoplasia, multiple leiomyomas, overlying
MeSH terms: Case Reports

Background:
Esophageal leiomyoma is the most common benign submucosal mesenchymal tumor. Esophageal intraepithelial neoplasia includes low-grade and high-grade intraepithelial neoplasia. The coexistence of epithelial lesions and the subepithelial lesion is rare. We recorded a case of esophageal low-grade intraepithelial neoplasia (LGIN) overlying multiple esophageal leiomyomas and followed with a review of the literature.
Case presentation:
A 49-year-old female patient came for the treatment of esophageal lesions. The submucosal eminences were observed in the right posterior wall and the left anterior wall of the esophagus by Esophagogastroduodenoscopy (EGD). Additionally, we noticed the mucosa of the right wall with brown background color and the dilated, tortuous vessels by narrow-band imaging (NBI). Then we ensured that the submucosal lesions originated from the esophageal mucosal muscle by endoscopic ultrasonography (EUS) and enhanced CT. Subsequently, the submucosal eminence of the right posterior wall and the overlying mucosal lesion were removed together by endoscopic submucosal dissection (ESD). Postoperative pathological diagnosed esophageal submucosal leiomyoma with focal LGIN. Review EGD showed white scars on the right wall of the upper esophagus three months later, while pathological biopsy showed slight squamous epithelial hyperplasia in the left wall. We decided that the left submucosal lesion can be resected at a selective-time operation, and we continue to follow up as planned.
Conclusions:
The case of intraepithelial neoplasia overlying the submucosal tumor is rare. Either missed diagnosis or overdiagnosis should be avoided through EGD and pathological biopsy.
Multiple subacute bilateral cerebral infarcts in a 9-year-old Nigerian male: a case of childhood primary angiitis of the central nervous system
Authors: Christian Chukwukere Ogoke, Ifeanyi Innocent Ike, Augustine Emeka Osuji, Kingsley Asinobi, Chukwudi Ernest Okeke
Journal: Pan Afr Med J (2018)
Keywords: primary cns vasculitis, aphasia, cerebral infarcts, childhood pacns, progressive limb weakness
MeSH terms: Cerebral Infarction / etiology, Immunosuppressive Agents / therapeutic use, Vasculitis, Central Nervous System / diagnosis, Case Reports, Cerebral Infarction / diagnostic imaging, Cerebral Infarction / drug therapy, Child, Humans, Male, Neuroimaging / methods, Nigeria, Treatment Outcome, Vasculitis, Central Nervous System / complications, Vasculitis, Central Nervous System / drug therapy, Immunosuppressive Agents, Primary angiitis of the central nervous system

A Primary Angiitis of the Central Nervous System (PACNS) is ill-defined, complex and rare in children. Clinical presentation is variable, diagnosis is challenging and it is life-threatening but treatable. The index case is a 9-year old male who presented with progressively slurred speech, progressive weakness of the limbs, hemifacial weakness and vomiting. There were no clinical or laboratory features of systemic vasculitis. Neuroimaging showed multiple subacute infarcts in both cerebral hemispheres. He responded well to immunosuppressive therapy. The case report aims to underscore the need for high index of suspicion, early neuroimaging for progressive nonspecific decline in neurologic function and the treatable nature of the condition if diagnosis is made early.
Pyelolithotomy in a Patient with Complete Coraliform Calcification Induced by a Double-J Ureteral Stent
Authors: Miguel Bonfitto, Analaura de Oliveira Cury, Victoria Caroline Pagelkopf, Vinicius Ramos Bezerra de Morais, Carlos Abib Cury
Journal: Case Rep Urol (2019)
MeSH terms: Case Reports

The placement of a double-J ureteral stent enables the treatment of upper urinary tract obstruction. Despite advances, forgetting the stent favors the occurrence of calcification, leading to increased morbidity rates, lawsuits, and a financial burden on the healthcare system. This paper describes a successful pyelolithotomy for the removal of a calcified double-J ureteral stent.
Brain metastasis as an initial manifestation of ovarian carcinoma: a case report
Authors: Zafer Selçuk Tuncer, Gokhan Boyraz, Aslıhan Yazıcıoğlu, Ilker Selcuk, Senem Özge Yücel
Journal: Case Rep Med (2012)
MeSH terms: Retracted Publication

Brain metastasis is a rare and late manifestation of ovarian carcinoma. A 30-year-old woman initially presenting with neurologic symptoms was later found to have mucinous ovarian carcinoma. The patient had a 6 cm adnexal mass with multiple millimetric brain metastatic lesions. Following a surgical staging laparotomy procedure, the patient received brain irradiation and systemic chemotherapy.
Huntington's Disease: Two-Year Observational Follow-Up of Executive Function Evaluation with CNS Vital Signs Test in an Adult Patient
Authors: Anna Lucia Spear King, Alexandre Martins Valença, Adriana Cardoso de Oliveira E Silva, Ana Claudia Cerqueira, Lígia Maria Chaves Ferraz, Antonio Egidio Nardi
Journal: Case Rep Med (2011)
MeSH terms: Case Reports

Huntington's disease (HD) is a genetic, degenerative, and progressive central nervous system disease. It is characterized by motor abnormalities and cognitive and psychiatric symptoms. Objective. To describe the precise degree of clinical severity of patients with HD through a new neurocognitive assessment. Methods. Unprecedented battery of computerized tests, CNSVS (Central Nervous System Vital Signs), was applied at three different moments in 2008, 2009, and 2010. The accurate and reliable CNSVS objectively provided the cognitive state of patients and allowed for the evaluation of disease progression. Case Report. P., 26, female, without any medication, with normal psychomotor development is a parent carrier of HD. In 2008, she was diagnosed with HD in accordance with the Medical Genetics Laboratories. Conclusion. The tests may be useful to reveal the exact measure of the current evolutionary stage of HD patients, allowing for more efficient planning of treatment and future procedures, such as the medication, therapy, and physical activity to be administered.
A Systemic Capillary Leak Syndrome (Clarkson Syndrome) in a Patient with Chronic Lymphocytic Leukemia: A Case Report in an Out-of-Hospital Setting
Authors: Manon Durand Bechu, Antoine Rouget, Christian Recher, Elie Azoulay, Vincent Bounes
Journal: Case Rep Emerg Med (2016)

Systemic Capillary Leak Syndrome (SCLS) is a rare disease with poor prognosis, characterized by the occurrence of mucocutaneous and visceral edema with hypotension, hemoconcentration, and unexpected hypoalbuminemia. The disease can be idiopathic (Clarkson syndrome) or secondary to other diseases and treatments. We describe this syndrome in a prehospitalized, 63-year-old patient with chronic lymphocytic leukemia and an idiopathic form of SCLS manifesting as hypovolemic shock. Initial care is hospitalization in intensive care. In addition to etiological treatment if fluid replacement is necessary, treatment must be closely monitored for secondary overload complications. Catecholamine rather than arrhythmogenic support may be associated.
Deep brain stimulation of nucleus accumbens region in alcoholism affects reward processing
Authors: Marcus Heldmann, Georg Berding, Jürgen Voges, Bernhard Bogerts, Imke Galazky, Ulf Müller, Gunther Baillot, Hans-Jochen Heinze, Thomas F Münte
Journal: PLoS One (2012)
MeSH terms: Alcoholism / therapy, Deep Brain Stimulation, Nucleus Accumbens / physiopathology, Reward, Case Reports, Research Support, Non-U.S. Gov't, Adult, Alcoholism / diagnostic imaging, Alcoholism / physiopathology, Gambling, Humans, Male, Neuropsychological Tests, Nucleus Accumbens / diagnostic imaging, Radionuclide Imaging, Risk-Taking

The influence of bilateral deep brain stimulation (DBS) of the nucleus nucleus (NAcc) on the processing of reward in a gambling paradigm was investigated using H(2)[(15)O]-PET (positron emission tomography) in a 38-year-old man treated for severe alcohol addiction. Behavioral data analysis revealed a less risky, more careful choice behavior under active DBS compared to DBS switched off. PET showed win- and loss-related activations in the paracingulate cortex, temporal poles, precuneus and hippocampus under active DBS, brain areas that have been implicated in action monitoring and behavioral control. Except for the temporal pole these activations were not seen when DBS was deactivated. These findings suggest that DBS of the NAcc may act partially by improving behavioral control.
Impaired STAT3-Dependent Upregulation of IL2Rα in B Cells of a Patient With a STAT1 Gain-of-Function Mutation
Authors: Menno C van Zelm, Julian J Bosco, Pei M Aui, Samuel De Jong, Fiona Hore-Lacy, Robyn E O'Hehir, Robert G Stirling, Paul U Cameron
Journal: Front Immunol (2019)
Keywords: il2rα, stat1, stat3, chronic mucocutaneous candidiasis, gain-of-function, hypogammaglobulinemia
MeSH terms: B-Lymphocytes / metabolism, Gain of Function Mutation, Interleukin-2 Receptor alpha Subunit / metabolism, STAT1 Transcription Factor / genetics, STAT1 Transcription Factor / metabolism, STAT3 Transcription Factor / metabolism, Case Reports, Research Support, Non-U.S. Gov't, Gene Rearrangement, Genotype, Humans, Immunoglobulins / genetics, Immunophenotyping, Lymphocyte Activation / genetics, Lymphocyte Activation / immunology, Male, Phosphorylation, Signal Transduction, Immunoglobulins, Interleukin-2 Receptor alpha Subunit, STAT1 Transcription Factor, STAT1 protein, human, STAT3 Transcription Factor, STAT3 protein, human

Heterozygous STAT1 gain-of-function (GOF) mutations form the most common genetic cause of chronic mucocutaneous candidiasis (CMC). In such patients, increased STAT1 function leads to impaired STAT3-dependent activation of IL-17A and IL-17F in T cells, thereby causing impaired Th17 responses to Candida. In spite of the critical role of STAT3 in IL-21 signaling in B cells, nearly all STAT1 GOF patients have normal or high serum IgG. We here present a 44 year-old male with childhood onset of CMC and antibody deficiency since early adulthood. Sequence analysis of STAT1 revealed a heterozygous missense mutation in the coiled-coil domain (p.D168E), which resulted in increased STAT1 phosphorylation of B-cells activated with IFNα and IFNγ. IL-21 induced STAT3 phosphorylation and nuclear localization were normal, but resulted in impaired upregulation of IL2Rα. This newly identified B-cell intrinsic impairment of STAT3 function could underlie the progressive development of hypogammaglobulinemia. Considering the high risk of bronchiectasis and irreversible organ damage, this case illustrates the need for monitoring of IgG levels and/or function in adult patients with STAT1 GOF mutations.
Page kidney: A rare cause of secondary hypertension
Authors: Ilonka Warnich, Mark Nicolaou, Zelia Sofianos, Jacobus A Pienaar, Jacob Varghese
Journal: SA J Radiol (2019)
Keywords: page kidney, hypertension, secondary hypertension, subcapsular collection, young
MeSH terms: Case Reports

Page kidney is a rare phenomenon that can present with hypertension. The presence of a subcapsular perirenal collection causes parenchymal compression leading to renal hypoperfusion. Subsequent activation of the renin-angiotensin-aldosterone system results in an increase in systemic blood pressure. The causes of renal subcapsular collections are varied, with most cases being secondary to post-traumatic haematomas. We present the case of a young hypertensive patient, treated as primary hypertension with persistently uncontrolled blood pressures. This was despite good treatment adherence. On further investigation, imaging identified the presence of bilateral subcapsular collections. This case illustrates the importance of a thorough workup in a young hypertensive patient with refractory hypertension. Given that Page kidney is curable, timeous intervention can save the patient from unnecessary medications and the morbidity of uncontrolled blood pressures.
Case report: Genetic defects in laminin α5 cause infantile steroid-resistant nephrotic syndrome
Authors: Yoon Sunwoo, Naye Choi, Jeesu Min, Jihyun Kim, Yo Han Ahn, Hee Gyung Kang
Journal: Front Pediatr (2023)
Keywords: lama5, srns, case report, genetic nephrotic syndrome, laminin
MeSH terms: Case Reports

Single gene pathogenic mutations have been implicated in up to 30% of pediatric steroid-resistant nephrotic syndrome (SRNS) cases, mostly in infantile patients. Among them is LAMA5, which has been recently discovered and encodes the laminin α5 chain. The laminin α5β2γ1 heterotrimer is an essential component of the glomerular basement membrane and is necessary for embryogenesis and immune modulation. Biallelic LAMA5 variants have been identified in one adult and ten pediatric nephrotic syndromes (NS) patients with variable phenotypes. Biallelic truncating mutations in this gene have recently been proven to cause SRNS. Here, we present another case of infantile SRNS related to novel compound heterozygous variations of LAMA5 (c.3434G > A, p.Cys1145Tyr and c.6883C > T, p.Gln2295*), the first reported case with one missense and one nonsense allele. A 10-month-old female patient presented with eyelid edema and massive proteinuria without any extrarenal symptoms or family history. The patient was diagnosed with SRNS. Renal biopsy revealed focal segmental glomerulosclerosis with widely effaced epithelial foot processes and a "moth-eaten" appearance. She progressed to end stage kidney disease (ESKD), requiring dialysis at 31 months of age, and underwent a deceased-donor kidney transplant at 6 years of age. Four months after transplantation, she developed Ebstein-Barr Virus (EBV) infection related to post-transplantation lymphoproliferative disorder (PTLD). After chemotherapy, the patient remained healthy with adequate renal function without disease recurrence for the past 7 years. We also identified previous cases of biallelic LAMA5 variants associated with the nephrotic phenotype and analyzed the available clinical and genetic information. All reported patients had an onset of NS ranging from 3 months to 8 years, with no other syndromic features. Response to therapy and renal outcomes varied greatly; most patients exhibited steroid resistance, five progressed to ESKD, and two received kidney transplantation (KT). There was one report of PTLD. Our patient's phenotype was markedly more severe than those with biallelic missense variants and somewhat less severe than those with two truncating variants. LAMA5 defects may also play a role in PTLD, though no conclusions can be made with such limited cases. LAMA5 should be considered a candidate gene for SRNS and should be actively tested in cases with no other genetic diagnosis.