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 prevalence of markers for human immunodeficiency virus types 1 and 2 (HIV-1, HIV-2), human T-lymphotropic virus type I (HTLV-I), hepatitis B virus (HBV) and hepatitis C virus (HCV), and cytomegalovirus (CMV) was evaluated in a population of 305 multiply transfused thalassemia patients in Belgium, France, and Italy (Sicily). No patients were found positive for HIV-2 antibodies. Two French patients were seropositive for HIV-1, having been infected before systematic blood screening. Antibodies to HTLV-I were found in two Sicilian patients. A positive anti-HCV enzyme-linked immunosorbent assay was found in one-third of the patients and a positive CMV IgG test in two-thirds. Twenty-two percent of the patients in the three countries were uninfected by HBV and were not vaccinated. With the exception of HIV-1, HIV-2, HTLV-I, and anti-hepatitis B surface antigen assays, all markers were encountered more frequently in Sicilian patients than in French or Belgian patients. This study emphasizes the need to improve HBV vaccination coverage in the three countries. At present, data indicate that the introduction of routine screening for HTLV-I should be considered, particularly in Sicily.
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PMID:Prevalence of markers for human immunodeficiency virus types 1 and 2, human T-lymphotropic virus type I, cytomegalovirus, and hepatitis B and C virus in multiply transfused thalassemia patients. The French Study Group On Thalassaemia. 132 85

Non-A, non-B hepatitis, recently renamed as hepatitis C virus (HCV), accounts for over 90% of hepatitis cases worldwide associated with blood transfusions. Application of a recombinant-based enzyme immunoassay for the detection of antibodies to HCV to a sample of 500 male Saudi blood donors and 260 healthy Saudi pregnant women indicated that HVC is endemic in the Saudi population. Anti-HCV was detected in 28 (5.6%) of the blood donors and 12 (4.6%) of the pregnant women, for an overall frequency of 5.3% in healthy Saudi adults who had never received blood transfusions. This rate is at least 5 times higher than that reported for the US and Western Europe. Also assessed was the HCV rate in subsamples of Saudis considered at risk of this infection. Here, anti-HCV was detected in 22 (78.6%) hemophiliacs, 26 (33.3%) patients with thalassemia and sickle cell disease, 17 (26.1%) hemodialysis patients with renal failure, and 35 (15.9%) individuals with a sexually transmitted disease. The prevalence of anti-HBc ranged from 28% in blood donors to 46% in hemophiliacs. The significantly higher prevalence of HCV in patients with sexually transmitted diseases than in blood donors suggests that this disease is transmitted through heterosexual contact as well as blood transfusions. Given the high baseline level of HCV infection in the Saudi population and the possibility of serious sequelae (e.g., chronic active hepatitis, cirrhosis, and hepatocellular carcinoma), routine anti-HCV screening of blood donations is urged.
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PMID:Hepatitis C virus antibodies in high-risk Saudi groups. 177 46

Seventeen of 73 (23.3%) multiply transfused patients with thalassaemia major (age range, 1-39 years) tested positive for antibody to hepatitis C virus (anti-HCV). Eleven of the 24 patients regularly transfused in countries outside Britain were anti-HCV seropositive; only six of the 49 regularly transfused in Britain were seropositive. The incidence of anti-HBs and anti-HBc was similar to that of anti-HCV in both the British and foreign patients. The anti-HCV seropositive patients showed significantly higher plasma aspartate aminotransferase activities (AST), mean (SD) 10.2 (70.3) U/l, and serum ferritin concentrations, 4067 (2708) micrograms/l, than the anti-HCV seronegative patients (AST, 33.9 (15.6) U/l; serum ferritin 2051 (2092) U/l), respectively. Among the 36 patients who had earlier undergone liver biopsy 10 of 21 with histological features of chronic active hepatitis or cirrhosis, or both, were seropositive for anti-HCV whereas only one of 15 without histological evidence of chronic viral hepatitis was seropositive for anti-HCV. It is concluded that HCV is a major cause of chronic hepatitis in patients with thalassaemia major and is associated with raised AST activity and serum ferritin concentration compared with patients seronegative for anti-HCV.
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PMID:Antibody to hepatitis C virus in multiply transfused patients with thalassaemia major. 211 95

To determine the prevalence of hepatitis B virus (HBV), hepatitis C virus (HCV) and human immunodeficiency virus (HIV) markers among Bahraini children with hereditary haemolytic anaemias, a cross-sectional study was conducted at the paediatric outpatient clinic of Sulimaniya Medical Center in the State of Bahrain. A total of 242 patients with hereditary haemolytic anaemias were enrolled in the study: 171 (71%) with sickle cell syndromes, 59 (24%) with beta thalassaemia major and 12 (5%) with alpha thalassaemia. Among the 191 multi-transfused patients, 39 (20.5%) had one or more markers for HBV, 78 (40%) were seropositive for HCV antibody, and three (1.6%) were seropositive for HIV antibody. In contrast, none of the 51 non-transfusion group was seropositive for HBV and HIV antibodies but one patient was seropositive for HCV antibody. HBV, HCV and HIV infections therefore remain a major hazard for children with hereditary haemolytic anaemias, despite blood donor screening. More refined and sensitive tests which would detect infection in all stages of the disease are required. Hepatitis B vaccine should be given to all children with hereditary haemolytic anaemias.
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PMID:Prevalence of hepatitis B, hepatitis C and human immune deficiency virus markers among patients with hereditary haemolytic anaemias. 767 12

Thirty-four of 99 multiply transfused Chinese (49 females, 50 males) with thalassaemia major were positive for antibody to hepatitis C virus. There was no sex predominance in seropositivity with 18 females and 16 males positive. The mean (+/- SD) age and units of blood transfused were significantly higher in the seropositive patients (167 +/- 48 months, 206 +/- 82 units respectively) than the seronegative patients (113 +/- 56 months, 124 +/- 80 units respectively). The seropositive patients had higher mean (+/- SD) serum alanine aminotransferase, aspartate aminotransferase and ferritin concentrations (91 +/- 82 IU/L, 67 +/- 38 IU/L, 4797 +/- 2522 ng/ml respectively) than the seronegative patients (38 +/- 29 IU/L, 48 +/- 28 IU/L, 3620 +/- 2140 ng/ml respectively). Serum ferritin had an independent and significant effect on serum alanine aminotransferase in addition to that of seropositivity to hepatitis C virus.
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PMID:Hepatitis C virus antibody in multiply transfused Chinese with thalassaemia major. 769 Jun 34

There have been many advances in supportive treatment used for beta-thalassemia major. Survival has increased substantially, and an increasing number of patients reach adolescence and adulthood. These older patients present new clinical challenges. Complications of transfusion, most commonly hepatitis C, are still a cause of mortality and morbidity. The achievement of optimal growth and development, including fertility, is an important goal of conservative management. Long-term survival has also been achieved with bone marrow transplantation. Assessment of growth, development and iron balance in the years after transplantation reveals residual problems requiring treatment despite cure of thalassemia. New therapies of beta-thalassemia are still being developed, both supportive and curative in nature. Supportive care improvements include oral chelation and methods to increase HbF production. Advances in curative modalities include use of new sources of stem cells, such as cord blood and fetal liver. In the future, gene therapy may allow for cure of the older patient without the mortality and morbidity of allogenic transplantation. Treatment of thalassemia major requires consideration of the available therapeutic options for each patient, and the risk/benefit ratio of a supportive versus curative approach.
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PMID:Thalassemia major 1995: older patients, new therapies. 779 22

The purpose of this study was to determine whether interferon-alfa (IFN-alpha) therapy benefits patients with transfusion-dependent thalassemia and chronic active hepatitis C, and whether their iron burden modifies the response to this therapy. We conducted a controlled trial of recombinant IFN-alpha (3 million units per square meter of body surface area, three times a week for 15 months) in 65 patients with thalassaemia major and chronic active hepatitis C; 14 of them were untreated control subjects. In 21 of the 51 treated patients, alanine aminotransferase values returned to normal within 6 months, and hepatitis C virus ribonucleic acid was no longer detected in serum; no changes were detected among control subjects. The response to IFN-alpha therapy was inversely related (p < 0.002) to the liver iron burden as assessed by atomic absorption, the histologic semiquantitative method, or both methods. During 3 years of follow-up, two responder patients had relapses. We conclude that IFN-alpha represents a useful therapeutic option for children with transfusion-dependent thalassemia and chronic active hepatitis C with a mild to moderate iron burden.
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PMID:Effect of iron overload on the response to recombinant interferon-alfa treatment in transfusion-dependent patients with thalassemia major and chronic hepatitis C. 802 61

The pathogenesis of posttransfusion hepatitis was determined in 14 children with beta-thalassemia. All had blood samples obtained in 1980 or 1981, were vaccinated against hepatitis B virus in 1983 and had another serum sample collected in 1989. Seven children had detectable antibodies against hepatitis C virus before vaccination, and all were positive in 1989. With specific solid-phase enzyme immunoassays, all children had antibodies against hepatitis B virus, X and polymerase antigens in 1981, and six had one or both antibodies in 1989. Hepatitis B virus infection was confirmed by means of polymerase chain reaction, which demonstrated virus DNA in 13 of the 14 children. The amplification products spanning the X/precore region were smaller than expected, suggesting mutations in this region. Cloning and sequencing of these products revealed deletions spanning part or all of the X gene. The results show that these children were infected with hepatitis B virus even without other markers in serum, that hepatitis B persists years after vaccination and that such infections are associated with the presence of X deletion mutants. Coinfection with hepatitis B and C viruses, the former containing a new class of variants, is common in children with beta-thalassemia.
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PMID:Pathogenesis of posttransfusion viral hepatitis in children with beta-thalassemia. 811 79

Seventy five Saudi children, 55 with sickle cell anaemia and 20 with beta thalassaemia major, who were negative for all hepatitis B virus (HBV) markers five years ago were recently investigated for exposure to HBV and hepatitis C virus (HCV) infection. Of the 55 patients with sickle cell anaemia and 20 with beta thalassaemia major, 20 and five patients respectively had been vaccinated against HBV earlier and all of them still had protective antibody (anti-HBs 42-96 IU) 3-5 years after vaccination and there was no vaccine failure. Among the non-vaccinated children the exposure rates to HBV were 14.3% among those with sickle cell anaemia and 26.7% among those with beta thalassaemia and this was not statistically significant when compared with the exposure rate to HBV among the general paediatric population (20.1%). Anti-HCV positivity among those with beta thalassaemia major and sickle cell anaemia was 70% and 18.2%, respectively, and this was significantly higher than anti-HCV positivity among the control group (0.8%). Anti-HCV positivity was directly related to the amount of blood transfused and to the duration of transfusion. The results of the study show that although the exposure rates to HBV among patients with sickle cell anaemia and beta thalassaemia major were not significantly different than that among the general paediatric population, infection with HBV still takes place among non-vaccinated patients despite strict precautionary measures taken. Hence early vaccination against HBV would probably be the only effective way of controlling HBV infection. For HCV infection, and because a vaccine against HCV is still not available, preventive measures such as blood screening for anti-HCV before transfusion and stringent infection control measures are crucial steps to be implemented for the control of spread of HCV among these groups of patients.
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PMID:Decline in hepatitis B infection in sickle cell anaemia and beta thalassaemia major. 825 83

A specific enzyme immunoassay (EIA) for the diagnosis of hepatitis C virus (HCV) infection was developed by recombinant DNA technology. Abbott HCV EIA was used to detect antibody to HCV (anti-HCV) in non-transfused and multiply-transfused thalassemia patients. None of 11 non-transfused patients had anti-HCV but 3 of 52 (5.8%) multiply-transfused patients had anti-HCV. This study showed that the prevalence rate of HCV infection is low in thalassemia patients. However, it is still important to identify hepatitis C virus infected patients in high risk groups because hepatitis C is associated with chronic hepatitis, cirrhosis and hepatocellular carcinoma.
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PMID:Antibody to hepatitis C virus in thalassemia patients. 827 96


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