{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,19]],"date-time":"2026-02-19T07:37:01Z","timestamp":1771486621103,"version":"3.50.1"},"reference-count":25,"publisher":"BMJ","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Reports"],"accepted":{"date-parts":[[2016,2,25]]},"abstract":"<jats:p>AL amyloidosis is a clonal plasma cell proliferative disorder characterised by extracellular tissue deposits of insoluble fibrils derived from \u03ba or \u03bb immunoglobulin light chains. The most common organs affected by AL amyloidosis are the kidney, presenting with nephrotic syndrome and\/or progressive renal dysfunction, and the heart, with restrictive cardiomyopathy. Hepatic deposition of fibrils occurs in half the cases but the liver is rarely the predominantly affected organ. The most common presentation of hepatic amyloidosis is hepatomegaly with elevated alkaline phosphatase. Acute liver failure with cholestasis and jaundice is a rare complication, with a prevalence of approximately 5%, and is usually associated with a worse prognosis. We report a case of a 39-year-old man admitted to our nephrology department with an unusual presentation of primary amyloidosis with nephrotic syndrome and acute liver failure, complicated by obstructive cholestasis resulting in death 2\u2005months after diagnosis.<\/jats:p>","DOI":"10.1136\/bcr-2016-214392","type":"journal-article","created":{"date-parts":[[2016,3,10]],"date-time":"2016-03-10T09:43:29Z","timestamp":1457603009000},"page":"bcr2016214392","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":7,"title":["Acute liver failure due to primary amyloidosis in a nephrotic syndrome: a swiftly progressive course"],"prefix":"10.1136","volume":"2016","author":[{"given":"Brigite Aguiar","family":"Cardoso","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rita","family":"Leal","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Helena","family":"S\u00e1","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M\u00e1rio","family":"Campos","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2016,3,10]]},"reference":[{"key":"2025042702560571000_2016.mar09_2.bcr2016214392.1","first-page":"1","article-title":"AL Amyloidosis","volume":"54","author":"Desport","year":"2012","journal-title":"Orphanet J Rare Dis"},{"key":"2025042702560571000_2016.mar09_2.bcr2016214392.2","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9270(98)00509-7"},{"key":"2025042702560571000_2016.mar09_2.bcr2016214392.3","first-page":"205","article-title":"Subacute liver failure secondary to amyloid light-chain amyloidosis","volume":"8","author":"Hydes","year":"2012","journal-title":"Gastroenterol Hepatol (N Y)"},{"key":"2025042702560571000_2016.mar09_2.bcr2016214392.4","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2010-06-290668"},{"key":"2025042702560571000_2016.mar09_2.bcr2016214392.5","doi-asserted-by":"publisher","DOI":"10.1007\/s11899-009-0013-6"},{"key":"2025042702560571000_2016.mar09_2.bcr2016214392.6","doi-asserted-by":"publisher","DOI":"10.4065\/83.3.297"},{"key":"2025042702560571000_2016.mar09_2.bcr2016214392.7","doi-asserted-by":"crossref","first-page":"1817","DOI":"10.1182\/blood.V79.7.1817.1817","article-title":"Incidence and natural history of primary systemic amyloidosis in Olmsted County, Minnesota, 1950 through 1989","volume":"79","author":"Kyle","year":"1992","journal-title":"Blood"},{"key":"2025042702560571000_2016.mar09_2.bcr2016214392.8","first-page":"665","article-title":"Amyloidosis AL. Clinical and laboratory features in 229 cases","volume":"58","author":"Kyle","year":"1983","journal-title":"Mayo Clin Proc"},{"key":"2025042702560571000_2016.mar09_2.bcr2016214392.9","first-page":"45","article-title":"Primary systemic amyloidosis: clinical and laboratory features in 474 Cases","volume":"32","author":"Kyle","year":"1995","journal-title":"Semin Hematol"},{"key":"2025042702560571000_2016.mar09_2.bcr2016214392.10","doi-asserted-by":"publisher","DOI":"10.1373\/clinchem.2004.046870"},{"key":"2025042702560571000_2016.mar09_2.bcr2016214392.11","doi-asserted-by":"publisher","DOI":"10.1046\/j.1523-1755.2002.00085.x"},{"key":"2025042702560571000_2016.mar09_2.bcr2016214392.12","doi-asserted-by":"publisher","DOI":"10.1111\/j.1523-1755.2004.00833.x"},{"key":"2025042702560571000_2016.mar09_2.bcr2016214392.13","first-page":"769","article-title":"Subacute renal failure as a form of presentation of Al-amyloidosis","volume":"27","author":"Vozmediano","year":"2007","journal-title":"Nefrologia"},{"key":"2025042702560571000_2016.mar09_2.bcr2016214392.14","doi-asserted-by":"crossref","first-page":"511","DOI":"10.1111\/j.1537-2995.2006.01143.x","article-title":"Light-chain removal by plasmapheresis in myeloma-associated renal failure","volume":"47","author":"Cserti","year":"2007","journal-title":"Transfusion"},{"key":"2025042702560571000_2016.mar09_2.bcr2016214392.15","doi-asserted-by":"publisher","DOI":"10.1177\/106689699300100108"},{"key":"2025042702560571000_2016.mar09_2.bcr2016214392.16","doi-asserted-by":"publisher","DOI":"10.1097\/01.md.0000091183.93122.c7"},{"key":"2025042702560571000_2016.mar09_2.bcr2016214392.17","doi-asserted-by":"publisher","DOI":"10.1586\/egh.09.59"},{"key":"2025042702560571000_2016.mar09_2.bcr2016214392.18","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-10-388462"},{"key":"2025042702560571000_2016.mar09_2.bcr2016214392.19","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2015-01-620302"},{"key":"2025042702560571000_2016.mar09_2.bcr2016214392.20","doi-asserted-by":"crossref","unstructured":"DeLeve LD . Cancer chemotherapy. In: Kaplowitz N , DeLeve LD , eds. Drug-induced liver disease. 3rd edn. Amsterdam: Elsevier, 2013:551.","DOI":"10.1016\/B978-0-12-387817-5.00030-3"},{"key":"2025042702560571000_2016.mar09_2.bcr2016214392.21","doi-asserted-by":"publisher","DOI":"10.1002\/hep.1840040121"},{"key":"2025042702560571000_2016.mar09_2.bcr2016214392.22","doi-asserted-by":"publisher","DOI":"10.1038\/nrrheum.2010.84"},{"key":"2025042702560571000_2016.mar09_2.bcr2016214392.23","doi-asserted-by":"publisher","DOI":"10.1097\/00007611-199902000-00016"},{"key":"2025042702560571000_2016.mar09_2.bcr2016214392.24","doi-asserted-by":"publisher","DOI":"10.1016\/S0025-6196(12)65706-1"},{"key":"2025042702560571000_2016.mar09_2.bcr2016214392.25","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(88)90505-0"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2016-214392","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,27]],"date-time":"2025-04-27T09:56:14Z","timestamp":1745747774000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2016-214392"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,3,10]]},"references-count":25,"alternative-id":["10.1136\/bcr-2016-214392"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2016-214392","relation":{},"ISSN":["1757-790X"],"issn-type":[{"value":"1757-790X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,3,10]]}}}