Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019045 (hemoglobinopathies)
 2,704 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Most pathologic studies of liver disease in sickle cell anemia and its variants were performed retrospectively on autopsy specimens, and, because of the prominent histologic features of intrasinusoidal sickling and Kupffer cell erythrophagocytosis, hepatic dysfunction was attributed to the intrahepatic sickling of erythrocytes in this hemoglobinopathy. We compared the liver histology from 19 patients who had liver biopsies to the autopsy specimens from 32 patients who succumbed to the complications of the hemoglobinopathy. In the former, nine patients had histological evidence of viral hepatitis. Four of these patients had both serological and immunohistochemical evidence of hepatitis B surface antigen. The features of biliary tree obstruction were found in two cases and alcoholic cirrhosis and sarcoid granuloma in one case each. Only one patient, who had recovered from septic shock, showed ischemic necrosis. In five patients incidentally biopsied during cholecystectomy, no significant lesions were found. Fourteen of the autopsy specimens showed ischemic necrosis, a result which was significantly different from the biopsy group. Ten cases had no significant morphologic changes other than heavy iron deposits. There were two cases with chronic active hepatitis, two with diffuse fibrosis, and one case each of cirrhosis, acute viral hepatitis, cholestasis, and giant cell hepatitis. Intrahepatic sickling and erythrophagocytosis were seen in almost all specimens and did not correlate with liver disease or transaminase elevation. Other than the patient with septic shock, ischemic necrosis was found only in postmortem material. These histological features may represent red cell destruction rather than the etiology of liver disease in these patients.
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PMID:Pathological spectrum of liver diseases in sickle cell disease. 394 29

The authors report two studies : 1) Retrospective : study of liver biopsies in 44 Africans : 22 were HBs Ag positive and 22 HBs Ag negative ; 10 of the HBs Ag positive subjects were found to have chronic persistent hepatitis (CPH), 4 chronic active hepatitis (CAH) and 3 cirrhosis ; 2 of the HBs Ag negative subjects were found to have CPH and 3 CAH ; as far a biological findings are concerned, only the CAH and cirrhosis groups differed from the control group, with higher prothrombin times, transaminase levels and gammaglobulin levels. 2) Prospective : study of the incidence of HBs Ag carriage in 168 Africans ; 17.8 % of them were found to be carriers ; no correlation was found between HBs Ag carriage and S. haematobium schistosomiasis or hemoglobinopathies.
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PMID:[Hepatopathies associated with chronic carriage of HBs antigen in black African immigrants in France]. 631 28