{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,11]],"date-time":"2026-06-11T16:12:24Z","timestamp":1781194344707,"version":"3.54.1"},"reference-count":11,"publisher":"BMJ","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Reports"],"accepted":{"date-parts":[[2015,6,8]]},"abstract":"<jats:p>A man in his late 40s with sickle cell anaemia (HbSS) presented to the emergency department with 2\u2005weeks of diffuse oedema, increased abdominal girth and dyspnoea. His anasarca was thought to be indicative of an acute decompensation of his known liver cirrhosis with transfusion-induced haemosiderosis. While his anasarca improved with diuresis, his direct hyperbilirubinaemia suddenly worsened without any signs of haemolysis, biliary disease or obstruction. He also developed an acute worsening in serum creatinine (1.17\u20137.0\u2005mg\/dL in 7\u2005days) despite subsequent treatment for presumed hepatorenal syndrome (HRS). Given his clinical decline, the patient's goals of care were transitioned to comfort measures only. His clinical presentation and rapid liver and renal deterioration were most typical of sickle cell intrahepatic cholestasis (SCIC). SCIC can lead to rapid deterioration in renal function and can be mistaken for HRS. When SCIC is suspected, consideration of exchange transfusions should be made early.<\/jats:p>","DOI":"10.1136\/bcr-2015-210166","type":"journal-article","created":{"date-parts":[[2015,7,1]],"date-time":"2015-07-01T04:49:18Z","timestamp":1435726158000},"page":"bcr2015210166","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":8,"title":["Acute on chronic liver failure in a patient with sickle cell anaemia (HbSS)"],"prefix":"10.1136","volume":"2015","author":[{"given":"Dana DaEun","family":"Im","sequence":"first","affiliation":[{"name":"Harvard Medical School, Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Utibe","family":"Essien","sequence":"additional","affiliation":[{"name":"Massachusetts General Hospital, Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacqueline W","family":"DePasse","sequence":"additional","affiliation":[{"name":"Massachusetts General Hospital, Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Victor","family":"Chiappa","sequence":"additional","affiliation":[{"name":"Massachusetts General Hospital, Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2015,7,1]]},"reference":[{"key":"2025082801363725000_2015.jul01_1.bcr2015210166.1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2013-12-542076"},{"key":"2025082801363725000_2015.jul01_1.bcr2015210166.2","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9343(80)80008-8"},{"key":"2025082801363725000_2015.jul01_1.bcr2015210166.3","first-page":"331","article-title":"Hepatic lesions in sickle cell anemia","volume":"33","author":"Song","year":"1957","journal-title":"Am J Pathol"},{"key":"2025082801363725000_2015.jul01_1.bcr2015210166.4","doi-asserted-by":"publisher","DOI":"10.1053\/jhep.2001.24114"},{"key":"2025082801363725000_2015.jul01_1.bcr2015210166.5","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.290.6470.744"},{"key":"2025082801363725000_2015.jul01_1.bcr2015210166.6","doi-asserted-by":"publisher","DOI":"10.1097\/00007611-197705000-00008"},{"key":"2025082801363725000_2015.jul01_1.bcr2015210166.7","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.20317"},{"key":"2025082801363725000_2015.jul01_1.bcr2015210166.8","first-page":"2048","article-title":"Sickle cell intrahepatic cholestasis: approach to a difficult problem","volume":"90","author":"Shao","year":"1995","journal-title":"Am J Gastroenterol"},{"key":"2025082801363725000_2015.jul01_1.bcr2015210166.9","doi-asserted-by":"crossref","first-page":"1","DOI":"10.4081\/hr.2013.e1","article-title":"Successful orthotopic liver transplantation in an adult patient with sickle cell disease and review of the literature","volume":"5","author":"Blinder","year":"2013","journal-title":"Hematol Rep"},{"key":"2025082801363725000_2015.jul01_1.bcr2015210166.10","doi-asserted-by":"publisher","DOI":"10.1002\/lt.22257"},{"key":"2025082801363725000_2015.jul01_1.bcr2015210166.11","doi-asserted-by":"publisher","DOI":"10.1002\/lt.20999"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2015-210166","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,28]],"date-time":"2025-08-28T08:36:45Z","timestamp":1756370205000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2015-210166"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,7,1]]},"references-count":11,"alternative-id":["10.1136\/bcr-2015-210166"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2015-210166","relation":{},"ISSN":["1757-790X"],"issn-type":[{"value":"1757-790X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,7,1]]}}}