OpenMed/multicare-articles huggingface dataset:
A case report of jejunum transplantation in the treatment of severe cervical esophageal stricture in patients with dystrophic epidermolysis bullosa
Authors: Zhen Xu, Yong Zhang, Yanjiao Hu, Xia Xiu, Bowen Yang, Tianqiao Huang, Yichuan Huang
Journal: Front Oncol (2023)
Keywords: dysphagia, epidermolysis bullosa, esophageal stenosis, jejunum transplantation, surgical treatment
MeSH terms: Case Reports

Epidermolysis bullosa (EB) is a rare disorder caused by autosomal genetic variation. Its main clinical features include skin and mucous membrane blisters, erosion, repeated ulcers and scar formation. The lesions mostly involve the skin, oral cavity, digestive system and urinary system. Epidermolysis bullosa complicated with esophageal stenosis is a common gastrointestinal manifestation of this disorder. Currently, there is no cure for EB, and thus symptomatic treatment is usually applied. Here we describe the case of a patient with recessive dystrophic EB complicated with severe esophageal stenosis. The narrow segment of esophagus was removed and the free part of jejunum was transplanted into the esophageal defect to reconstruct the esophagus and restore the patient's normal swallowing. For patients with EB complicated with severe esophageal stenosis, surgical resection of the diseased esophagus and jejunal transplantation can be used to repair the esophageal and restore normal swallowing pathway, providing an effective treatment for this condition.
Venetoclax combined with azacitidine in blastic plasmacytoid dendritic cell neoplasm: a case report and comprehensive review on the current and future treatment
Authors: Xiaoning Wang, Jiashuo Guo, Yan Liu, Na Zheng, Shaohan Xu, Lianhui Wu, Ruirui Yuan, Liying Xue, Jie Li
Journal: Front Med (Lausanne) (2024)
Keywords: bcl2 inhibitor, azacitidine, blastic plasmacytoid dendritic cell neoplasm, combination therapy, hypomethylating agent, venetoclax
MeSH terms: Case Reports

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an extremely rare hematological malignancy with a highly aggressive behavior and median survival of <2 years. Especially, most BPDCN patients present with extensive and non-specific skin lesions, usually leading to misdiagnosis as a skin disease and delay therapy. As for treatment, most patients with BPDCN experience relapse shortly after treatment with the traditional regimens. The alleviation of skin symptoms reflects the effects of clinical treatments. Herein, we report a case of a 71-year-old man with intermittent and gradually expanding skin lesions over his chest, abdomen, and back for 1 year. On admission, physical examination revealed extensive skin lesions and multiple enlarged lymph nodes. Laboratory examinations showed pancytopenia and numerous malignant cells in the peripheral blood smear (60%), bone marrow aspirate smear (73.5%). Immunophenotyping using flow cytometry and immunohistochemistry presented large numbers of BPDCN cells in the bone marrow, cervical lymph nodes and dermal tissue. PET/CT revealed multiple enlarged lymph nodes and splenomegaly. Once the diagnosis was identified as BPDCN, the patient began treatment with the oral BCL2 inhibitor venetoclax and subcutaneously administered azacitidine. After the first course, skin lesions reduced markedly and complete remission was achieved in the bone marrow. Our study and current cumulative data according to reviewing systematically suggest that venetoclax combined with azacitidine is safe, effective, and applicable in the treatment of BPDCN, especially for elderly relapsed/refractory patients. This study, therefore, significantly contributes to the literature on the current and future treatment for BPDCN.
Immune checkpoint inhibitor-related acquired amegakaryocytosis thrombocytopenia: a case report and literature review
Authors: Valérian Rivet, Vincent Sibaud, Jérémie Dion, Thibaut Volosov, Mélanie Biteau, Andréa Pastissier, Karen Delavigne, Pierre Cougoul, Odile Rauzy, Thibault Comont
Journal: Front Oncol (2024)
Keywords: acquired megakaryocytic thrombocytopenia, eltrombopag, immune checkpoint inhibitors (ici), immune-related adverse events (irae), nivolumab, thrombopoietin receptor agonist (tpo-ra)
MeSH terms: Case Reports

Introduction:
Immune checkpoint inhibitors (ICIs) are used in several advanced malignancies and may cause various immune-related adverse events (irAEs). Among them, hematological irAEs are less described. Acquired amegakaryocytic thrombocytopenia (AAT) is a rare immune hematologic disorder characterized by severe thrombocytopenia and complete absence of megakaryocytes in bone marrow.
Case presentation:
Herein, we present the case of a patient in their 40s with metastatic melanoma who developed an AAT after 12 cycles of nivolumab (anti-PD1). His platelet count decreased by ≤5 × 109/l without other cytopenia. Bone marrow biopsy showed normal cellularity with a complete absence of megakaryocyte and T-CD8+ lymphocyte infiltration. Given the failure of systemic steroids, eltrombopag was started, an oral thrombopoietin receptor agonist (TPO-RA), and his platelet count subsequently increased with complete response.
Discussion:
Four other cases are described on literature with the same features than non-ICI-related AAT. All cases occurred after anti-PD/PD-L1 treatment with a median onset of 5 weeks. The presentation of our case is quite different with delayed cytopenia. Both ciclosporin and TPO-RA seem to be efficient therapies.
Conclusion:
TPO-RA could be preferred in oncologic patients, but safety data are still missing to define clear guidelines for immune-related AAT management.
Case Report: Insulin hypersensitivity in youth with type 1 diabetes
Authors: Einas H Alkhatib, Jody B Grundman, Anna M Adamusiak, Melena D Bellin, Joel P Brooks, Kevin S Buckley, Erin M Janssen, Maleewan Kitcharoensakkul, Kyle P McNerney, Thea L Pfeifer, Brooke I Polk, Brynn E Marks
Journal: Front Endocrinol (Lausanne) (2023)
Keywords: insulin allergy, insulin hypersensitivity, pancreas transplant, pediatrics, type 1 diabetes
MeSH terms: Diabetes Mellitus, Type 1 / complications, Diabetes Mellitus, Type 1 / drug therapy, Drug Hypersensitivity / diagnosis, Drug Hypersensitivity / etiology, Urticaria / chemically induced, Urticaria / complications, Urticaria / drug therapy, Review, Case Reports, Adolescent, Child, Humans, Insulin / adverse effects, Quality of Life, Insulin

Objective:
Immediate type I, type III, and delayed type IV hypersensitivity reactions to insulin are rare, but potentially serious complications of exogenous insulin administration required for the treatment of type 1 diabetes (T1D).
Methods:
We present four cases of insulin hypersensitivity reactions occurring in youth with T1D and a literature review of this topic.
Results:
Insulin hypersensitivity reactions included types I, III, and IV with presentations ranging from localized urticaria, erythematous nodules, and eczematous plaques to anaphylaxis with respiratory distress. Reactions occurred in youth with newly diagnosed T1D and in those with long-standing T1D who were using both injection and insulin pump therapy. Multidisciplinary care involving pediatric endocrinology and allergy/immunology utilizing trials of many adjunct therapies yielded minimal improvement. Despite the use of various treatments, including antihistamines, topical therapies, immunosuppressant medications, desensitization trials, and intravenous immune globulin, cutaneous reactions, elevated hemoglobin A1c levels, and negative effects on quality of life remain persistent challenges. One patient became one of the youngest pancreas transplant recipients in the world at age 12 years due to uncontrollable symptoms and intolerable adverse effects of attempted therapies.
Conclusion:
Although rare, insulin hypersensitivity reactions negatively affect glycemic control and quality of life. These cases demonstrate the varying severity and presentation of insulin hypersensitivity reactions along with the limited success of various treatment approaches. Given the life-sustaining nature of insulin therapy, further studies are needed to better understand the underlying pathophysiology of insulin hypersensitivity and to develop targeted treatment approaches.
A young adult with leptospirosis associated acute inflammatory demyelinating polyneuropathy
Authors: Herman Bagula, Ismail Banderker, Muhammed S Moosa
Journal: S Afr J Infect Dis (2023)
Keywords: aidp, guillain-barré syndrome, leptospirosis, polyneuropathy, post infectious
MeSH terms: Case Reports

Leptospirosis is a zoonotic disease that commonly affects the liver and kidney. It can rarely affect the neurological system with aseptic meningitis being the commonest neurological presentation. We present the case of a patient with leptospirosis complicated by acute inflammatory demyelinating polyneuropathy.
Contribution:
To our knowledge, this is the first reported case of acute inflammatory demyelinating polyneuropathy as a complication of leptospirosis in South Africa.
Association of the Osseodensification Technique with Soft Tissue Substitute in a Limitrophe Edentulous Area: A Clinical Case Report with 5-Year Follow-Up
Authors: André Luiz Berbel de Souza, Rogério Jorqueira Dos Reis, Rodrigo Mendes Ferreiro Girondo, Rafaela Pascon, Alexandre Cabrera, Gustavo Vargas da Silva Salomão
Journal: Case Rep Dent (2024)
MeSH terms: Case Reports

The osseodensification (OD) technique differs from conventional milling for dental implant installation in that it preserves the prepared bone and compacts it toward the apex and lateral walls of the socket, resulting in bone compaction. By enabling autografting, bone expansion, and high implant insertion torques, OD has become an increasingly popular option. The aim of this clinical case report is to demonstrate the predictability of combining OD with guided bone and tissue regeneration (GBR/GTR) techniques for bone expansion in the maxilla with reduced thickness, while avoiding other reconstructive surgeries. The report presents the treatment of a 32-year-old female patient who had cosmetic concerns regarding the anterior maxillary region. The patient was using an adhesive prosthesis with pontic on tooth 13 fixed between teeth 12 and 14. After the case was planned, it was decided that bone expansion in the region would be performed using the OD technique. The implant installation (AR Torque, 3.5 × 11.5 mm, Conexão®) and guided bone regeneration (GBR) were done with the assistance of L-PRF (Stick Bone, associated with L-PRF membrane). Following the osseointegration period, a provisional resin crown was fabricated, and a collagen matrix membrane (Mucoderm®) was used to increase vestibular soft tissue volume and shape the patient's gingival profile. After a period of 120 days, the final crown was created and observed for a span of 5 years. The results showed stability of the case along with maintaining its esthetic and satisfactory function. The use of the osseodensification technique coupled with a connective tissue graft substitute has been anticipated for a long time. It has proven to be an excellent alternative to autogenous grafts.
Phacoemulsification in Nanophthalmic Eye, a Way to Manage Glaucoma: Case Report
Authors: Dania Bamefleh, Konrad Schargel, Valmore A Semidey, Faisal A Altahan, Edward Schargel
Journal: Case Rep Ophthalmol Med (2024)
Keywords: closed-angle glaucoma, nanophthalmos, phacoemulsification, short eyes
MeSH terms: Case Reports

A rare condition called nanophthalmos causes variable degrees of vision impairment. One may present with nanophthalmos as a hereditary or sporadic condition. There have been documented cases of nanophthalmos treated with bilateral cataract extraction and intraocular lens (IOL) implantation for intractable secondary glaucoma or chronic angle-closure glaucoma. We describe a case of closed-angle glaucoma in a nanophthalmic eye with increased intraocular pressure (IOP) on full medical treatment, along with concurrent drug side effects. As a first surgical procedure, we recommend phacoemulsification of the clear lens + IOL. The challenge in treating nanophthalmic eyes lies in managing the possibility of developing glaucoma in an eye where anatomical conditions make surgery extremely risky. This must be balanced against the advantages of lessening exposure contact in the trabecular meshwork and optimizing the anterior chamber for potential future glaucoma surgery, which can improve the prognosis in these cases. Lastly, it is critical to have a thorough conversation with the patient about the aims, risks, and advantages. The patient's understanding and expectations should also be crystal apparent. The primary objective should always be to enhance the circumstances for the most effective glaucoma therapy, not to perform refractive surgery.
Successful bladder-sparing partial cystectomy for muscle-invasive domal urothelial carcinoma with sarcomatoid differentiation: a case report
Authors: Mark Sultan, Ahmad Abdelaziz, Muhammed A Hammad, Juan R Martinez, Shady A Ibrahim, Mahra Nourbakhsh, Ramy F Youssef
Journal: Ther Adv Urol (2024)
Keywords: bladder preserving therapy, case report, muscle invasive bladder cancer, partial cystectomy, sarcomatoid urothelial carcinoma
MeSH terms: Case Reports

High-grade (HG) urothelial carcinoma (UC) with variant histology has historically been managed conservatively. The presented case details a solitary lesion of muscle-invasive bladder cancer (MIBC) with sarcomatoid variant (SV) histology treated by partial cystectomy (PC) and adjuvant chemotherapy. A 71-year-old male with a 15-pack year smoking history presented after outside transurethral resection of bladder tumor (TURBT). Computerized tomography imaging was negative for pelvic lymphadenopathy, a 2 cm broad-based papillary tumor at the bladder dome was identified on office cystoscopy. Complete staging TURBT noted a final pathology of invasive HG UC with areas of spindle cell differentiation consistent with sarcomatous changes and no evidence of lymphovascular invasion. The patient was inclined toward bladder-preserving options. PC with a 2 cm margin and bilateral pelvic lymphadenectomy was performed. Final pathology revealed HG UC with sarcomatoid differentiation and invasion into the deep muscularis propria, consistent with pathologic T2bN0 disease, a negative margin, and no lymphovascular invasion. Subsequently, the patient pursued four doses of adjuvant doxorubicin though his treatment was complicated by hand-foot syndrome. At 21 months postoperatively, the patient developed a small (<1 cm) papillary lesion near but uninvolved with the left ureteral orifice. Blue light cystoscopy and TURBT revealed noninvasive low-grade Ta UC. To date, the patient has no evidence of HG UC recurrence; 8 years after PC. Patient maintains good bladder function and voiding every 3-4 h with a bladder capacity of around 350 ml. Surgical extirpation with PC followed by adjuvant chemotherapy may represent a durable solution for muscle invasive (pT2) UC with SV histology if tumor size and location are amenable. Due to the sparse nature of sarcomatous features within UC, large multicenter studies are required to further understand the clinical significance and optimal management options for this variant histology.
Case report on antipsychotic induced catatonia in an incarcerated patient
Authors: Han Nguyen, Ruslana Cannell, Suman Rollakanti, Johanna Rosenthal, David Seigler
Journal: Front Psychiatry (2023)
Keywords: bush-francis scale, antipsychotic induced catatonia, benzodiazepines, extrapyramidal symptoms, first-generation antipsychotics, forensic psychiatry, haloperidol decanoate, long acting injectable
MeSH terms: Case Reports

Antipsychotic-induced catatonia is an iatrogenic and debilitating adverse reaction, but there is a dearth of recent documented cases. This report describes a 35-year-old incarcerated Korean-American male with a history of unspecified psychosis who presented for antipsychotic induced catatonia after administration of haloperidol decanoate intramuscular (200 mg across the span of 1 week). Neurologic workup was performed including MRI, lumbar puncture, and electroencephalography. Despite an approximate month long hospitalization, benzodiazepine challenge, benztropine trial, and amantadine adjunct, our patient continued to experience bradykinesia, waxy flexibility, and mask-like facies, and was minimally verbally responsive. Several challenges in the treatment of incarcerated individuals at the hospital are highlighted in this case report, including adverse reaction to medication, difficulty of care coordination, and limited access to health records among providers.
Is arterial spin labelling necessarily low perfusion for cavernous sinus venous malformation? A case of hyperperfusion cavernous sinus venous malformation
Authors: Dan Luo, Xinlan Xiao
Journal: BJR Case Rep (2023)
Keywords: asl, cavernous sinus venous malformation, meningiomas
MeSH terms: Case Reports

Generally, due to the complexity of the skull base structures, it is difficult to differentiate cavernous vascular malformation and meningioma in the cavernous sinus area using conventional imaging studies. Cavernous sinus venous malformation are characterized by increased capillary masses without a direct arterial supply, typically leading to low perfusion. On the other hand, meningiomas receive arterial blood supply to the tumour and often exhibit high perfusion. So, arterial spin labelling (ASL) can be helpful in distinguishing between the 2 tumour types. However, in our specific case of a cavernous sinus venous malformation, the ASL imaging showed hyperperfusion. Further analysis revealed that this hyperperfusion on ASL can occur when cavernous sinus venous malformation is associated with arteriovenous fistula malformation.
Bilateral Endophthalmitis in a Postpartum Immunocompetent Patient
Authors: Rola N Hamam, Giulia Firmani
Journal: Case Rep Ophthalmol Med (2024)
MeSH terms: Case Reports

Purpose:
The aim of this report is to present the onset of bilateral endophthalmitis negative to culture testing and vitreous tapping in a postpartum immunocompetent patient.
Methods:
A 33-year-old patient developed floaters and severe blurry vision in both eyes 3 weeks after childbirth. With no previous surgery, no comorbidities in her clinical history, and negative diagnostic reports, endogenous endophthalmitis was suspected. Two days later, a pars plana vitrectomy was performed in both eyes one week apart, and intravitreal antibiotics and antifungals were administered during the surgery. No infectious source was identified since the cultures from the aqueous and vitreous humor returned negative in both cases.
Results:
Residual fibrosis around the fovea in the right eye and on the optic disc in the left eye was described. Nevertheless, the best corrected visual acuity of the patient was 20/20 in both eyes 4 months after the onset of the presumed endogenous endophthalmitis.
Conclusion:
This is the first report presenting a bilateral case of postpartum endophthalmitis negative to culture testing and vitreous tapping in a healthy patient with no previous surgeries nor long-term treatment. Early pars plana vitrectomy was fundamental for the correct management of this condition.
Case report: Susac syndrome-two ends of the spectrum, single center case reports and review of the literature
Authors: Martina Cviková, Jakub Štefela, Vít Všianský, Michal Dufek, Irena Doležalová, Jan Vinklárek, Roman Herzig, Markéta Zemanová, Vladimír Červeňák, Jaroslav Brichta, Veronika Bárková, David Kouřil, Petr Aulický, Pavel Filip, Viktor Weiss
Journal: Front Neurol (2024)
Keywords: susac syndrome, black blood imaging, branch retinal arterial occlusion, hearing loss, neuroimmunology, stroke, vasculitis
MeSH terms: Case Reports

Susac syndrome is a rare and enigmatic complex neurological disorder primarily affecting small blood vessels in the brain, retina, and inner ear. Diagnosing Susac syndrome may be extremely challenging not only due to its rarity, but also due to the variability of its clinical presentation. This paper describes two vastly different cases-one with mild symptoms and good response to therapy, the other with severe, complicated course, relapses and long-term sequelae despite multiple therapeutic interventions. Building upon the available guidelines, we highlight the utility of black blood MRI in this disease and provide a comprehensive review of available clinical experience in clinical presentation, diagnosis and therapy of this disease. Despite its rarity, the awareness of Susac syndrome may be of uttermost importance since it ultimately is a treatable condition. If diagnosed in a timely manner, early intervention can substantially improve the outcomes of our patients.
Case report: Near-fatal hypermagnesemia resulting from the use of Epsom salts in a patient with normal renal function
Authors: Gui-Fei Si, Yu-Xin Ge, Xiao-Pan Lv, Yu-Quan Li, Xue-Mei Chen, Xue-Min Yuan
Journal: Front Med (Lausanne) (2024)
Keywords: epsom salts, hypermagnesemia, magnesium sulfate, normal renal function, peptic ulcer, subtotal gastrectomy
MeSH terms: Case Reports

Hypermagnesemia commonly occurs in patients with renal dysfunction. Diagnosing hypermagnesemia represents a challenge due to its rarity and the absence of routine monitoring of magnesium levels. Furthermore, the lack of awareness among clinicians regarding this uncommon condition frequently leads to delayed diagnoses. Few patients survive with a serum magnesium level exceeding 7 mmol/L. This article presents a case study of near-fatal hypermagnesemia resulting from the oral administration of Epsom salts in a patient with normal renal function. A 60-year-old female presented to the gastroenterology department on Oct. 6, 2023, with a 3-day history of black stools. She underwent subtotal gastrectomy in 2005 and has a stable history of nephrotic syndrome. To investigate the cause of her bleeding, electronic gastroscopy and colonoscopy were scheduled for Oct. 11, 2023. She experienced a sudden loss of consciousness 30 min after the ingestion of Epsom salts. The attending physician suspected a severe magnesium poisoning. She was promptly administered calcium gluconate, underwent tracheal intubation with ambu bag ventilation, and received early continuous renal replacement therapy (CRRT). Swift diagnosis and CRRT contributed to a reduction in her serum magnesium levels from an initial 8.71 mmol/L to 1.35 mmol/L, leading to a remarkable improvement in the toxic symptoms associated with hypermagnesemia. Subsequently, she was managed in the gastroenterology department, with gastroscopy revealing bleeding from the gastrointestinal anastomotic ulcer. Following conservative treatments including acid suppression, stomach protection, and hemostasis, her symptoms improved, and she was successfully discharged. This study aims to alert clinicians to the possibility of hypermagnesemia in individuals with normal renal function. Physicians should exercise caution when prescribing Epsom salts to patients with underlying gastrointestinal conditions. If necessary, alternative drug therapies may be considered to mitigate the risk of hypermagnesemia. Timely intervention is pivotal in averting life-threatening complications linked to hypermagnesemia.