{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,10,9]],"date-time":"2024-10-09T04:20:27Z","timestamp":1728447627382},"reference-count":7,"publisher":"Maad Rayan Publishing Company","issue":"3","content-domain":{"domain":["mejdd.org"],"crossmark-restriction":false},"short-container-title":["Middle East J Dig Dis"],"published-print":{"date-parts":[[2024,7,31]]},"abstract":"<jats:p>Background: Budd-Chiari syndrome (BCS) is characterized by hepatic venous outflow tract obstruction and is commonly associated with an underlying hypercoagulable state. Systemic amyloidosis is a disorder characterized by systemic deposition of misfolded proteins leading to end organ damage. Amyloidosis is commonly associated with coagulation abnormalities, mainly leading to increased bleeding diathesis. Here, we report a case of amyloid light chain (AL) amyloidosis presenting as BCS. A 40-year-old man presented with abdominal distension along with anorexia and weight loss. On evaluation, he had severe hypoalbuminemia, raised alkaline phosphatase, and non-visualization of hepatic veins on abdominal imaging. Further evaluation confirmed the diagnosis of AL amyloidosis with renal, cardiac, and hepatic involvement. AL amyloidosis rarely can present with BCS. A high index of suspicion is needed as symptoms can be variable and non-specific.<\/jats:p>","DOI":"10.34172\/mejdd.2024.391","type":"journal-article","created":{"date-parts":[[2024,10,8]],"date-time":"2024-10-08T08:32:48Z","timestamp":1728376368000},"page":"196-199","update-policy":"http:\/\/dx.doi.org\/10.34172\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Systemic Amyloidosis Presenting as Budd-Chiari Syndrome: A Case Report"],"prefix":"10.34172","volume":"16","author":[{"given":"Naman","family":"Lodha","sequence":"first","affiliation":[{"name":"Department of General Medicine, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Samarth","family":"Bhat K S","sequence":"additional","affiliation":[{"name":"Department of General Medicine, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kartikeya","family":"Mathur","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vikrant","family":"Verma","sequence":"additional","affiliation":[{"name":"Department of Pathology, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rengarajan","family":"Rajagopal","sequence":"additional","affiliation":[{"name":"Department of Diagnostic and Interventional Radiology, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chhagan","family":"Lal Birda","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ashish","family":"Agarwal","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"20123","published-online":{"date-parts":[[2024,7,31]]},"reference":[{"key":"R1","doi-asserted-by":"publisher","DOI":"10.1056\/nejm197707142970203"},{"key":"R2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.v97.6.1885"},{"key":"R3","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2001.02667.x"},{"key":"R4","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.21603"},{"key":"R5","doi-asserted-by":"publisher","DOI":"10.1080\/13506129.2017.1414042"},{"key":"R6","doi-asserted-by":"publisher","DOI":"10.1053\/j.ajkd.2006.03.036"},{"key":"R7","doi-asserted-by":"publisher","DOI":"10.3109\/13506129909007339"}],"container-title":["Middle East Journal of Digestive Diseases"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/mejdd.org\/index.php\/mejdd\/article\/view\/2998\/2371","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/mejdd.org\/index.php\/mejdd\/article\/view\/2998\/2371","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,10,8]],"date-time":"2024-10-08T08:32:51Z","timestamp":1728376371000},"score":1,"resource":{"primary":{"URL":"http:\/\/mejdd.org\/index.php\/mejdd\/article\/view\/2998"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,7,31]]},"references-count":7,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2024,7,31]]}},"URL":"https:\/\/doi.org\/10.34172\/mejdd.2024.391","relation":{},"ISSN":["2008-5249"],"issn-type":[{"value":"2008-5249","type":"print"}],"subject":[],"published":{"date-parts":[[2024,7,31]]},"assertion":[{"value":"Iranian Association of Gastroerterology and Hepatology","name":"journal_owner","label":"Journal Owner"},{"value":"Iranian Association of Gastroerterology and Hepatology","name":"journal_publisher","label":"Journal Publisher"},{"value":"2023-11-07","order":1,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-05-07","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-07-31","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}