Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0039730 (thalassemia)
 10,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The systematic screening of 253 children with transfusion-dependent homozygous beta-thalassaemia revealed a high incidence of hepatitis B virus markers. The highest frequencies of hepatitis B surface antigen (HBsAg) and antibody to hepatitis B core antigen (anti-HBc) were found in the group of patients with the smallest number of transfusions, while the highest frequency of antibody to hepatitis B surface antigen (anti-HBs) was detected in the patients who had had the largest number of transfusions. Follow-up of these patients showed (a) a high incidence of acute hepatitis B, which was mainly subclinical; (b) normal hepatitis B surface antigen clearance and normal antibody to hepatitis B surface development; and (c) a high frequency of increased transaminase values for over six months. In all the subjects with persistently high transaminase, histological examination revealed chronic persistent hepatitis or chronic active hepatitis. Apart from two cases of chronic active hepatitis with no B virus markers, and two cases of chronic persistent hepatitis with HBsAg and anti-HBc in the serum, all these subjects were anti-HBs positive but HGsAg and anti-HBc negative.
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PMID:Chronic liver disease in transfusion-dependent thalassaemia: hepatitis B virus marker studies. 743 Mar 60

Forty-eight household contacts of 25 children with homozygous beta-thalassaemia and chronic hepatitis C (index cases) were evaluated for antibodies against hepatitis C virus (HCV) and increased transaminase values in the blood. The mean age +/- SD of the household contacts was 36.4 +/- 17.0 years (range 5-67) and 20 of them were males. All thalassaemic patients (age 14.3 +/- 3.0 years, range 8-19) were positive for anti-HCV antibodies by repeated determinations. HCV-RNA was detected in the blood of 22 of 23 patients tested by polymerase chain reaction. Liver biopsies were performed in 18 patients and showed chronic active hepatitis in 14 and chronic persistent hepatitis in 4. The mean duration of contact between the index cases and the household contacts while the index cases were anti-HCV positive was 45.3 +/- 10.2 months (range 17-57). None of the household contacts was found to be positive for anti-HCV antibodies nor did they have elevated transaminases in the two examinations performed within an interval of about 2 years. Among the HCV-negative household contacts are included 14 who mentioned needlestick injuries with needles used by the index cases.
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PMID:Lack of transmission of hepatitis C in household contacts of children with homozygous beta-thalassaemia. 906 12

No experience has been reported to date in treating chronic hepatitis C virus (HCV) infection with interferon (IFN) therapy after BMT, mainly due to concerns related to the impact of an immunomodulatory drug in patients who are immunologic and haematologic chimeras. However, chronic inflammatory activity related to HCV infection results in a chronic fibrogenous mechanism potentially leading to liver cirrhosis and hepatocellular carcinoma. Moreover, patients transplanted for beta-thalassemia could be at greater risk because of concomitant iron overload and pre-existing fibrous liver damage. Eleven patients with serological, biochemical, histological and molecular biological evidence of HCV infection were included in the study and treated for 6-12 months with recombinant IFN 24-65 months following BMT. The serum alanine aminotransferase (ALT) was persistently elevated (range 85-1242 U/l; mean 416) for at least 1 year prior to IFN treatment. Ten patients completed the protocol; five were considered as responders to treatment. In these five patients the liver histology showed an overall reduction of inflammation and necrosis: histological inflammatory activity improved from chronic active hepatitis (CAH) to chronic persistent hepatitis (three patients) or minimal residual inflammatory activity (two patients). The Knodell total activity score varied from 5.4 (range 3-9) to 1.4 (range 1-2; P = 0.05). All responding patients revealed negativization of serum HCV-RNA, that has been persistent in four (follow-up 1-3 years). ALT level fell to 15-80 U/l (mean 52; P = 0.0027). No major complications occurred during the therapy and no influence on marrow engraftment parameters were noted. We conclude that IFN therapy does not adversely interfere with engraftment and that it is a feasible therapy for treatment of chronic hepatitis C virus after BMT.
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PMID:Alpha-interferon treatment of chronic hepatitis C after bone marrow transplantation for homozygous beta-thalassemia. 938 79