Sandy, Natascha S. MD1; Cavalaro-Silva, Marcia Alessandra C. P. MD2; Cardoso, Silvia R. MD3; Bellomo-Brandao, Maria Angela MD, PhD3
doi : 10.14309/crj.0000000000000542
March 2021 - Volume 8 - Issue 3 - p e00542
Azañedo, Claudia MD1; Larrea, Pedro MD1; Alva, Edgar MD1; León-Moreno, Julio MD1
doi : 10.14309/crj.0000000000000545
March 2021 - Volume 8 - Issue 3 - p e00545
Hanna, Angy MD1; Gjeorgjievski, Mihajlo MD1; Amin, Mitual MD2; Cappell, Mitchell S. MD, PhD1,3
doi : 10.14309/crj.0000000000000543
March 2021 - Volume 8 - Issue 3 - p e00543
McLain, Julian H. MD1; Khadarian, Kevork MD1; Shojaie, Layla MD1; Lubman, Richard MD1; Chang, Ching-Fei MD1; Lindgren, Brett DO1; Shao, Ling MD, PhD1
doi : 10.14309/crj.0000000000000517
March 2021 - Volume 8 - Issue 3 - p e00517
A 35-year-old woman presented to the hospital with a 4-week history of large-volume chylous ascites refractory to paracentesis and new-onset dyspnea. Thoracic computed tomography revealed diffuse pulmonary cystic lesions with pleural effusions, and abdominal computed tomography showed ascites with large bilateral retroperitoneal masses displaying positron emission tomography avidity. Biopsy of the masses demonstrated lymphatic invasion by a perivascular epithelioid cell neoplasm, a smooth muscle tumor. The patient was diagnosed as having the sporadic form of lymphangioleiomyomatosis and was treated with the mammalian target of rapamycin pathway inhibitor sirolumus with clinical improvement.
Myneedu, Kanchana MD1; Chavez, Luis O. MD1; Sussman, Norman L. MD2; Michael, Majd MD3; Padilla, Angelica MD4; Zuckerman, Marc J. MD3
doi : 10.14309/crj.0000000000000547
March 2021 - Volume 8 - Issue 3 - p e00547
Common variable immunodeficiency (CVID) is characterized by defective immunoglobulin synthesis because of impaired B-cell function. Liver abnormalities including autoimmune hepatitis (AIH) have been described in up to 10% of patients. We report a 27-year-old woman with CVID who presented with liver dysfunction secondary to AIH. AIH is both uncommon and challenging diagnostically in patients with CVID because they have low IgG levels and often have low or undetectable autoantibody levels. Liver biopsy and response to therapy play an important role in establishing the diagnosis. Corticosteroids are the mainstay of therapy, with or without immune modulators.
Patel, Priyesh MD1; Chadalavada, Pravallika MD2; Singh, Amandeep MD3; Gurajala, Ram Kishore MD1; Achkar, Jean-Paul MD3
doi : 10.14309/crj.0000000000000550
March 2021 - Volume 8 - Issue 3 - p e00550
The presentation of an upper gastrointestinal bleed secondary to an accessory splenic artery is a rare circumstance described only in 2 previous case reports. This report is the first to describe an upper gastrointestinal bleed consequent of a submucosal accessory splenic artery arising from the left phrenic artery, requiring multiple endoscopies and endovascular embolization. Vascular anatomic variants can pose a challenge to treatment, especially when they are unknown. This case adds to the limited number of case reports involving accessory splenic arteries.
Lee, Sean MD1; Varshney, Neha MD2; Taleban, Sasha MD3
doi : 10.14309/crj.0000000000000549
March 2021 - Volume 8 - Issue 3 - p e00549
Atrophic gastritis can be environmental in origin and involve the antrum or autoimmune in origin and involve the body and fundus. We present a rare case of autoimmune atrophic pangastritis, a distinct type of autoimmune gastritis affecting the entire stomach, which should be considered in patients with other autoimmune disorders.
Evans, Neil Shadbeh MD1; Ray, Jennifer MD2; Prather, Charlene MD2
doi : 10.14309/crj.0000000000000525
March 2021 - Volume 8 - Issue 3 - p e00525
Familial Mediterranean fever (FMF) was previously believed to be an autosomal recessive disease. We present a patient with only one pathogenic variation of the MEFV gene due to the c.2177T>C mutation. The patient had clinical features of recurrent fevers and abdominal pain, serositis, and a history of multiple abdominal surgeries for pain. He was eventually diagnosed with FMF. This case report demonstrates an example of the rare autosomal-dominant phenotype of FMF.
Patel, Rupal H. MD1; Everett, Brendan T. MD2; Akselrod, Dmitriy MD3; Frasca, Joseph D. MD2; Gordon, Stuart R. MD1
doi : 10.14309/crj.0000000000000548
March 2021 - Volume 8 - Issue 3 - p e00548
We report the case of a patient with a benign refractory esophagojejunal anastomotic stricture for which a 20-mm lumen-apposing metal stent was placed, resulting in a fatal aortoenteric fistula. We report this case to alert others to this potential complication of LAMS placement for esophageal strictures and recommend caution when using the 20-mm LAMS in similar settings.
Shaheen, Aciel Ahmed MD1; Gill, Inayat DO1; Edhi, Ahmed Iqbal MD1; Amin, Mitual MD2,3; Cappell, Mitchell S. MD, PhD1,4
doi : 10.14309/crj.0000000000000546
March 2021 - Volume 8 - Issue 3 - p e00546
Pancreatic ganglioneuromas occur mostly in children and rarely in young adults, with no cases reported in adults older than 60 years. An 86-year-old-woman, with active advanced multiple myeloma, presented with epigastric pain for 2 days. Abdominal and pelvic computed tomography demonstrated a distended gallbladder, mildly dilated biliary tree, and a 13 × 8-mm hypodense mass in pancreatic body, without extrapancreatic invasion at endoscopic ultrasound. Fine-needle endoscopic ultrasound–guided core biopsy revealed characteristic histopathology of ganglioneuroma, as confirmed by immunohistochemical positivity for S100, SOX-10, and synaptophysin. This demonstrates novel finding of pancreatic ganglioneuroma occurring in the elderly. Lesion inclusion in the differential diagnosis may mandate tissue for pathologic diagnosis and complete lesion resection.
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