{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,17]],"date-time":"2026-07-17T18:47:58Z","timestamp":1784314078361,"version":"3.55.0"},"reference-count":19,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2007,6,12]],"date-time":"2007-06-12T00:00:00Z","timestamp":1181606400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2007,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>A 38\u2010year\u2010old Ghanaian man presented with a 6\u2010month history of worsening pruritus, jaundice, and ascites. He was previously fit and well and rarely drank alcohol. Screening tests for chronic liver disease including viral, autoimmune, and other metabolic causes including iron overload were unremarkable. A liver biopsy performed at the referring hospital demonstrated intralobular cholestasis and cirrhosis. He was listed for liver transplantation but subsequently developed sepsis with multiple organ failure and died. The sickle solubility test was positive. Blood smear showed cells consistent with liver failure and no sickle cells. Hemoglobin electrophoresis revealed HbA2 2.8%, HbF 0.5%, and HbS greater than HbA (49.6% vs. 41.3%) in the absence of blood transfusion. Sequence analysis of the \u03b2IVS2\u2010844 C \u2192 A). A diagnosis of sickle hepatopathy causing decompensated cirrhosis was made. This case is unusual insomuch as this patient was asymptomatic for over 35 years and represents a novel presentation of sickle cell disease. Sickle cell disease should be considered in appropriate patients when unusual presentations of liver disease arise. Am. J. Hematol., 2007. \u00a9 2007 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajh.20971","type":"journal-article","created":{"date-parts":[[2007,6,12]],"date-time":"2007-06-12T16:47:24Z","timestamp":1181666844000},"page":"852-854","source":"Crossref","is-referenced-by-count":10,"title":["Sickle liver disease\u2014An unusual presentation in a compound heterozygote for HbS and a novel \u03b2\u2010thalassemia mutation"],"prefix":"10.1002","volume":"82","author":[{"given":"Timothy J.S.","family":"Cross","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philip A.","family":"Berry","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nuzhat","family":"Akbar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julia","family":"Wendon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.L.","family":"Thein","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Phillip M.","family":"Harrison","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2007,8,6]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(97)07330-3"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1001\/jama.232.7.738"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.3109\/00365519309092574"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9343(80)80008-8"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.20685"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1053\/jhep.2001.24114"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/0016-5085(86)90276-3"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.290.6470.744"},{"key":"e_1_2_1_10_2","first-page":"169","article-title":"Clinical features of hepatic sequestration in sickle cell anaemia","volume":"22","author":"Hernandez P","year":"1989","journal-title":"Haematologia (Budap)"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF02029357"},{"key":"e_1_2_1_12_2","first-page":"49","article-title":"Plasma exchange for acute cholestasis in homozygous sickle cell disease","volume":"19","author":"Svarch E","year":"1986","journal-title":"Haematologia (Budap)"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1097\/00000441-199606000-00012"},{"key":"e_1_2_1_14_2","first-page":"2048","article-title":"Sickle cell intrahepatic cholestasis: Approach to a difficult problem","volume":"90","author":"Shao SH","year":"1995","journal-title":"Am J Gastroenterol"},{"key":"e_1_2_1_15_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(77)80436-8"},{"key":"e_1_2_1_16_2","doi-asserted-by":"publisher","DOI":"10.1136\/gut.37.1.144"},{"key":"e_1_2_1_17_2","doi-asserted-by":"publisher","DOI":"10.1097\/00043426-200110000-00011"},{"key":"e_1_2_1_18_2","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.295.6592.234"},{"key":"e_1_2_1_19_2","first-page":"513","article-title":"Phenotypes of individuals with a \u03b2 thal classical allele associated either with a \u03b2 thal silent allele or with \u03b1 globin gene triplication","volume":"82","author":"Bianco I","year":"1997","journal-title":"Haematologica"},{"key":"e_1_2_1_20_2","doi-asserted-by":"publisher","DOI":"10.1097\/00043426-200303000-00015"}],"container-title":["American Journal of Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fajh.20971","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajh.20971","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,11,14]],"date-time":"2023-11-14T17:59:23Z","timestamp":1699984763000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajh.20971"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2007,8,6]]},"references-count":19,"journal-issue":{"issue":"9","published-print":{"date-parts":[[2007,9]]}},"alternative-id":["10.1002\/ajh.20971"],"URL":"https:\/\/doi.org\/10.1002\/ajh.20971","archive":["Portico"],"relation":{},"ISSN":["0361-8609","1096-8652"],"issn-type":[{"value":"0361-8609","type":"print"},{"value":"1096-8652","type":"electronic"}],"subject":[],"published":{"date-parts":[[2007,8,6]]}}}