OpenMed/multicare-articles huggingface dataset:
Systemic Lupus Erythematosus (SLE) with Acute Nephritis, Antineutrophil Cytoplasmic Antibody- (ANCA-) Associated Vasculitis, and Thrombotic Thrombocytopenic Purpura (TTP): A Rare Case Report with Literature Review
Authors: Sohail Farshad, Christopher Kanaan, Solomiia Savedchuk, Dillon S Karmo, Alexandra Halalau, Abhishek Swami
Journal: Case Rep Rheumatol (2019)
MeSH terms: Case Reports

Thrombotic thrombocytopenic purpura (TTP) is a potentially fatal disorder that requires urgent identification and treatment. The association of TTP with systemic lupus erythematosus (SLE) and vasculitis has been reported, however, never simultaneously. A 33-year-old woman with a history of SLE presented with acute abdominal pain, fever, arthralgias, and skin rash. She had acute severe hypertension, diffuse abdominal tenderness, and petechial rash. Diagnostic work-up revealed active urine sediment with proteinuria and hematuria and elevated creatinine, anemia, and thrombocytopenia. She was diagnosed with acute lupus nephritis and early microangiopathic hemolytic anemia in the setting of hypertensive urgency and started on intravenous methylprednisolone 500 mg once a day. Within 48 hours, she developed shock with multiorgan dysfunction and succumbed to her illness. Laboratory tests later showed ADAMTS13 activity less than 10% consistent with TTP and p-antineutrophil cytoplasmic antibody (ANCA) positivity. Autopsy revealed small-vessel vasculitis of the visceral organs. Kidney biopsy demonstrated diffuse proliferative glomerulonephritis. This case illustrates the occurrence of SLE nephritis, p-ANCA vasculitis, and severe TTP with rapidly fatal course, and the importance of having a low threshold for initiating plasma exchange therapy. Here, we discuss the case and provide a literature review on cases of TTP with SLE and vasculitis.
Laparoscopic resection of a gastric plasma cell granuloma: a case report
Authors: Christopher Horn, Huw G Jones, Gareth Leopold, Anna Mainwaring, Ashraf Rasheed
Journal: Case Rep Pathol (2012)
MeSH terms: Case Reports

Plasma cell granuloma, also known as inflammatory myofibroblastic tumour or inflammatory pseudotumour, is a nonneoplastic process characterized by an unregulated growth of inflammatory cells. It most commonly occurs in the lung and upper respiratory tract, and only six other cases of gastric plasma cell granuloma exist. There are no other cases of intragastric laparoscopic resection of this type of lesion. Here, we present a case of a 60-year-old gentleman who had gradual onset epigastric discomfort and was thought to have a gastrointestinal stromal tumour on gastroscopy. Subsequent imaging and laparoscopic transgastric resection of the lesion confirmed the presence of a plasma cell granuloma. We discuss the aetiologies, presentation, investigation, and treatment of this rare disorder and make recommendations on the management.
Denosumab for the Treatment of Hypercalcemia in a Patient With Parathyroid Carcinoma: A Case Report
Authors: Abdallah Roukain, Heba Alwan, Massimo Bongiovanni, Gerasimos P Sykiotis, Peter A Kopp
Journal: Front Endocrinol (Lausanne) (2022)
Keywords: rankl, calcimimetics, denosumab, hypercalcemia, parathyroid carcinoma
MeSH terms: Denosumab / therapeutic use, Hypercalcemia / drug therapy, Parathyroid Neoplasms / complications, Case Reports, Review, Aged, 80 and over, Calcium / blood, Female, Humans, Hypercalcemia / etiology, Denosumab, Calcium

Background:
Refractory hypercalcemia is one of the major complications of parathyroid carcinoma.
Case report:
An 84-year old female patient presented with an acute confusional state due to hypercalcemia. This led to the diagnosis of primary hyperparathyroidism for which she underwent surgery. The initial histological diagnosis was interpreted as atypical parathyroid adenoma; the resection was microscopically incomplete. One year later, the patient presented with elevated calcium levels up to 3.89 mmol/l. Recurrent severe hypercalcemia required multiple hospitalizations. Review of the histology slides revealed that the initially resected lesion was in fact a parathyroid carcinoma. Treatment with the calcimimetic drug cinacalcet was poorly tolerated. Repeated administration of zoledronic acid only had transient effects on calcium levels, and bisphosphonate treatment was ultimately discontinued because of chronic renal failure. The patient then received denosumab (60 or 120 mg) when needed (nine doses over twenty months), the last dose in November 2020, which led to a reduction and control of here calcium levels. Currently, at three years after initial surgery, calcium levels are stable between 2.7-2.8 mmol/l and the patient has not required hospitalization for hypercalcemia for 10 months.
Discussion:
In case of parathyroid carcinoma, en-bloc resection is the first treatment. Denosumab has proven its efficiency in treating hypercalcemia in malignancy. Several case reports studied denosumab in hypercalcemia due to parathyroid carcinoma, and the treatment were efficient to decrease levels of calcium when repeated as needed or monthly. We report another case of refractory hypercalcemia treated with several doses of denosumab in a patient with parathyroid carcinoma.
Case Report: Mineralized Pulmonary Artery Thrombi in Two Dogs Treated for Meningoencephalitis of Unknown Origin
Authors: Suzanne Rosen, Leontine Benedicenti, Scott Petesch, Jennifer Reetz, Evelyn Marie Galban
Journal: Front Vet Sci (2020)
Keywords: cytosine arabinoside (ara-c), meningoencephalitis of unknown etiology (mue), meningoencephalitis of unknown origin, prednisone side effects, pulmonary arterial thromboembolism
MeSH terms: Case Reports

Meningoencephalitis of unknown origin (MUO) is a relatively common and very serious canine neurologic condition, which is typically associated with a poor long term prognosis despite treatment. This case series chronicles two dogs diagnosed with MUO who were treated with long term corticosteroids and cytosine arabinoside and lived well-beyond the typical survival time for this condition. Both eventually succumbed to respiratory signs associated with mineralized thrombi in their pulmonary arteries. Adverse effects from the two drugs used for treatment are reviewed in order to propose a possible mechanism to explain how long term use of these medications could result in such a phenomenon.
Acute thrombosis of the superior mesenteric artery in a 39-year-old woman with protein-S deficiency: a case report
Authors: Nicola Romano, Valerio Prosperi, Giancarlo Basili, Luca Lorenzetti, Valerio Gentile, Remo Luceretti, Graziano Biondi, Orlando Goletti
Journal: J Med Case Rep (2011)

Introduction:
Acute thromboembolic occlusion of the superior mesenteric artery is a condition with an unfavorable prognosis. Treatment of this condition is focused on early diagnosis, surgical or intravascular restoration of blood flow to the ischemic intestine, surgical resection of the necrotic bowel and supportive intensive care. In this report, we describe a case of a 39-year-old woman who developed a small bowel infarct because of an acute thrombotic occlusion of the superior mesenteric artery, also involving the splenic artery.
Case presentation:
A 39-year-old Caucasian woman presented with acute abdominal pain and signs of intestinal occlusion. The patient was given an abdominal computed tomography scan and ultrasonography in association with Doppler ultrasonography, highlighting a thrombosis of the celiac trunk, of the superior mesenteric artery, and of the splenic artery. She immediately underwent an explorative laparotomy, and revascularization was performed by thromboendarterectomy with a Fogarty catheter. In the following postoperative days, she was given a scheduled second and third look, evidencing necrotic jejunal and ileal handles. During all the surgical procedures, we performed intraoperative Doppler ultrasound of the superior mesenteric artery and celiac trunk to control the arterial flow without evidence of a new thrombosis.
Conclusion:
Acute mesenteric ischemia is a rare abdominal emergency that is characterized by a high mortality rate. Generally, acute mesenteric ischemia is due to an impaired blood supply to the intestine caused by thromboembolic phenomena. These phenomena may be associated with a variety of congenital prothrombotic disorders. A prompt diagnosis is a prerequisite for successful treatment. The treatment of choice remains laparotomy and thromboendarterectomy, although some prefer an endovascular approach. A second-look laparotomy could be required to evaluate viable intestinal handles. Some authors support a laparoscopic second-look. The possibility of evaluating the arteriotomy, during a repeated laparotomy with a Doppler ultrasound, is crucial to show a new thrombosis. Although the prognosis of acute mesenteric ischemia due to an acute arterial mesenteric thrombosis remains poor, a prompt diagnosis, aggressive surgical treatment and supportive intensive care unit could improve the outcome for patients with this condition.
Subtotal laparoscopic cholecystectomy for gangrenous gallbladder during recovery from COVID-19 pneumonia
Authors: Andrea Lovece, Emanuele Asti, Barbara Bruni, Luigi Bonavina
Journal: Int J Surg Case Rep (2020)
MeSH terms: Case Reports

Introduction:
Management of acute abdomen during COVID-19 pandemic may be challenging.
Presentation of case:
A 42-year old man was hospitalized for Covid-19 pneumonia. Fever, respiratory symptoms and hypoxemia significantly improved over the next 2 weeks, but the patient developed abdominal pain, nausea, and low-grade fever. Computed tomography scan revealed absence of contrast enhancement of gallbladder wall and a micro-perforation of the fundus. At laparoscopy, gallbladder gangrene was confirmed and a subtotal cholecystectomy performed. Special precautions were adopted for patient transportation from the ward to a dedicated operating room, and two teams with adequate personal protective equipment took charge of the procedure. The patient was discharged home on postoperative day 7 under protective lockdown measures for 2 weeks.
Discussion:
The pathogenesis of acute acalcolous gangrenous cholecystitis is multifactorial. It is unknown whether a prothrombotic state induced by COVID-19 contributes to wall ischemia and perforation. Percutaneous cholecystostomy should be avoided in patients with gallbladder gangrene. Contraindications to laparoscopy are not evidence-based since aerosolization is produced during both open and laparoscopic surgical procedures. However, personal protective equipment is key for prevention.
Conclusion:
Early diagnosis and surgical therapy are critical in patients with gangrenous cholecystitis. Subtotal laparoscopic cholecystectomy for gangrenous gallbladder is safe and effective.
Paget's Disease of the Vulva in Premenopausal Woman Treated with Only Surgery: A Case Report
Authors: Hamid Asmouki, Rachid Oumouloud, Abderrahim Aboulfalah, Abderraouf Soummani, Abdelouahed Marrat
Journal: Case Rep Oncol Med (2012)

Paget's disease of the vulva remains a rare condition with only a limited number of cases reported in the literature. It is an uncommon neoplasm usually of postmenopausal white women characterized by controversies in its prevalence, clinical features, treatment strategies, and prognostic. We here report a case of a primary Paget's disease of the vulva in premenopausal woman treated by only surgery with a favorable issue.
Duodenal Gangliocytic Paraganglioma Requiring a Pancreaticoduodenectomy: A Case Report and Review of the Literature
Authors: Laurie Adams, Theodore M Friedman, Timothy R Shaver, George Younan
Journal: Case Rep Surg (2018)
MeSH terms: Case Reports

Introduction:
Duodenal gangliocytic paragangliomas (GPs) are a subclass of duodenal neuroendocrine neoplasms and are exceedingly rare. They have been associated with an indolent behavior; however, they can rarely metastasize. Radical resection like a pancreaticoduodenectomy is sometimes indicated. We hereby present a case requiring major surgery and perform a literature search about this disease.
Presentation of case:
A 49-year-old Caucasian female, who presented with an upper gastrointestinal bleed, was found to have a hypodense mass in the second/third portion of the duodenum. A biopsy of the mass during upper endoscopy was inconclusive. A pancreaticoduodenectomy was recommended based on the high suspicion for a duodenal adenocarcinoma and was performed successfully. Her final pathology revealed a duodenal gangliocytic paraganglioma.
Discussion:
The majority of duodenal GPs present as gastrointestinal bleeds while others less commonly present with anemia, abdominal pain, duodenal obstructive symptoms, pancreatitis, or abnormal incidental findings on axial abdominal imaging. Duodenal GPs were initially viewed as benign tumors of the duodenum; however, there have been increasing incidence reports of hematogenous and lymphatic metastasis. Appropriate treatment of duodenal GPs is still controversial and ranges from local endoscopic submucosal resection to major radical surgery.
Conclusion:
Duodenal GPs are very rare tumors of the second portion of the duodenum presenting with upper gastrointestinal bleeding and local symptoms of surrounding organs. Local or radical resection is usually recommended to prevent bleeding and the minor risk of metastatic spread.
Novel Heterogenous CHS1 Mutations Identified in Five Japanese Patients with Chediak-Higashi Syndrome
Authors: Fuminori Tanabe, Hirotake Kasai, Michiko Morimoto, Shigeharu Oh, Hidetoshi Takada, Toshiro Hara, Masahiko Ito
Journal: Case Rep Med (2010)
MeSH terms: Case Reports

Chediak-Higashi syndrome (CHS) is a rare, autosomal recessive disorder characterized by oculocutaneous albinism, recurrent bacterial infections and progressive neurological dysfunction. We demonstrate novel heterogenous mutations of CHS1, the responsive gene of CHS, identified in five Japanese patients with CHS. Patients 1, 2, and 3 were siblings, and they had albinism of the skin and hair. They all had a heterogenous two-base deletion (c.5541-5542 del AA, p.Q1847fsX1850) in exon 18. Patient 4 had a heterogenous single-base insertion (c.3944-3945 ins C, p.T1315fsX1331) in exon 10. The patient exhibited severe early-onset phenotype and suffered from hemophagocytic lymphohistiocytosis. Patient 5 had two heterogenous nonsense mutations; c.7982C>G, p.S2661X in exon 30 and c.8281A>T, p.R2761X in exon 31. The patient suffered from infections in childhood and had visual disturbance and albinism of the skin and hair. The CHS1 mutations described here have not been reported previously.
Tension pneumocephalus related to radiotherapy for nasopharyngeal carcinoma
Authors: Esther Jimenez-Jimenez, Sebastià Sabater Martí, M Victoria Villas
Journal: Case Rep Oncol Med (2014)

Introduction. Tension pneumocephalus (TP) is a very rare complication related to radiotherapy for nasopharyngeal carcinoma (NPC). Case Presentation. A 46-year-old man was admitted to the hospital with an altered mental status and aqueous rhinorrhea for several hours of evolution. The computed tomography (CT) scan showed TP, a defect in the skull base and nasocranial fistula. The patient was receiving a second course of radiotherapy for local relapse. With medical treatment the patient recovered neurological status but died two days later. Discussion. In our knowledge, only 4 cases with similar characteristics have been reported in the literature. This is the first case report of TP during radiotherapy. TP was an abrupt and rapid process with neurological impairment for hours of evolution without suspicious osteoradionecrosis (OR) in previous scan images. The defect in the skull base could be due to a rapid disappearance of the tumor. The appearance of aqueous rhinorrhea and neurological symptoms must be viewed as signs of alarm.
Case report: transient small bowel intussusception presenting as right lower quadrant pain in a 6-year-old male
Authors: Mathew J Nelson, Tara Paterson, Christopher Raio
Journal: Crit Ultrasound J (2014)

In children presenting to the emergency room with right lower quadrant pain, ultrasound is the preferred initial modality. In our patient, a 6-year-old male with a sudden onset of severe right lower quadrant pain, the differential is broad, including appendicitis and intussusception. In order to narrow our differential and secure the diagnosis, our first modality was ultrasonography. With the increased use of point-of-care ultrasound in the emergency department, the diagnosis of appendicitis and ileo-colic intussusception has been made more frequently. In addition, other entities such as transient small bowel intussusception may be identified. As in our case, obstruction secondary to intussusception must be ruled out with observation, serial abdominal exams, clinical improvement, or further imaging.
Case report: Reducing the duration of positive-pressure ventilation for large mediastinal masses
Authors: Zaili Zhang, Meiru Jiang, Xijia Sun, Wenfei Tan
Journal: Front Cardiovasc Med (2022)
Keywords: case report, hyoxemia, large mediastinal mass, positive-pressure ventilation, sternotomy
MeSH terms: Case Reports

Large mediastinal masses (MMs) are rare and present some challenges in hemodynamic and airway management under general anesthesia. Multiple studies have reported cardiopulmonary collapse during general anesthesia. Maintenance of spontaneous ventilation, avoidance of muscle relaxants, and awake-intubation were usually recommended during general anesthesia for high-risk patients with large MMs. However, the recent notion challenged the classic teaching that maintaining spontaneous ventilation is superior to positive-pressure ventilation (PPV). In our case reports, we present two patients with large MMs during general anesthesia. In the first case, a 21-year-old male was administered a muscle relaxant during induction, followed by PPV, but his blood oxygen saturation decreased to 40% after 20 min. Finally, his oxygen saturation was restored by a sternotomy rather than by cardiopulmonary bypass (CPB) by femoral vascular intubation. In the second case, a 33-year-old male was also administered a muscle relaxant during induction followed by PPV, but for him, sternotomy was immediately performed, with stable blood oxygen saturation. Both patients recovered well and were discharged from hospital a week after surgery. Therefore, we present a recommendation that patients with large MMs could undergo PPV after the administration of a muscle relaxant during induction, but the cardiothoracic surgeon should immediately cleave the sternum.
Recurrent Gastrointestinal Stromal Tumors in the Imatinib Mesylate Era: Treatment Strategies for an Incurable Disease
Authors: Rebecca M Platoff, William F Morano, Luiz Marconcini, Nicholas DeLeo, Beth L Mapow, Michael Styler, Wilbur B Bowne
Journal: Case Rep Oncol Med (2017)
MeSH terms: Case Reports

Introduction:
Recurrence of gastrointestinal stromal tumors (GISTs) after surgical resection and imatinib mesylate (IM) adjuvant therapy poses a significant treatment challenge. We present the case of a patient who underwent surgical resection after recurrence and review the current literature regarding treatment.
Case presentation:
A 58-year-old man with a large intra-abdominal jejunal GIST was treated with complete surgical resection followed by IM. The patient experienced disease recurrence 3.5 years later and underwent IM dose escalation and reresection.
Conclusion:
Current strategies to treat recurrent GIST include dose escalation, modifying adjuvant tyrosine kinase inhibitor therapy, and surgery. High-level evidence will be required to better define the combinatory roles of tyrosine kinase inhibitor therapy, guided by molecular profiling, and surgery in the management of recurrent GIST.
Cecal bascule herniation through the foramen of Winslow
Authors: Aaron M Williams, Zachary Pickell, Mary R Shen, Naveen Sangji
Journal: Autops Case Rep (2021)
Keywords: colorectal surgery, hernia, intestinal obstruction, laparotomy
MeSH terms: Case Reports

Bowel obstructions can have a variety of causes, including impacted feces, adhesions, volvulus, non-internal hernias, and in rare cases internal hernias. We report a 63-year-old woman who presented to the emergency department with severe abdominal pain, nausea, vomiting, and obstructive symptoms that had started 12 hours earlier. A computed tomographic scan of the abdomen and pelvis showed a right internal hernia with a cecal bascule traversing through the foramen of Winslow, concerning for a closed-loop obstruction. The patient underwent an exploratory laparotomy with cecal bascule reduction and cecopexy. Given the increased mortality risk if undiagnosed, it is important to remain aware of internal hernias. Patient outcomes are markedly improved with early diagnosis and surgical intervention.
Rare aggressive natural killer cell leukemia presented with bone marrow fibrosis - a diagnostic challenge
Authors: Dina S Soliman, Ahmad Al Sabbagh, Halima El Omri, Firyal A Ibrahim, Aliaa M Amer, Ivone B Otazu
Journal: Springerplus (2014)
Keywords: aggressive natural killer cell leukemia, bone marrow fibrosis, dysmegakaryopoiesis

Aggressive natural killer cell leukemia is an extraordinary rare aggressive malignant neoplasm of natural killer cells. Although its first recognition as a specific entity was approximately 20 years ago, this leukemia has not yet been satisfactorily characterized as fewer than 200 cases have been reported in the literature and up to our knowledge, this is the first case report in Qatar. Reaching a diagnosis of aggressive natural killer leukemia was a challenging experience, because in addition to being a rare entity, the relative scarcity of circulating neoplastic cells, failure to obtain an adequate aspirate sample sufficient to perform flow cytometric analysis, together with the absence of applicable method to prove NK clonality (as it lack specific clonal marker); our case had atypical confusing presentation of striking increase in bone marrow fibrosis that was misleading and complicated the case further. The bone marrow fibrosis encountered may be related to the neoplastic natural killer cells' chemokine profile and it may raise the awareness for considering aggressive natural killer leukemia within the differential diagnosis of leukemia with heightened marrow fibrosis.
Pamidronate Rescue Therapy for Hypercalcemia in a Child With Williams Syndrome
Authors: Sami A Sanjad, Bilal Aoun, Halim Yammine, Amina Bassyouni, Pascale E Karam
Journal: Front Endocrinol (Lausanne) (2018)
Keywords: williams syndrome, furosemide, hypercalcemia, nephrocalcinosis, pamidronate
MeSH terms: Case Reports

A 15-month-old male infant diagnosed with Williams Syndrome (WS) was admitted with severe hypercalcemia and nephrocalcinosis. Intravenous hydration and furosemide failed to yield an appreciable and sustainable fall in serum calcium, while the injection of pamidronate achieved a significant decrease in serum calcium in a short period of time. This bisphosphonate could be considered as a second-line treatment for refractory hypercalcemia in WS.