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)

Ninety patients with thalassaemia major were investigated for the occurrence of antinuclear antibodies (ANA), and those with ANA were tested for antibodies to histones (AHA). ANA were detected in 7 of 27 thalassemics on oral iron chelator L1, and in 2 of 63 thalassaemics not on L1 (p < 0.01). AHA were seen in 4 of 7 thalassemics receiving L1 with positive ANA, and in none of the 2 not receiving L1 (p < 0.03). Joint pains were seen in patients receiving L1, but in none of the patients not receiving L1. There was no correlation between hepatitis B or HIV positivity and presence of ANA or joint pains. While some amount of background ANA-positivity was found in patients with thalassaemia major, it was significantly more in patients receiving L1. Laboratory evidence of drug-induced lupus-like reaction was seen only in patients who received L1. In view of serious concerns about the safety of L1 and wide variations in the incidence and severity of adverse reactions reported by different sources, an urgent regulatory audit of all trial centres is essential.
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PMID:Autoantibodies in thalassaemia major: relationship with oral iron chelator L1. 786 14

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

In Brazil, clinicians followed 32 transfusion-dependent beta-thalassemia patients, 1-49 years old, at the Regional Blood Center and the Department of Hematology of University Hospital of the School of Medicine of Ribeirao Preto to determine the prevalence of hepatitis B virus (HBV), hepatitis C virus (HCV), HIV-1, and HTLV-1. They also measured serum levels of ferritin and alanine aspartate transaminase (ALAT) to examine liver iron content and liver damage, respectively. 46.8% tested positive for antibodies to HCV, which was much higher than that of voluntary blood donors of the Regional Blood Center (1.4%) or of other countries. Yet it was about the same as that of multitransfused patients in the UK (23.2%), Italy (92.9%), and Saudi Arabia (33.3%). 3 of these 15 patients also tested positive for HBV markers. 15.5% tested positive only for HBV markers. 37.5% had no hepatitis markers. Hepatitis-positive people were older than those who tested negative for hepatitis (15.2 years vs. 8.5 years; p .05). The number of units of blood transfused and the levels of ferritin and ALAT were not statistically different between the 2 groups (192.1-336 vs. 135.2 and 36.6-52.3 U/l vs. 36.7 U/l, respectively). 75% of the HCV positive patients received more than 100 units of packed red blood cells while only 42% did in the HCV negative group. 2 people tested positive for HIV-1 1 of whom also tested positive for anti-HBs-Ag and the other for HCV antibodies. The HIV-1 cases had become infected before the blood bank began screening for HIV-1 in 1987. None of the patients receiving blood from the center became infected with HIV-1, yet 60% of hemophiliacs treated at the hospital were HIV-1 infected. No one tested positive for HTLV-1, even though all 32 patients had received more than 6250 units of blood not screened for HTLV-1. This reflected the low incidence of HTLV-1 in the general population (0.05%). No one was positive for HBs-Ag or HBe-Ag.
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PMID:The frequency of blood-born viral infections in a population of multitransfused Brazilian patients. 827 57

Molecular biology techniques are now a vital part of hepatitis virology, with a central role in studies of diagnosis, epidemiology, virology, pathogenesis, and natural history of infection. Cloning of the genome of hepatitis E virus has allowed its tentative classification as a calici- or related virus, and is the first step toward the development of a vaccine. Long-term implications of hepatitis C for groups such as children with hemophilia, thalassemia, and even leukemia can be better understood by comparison of virus load measured by molecular amplification of the plasma viral RNA with the serologic and clinical status of the respective cohorts of children. A new vaccine for hepatitis A has been licensed in several European countries, and recent experience with severe hepatitis in infants after unexpected transmission of hepatitis B from anti-hepatitis B e positive mothers reemphasizes the value of universal hepatitis B immunization programs. Mother-to-infant transmission of hepatitis C virus has now been well documented, but there are still insufficient data on the dynamics of this, particularly in the absence of passive immunoprophylaxis or a vaccine, to permit recommendations regarding the management of individual pregnancies or deliveries. There is especially too little information to suggest whether breast feeding may be an important mechanism for transmission.
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PMID:Hepatitis viruses and protection against infection in children. 837 24

A total of 74 multitransfused (MT) children of beta thalassaemia major were analysed for prevalent viral markers transmitted through transfusion. A higher incidence of serological markers for Hepatitis B virus (HBV), cytomegalovirus (CMV) could be observed in the group of MT children compared to control group. There was a significant trend (chi 2 = 33.4; P < 0.001) in the increase in prevalence of viral markers along with the increase in the number of transfusions. MT children receiving more than 50 transfusions were found to have evidence for at least one or multiple viral infections transmitted through blood. Children receiving more than 50 transfusions were characterized by marked alteration of T3, T8, and B cells while T4/T8 ratio was found to be significantly decreased (P < 0.001) only in the group of children receiving more than 100 transfusions. Relative assessment of the alteration of lymphocyte subsets in various groups of viral infection showed that cases with CMV IgM to have more marked influence on the alteration of T8 cells, T4/T8 ratio, and B cells compared to other groups of viral infections. Reassessment of the lymphocyte subset profile in MT children in the light of CMV IgM positive cases revealed that in children receiving more than 50 transfusions significant alterations of lymphocyte subjects were influenced by the presence of CMV IgM positive cases in these groups. Our study points out that the correlation between the alteration of lymphocyte subset profile and number of transfusion in MT children need to be reassessed in the light of acute CMV infection in the form of CMV IgM.
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PMID:Role of transfusion-mediated viral infections on the lymphocyte subset profile in multi-transfused children. 841 20

Some 20% of cases of posttransfusion and sporadic hepatitis non-A, non-B are anti-HCV negative. In 1995 it proved possible in collaboration of Genelabs with Boehringer Co. Mannheim to identify a new RNA virus which causes acute and chronic hepatitis in humans and tamarins. The genome of the virus contains some 2900 amino acids, and as to its structure, it resembles flaviviruses. It was described as hepatitis G virus (HGV). It differs from the hepatitis C, virus as it has only a 26% homology of amino acids. It is transmitted through blood during transfusion along with other parenteral routes of infection. Risk groups comprise i.v. drug addicts, blood donors and patients with thalassaemia and repeated blood transfusions. HGV can infect the liver as an independent virus or along with the virus of hepatitis B or C (dual infection). As to clinical aspects, hepatitis G is very mild and not associated with jaundice. Some patients develop chronic hepatitis. About half the patients infected with HGV have only a slightly raised transaminase activity, the remainder have normal liver enzymes. As compared with hepatitis C, the mean transaminase activity is one half. It can be diagnosed by assessment of HGV RNA by means of PCR. In the USA the prevalence of HGV RNA in blood donors with normal ALT activity is 1.7% and in donors with increased ALT activity 1.5%. The virus is sensitive to interferon, after treatment the serum concentration of HGV RNA declines rapidly but after withdrawal of treatment the values return to pre-treatment levels. This is the first report on the newly discovered hepatitis G virus.
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PMID:[The discovery of hepatitis G virus]. 862 84

Conventional treatment of beta thalassaemia major is based on regular blood transfusion from early childhood. Maximum effectiveness of transfusion therapy depends on the following. (1) Availability of safe blood. Donation programmes should aim at retaining repeat donors, who carry decreased risk of transmitting blood-borne infections. Donors should be screened with laboratory tests performed to the highest possible standard of quality. Selection of safe donors can be improved by the adoption of questionnaires containing direct questions on risk factors for transfusion transmissible infections. (2) Use of good quality red blood cells, which should be leucodepleted, preferably by filtration, that can be carried out at the bedside. (3) Regular evaluation of blood transfusion indices, including mean level of haemoglobin maintained, annual blood requirement, daily haemoglobin fall, mean transfusion interval, transfusion reaction rate. This can be assisted by the use of a computerized patient record. (4) Maintenance of a permanent record of the patient's blood group genotype (including at least Rh, Kell, Kidd and Duffy systems) and any red cell antibodies that develop. This is mandatory to ensure optimal survival of transfused red cells. (5) Continuous monitoring of transfusion transmissible infections. (6) Vaccination against hepatitis B of all suitable patients. (7) Intensive iron chelation. This should be done by regular subcutaneous administration of desferrioxamine B. Oral chelators, which are currently under laboratory and clinical evaluation, are not yet available for general use.
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PMID:Blood transfusion in beta thalassaemia major. 864 87

Exposure to hepatitis C virus (HCV) and its effect on ALT levels was studied in 35 transfusion dependent cases of thalassaemia major. Twenty-one (60%) cases were anti HCV positive and also showed raised Alanine Transaminase (ALT) levels. Of 14 anti HCV negative, Hepatitis B Surface Antigen (HBs Ag) negative seven showed raised ALT levels, indicating the chances of acute viraemia. Thus there is an urgent need to start anti HCV screening on all blood donations.
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PMID:Prevalence of antibody to hepatitis C virus in Pakistani thalassaemics by particle agglutination test utilizing C 200 and C 22-3 viral antigen coated particles. 871 23

Long term effects of BMT in thalassemia were monitored in 33 patients transplanted between 1987 and 1995 and compared with 155 patients matched for age and treated during the same period with conventional therapy (CT). The incidence of fulminant sepsis and growth impairment was significantly higher in transplanted patients, whereas the occurrence of hypothyroidism, hypogonadism, and cardiopathy was higher in CT patients. For diabetes, liver disease, and severe infections, the differences were not statistically significant. After BMT we performed monthly erythrocytaferesis for iron removal in 23 (70%) patients, obtaining a complete normalization of iron stores in 91% of cases; among untreated patients, 60% had evidence of iron up to 8.3 years after BMT. Protection against poliovirus, tetanus, diphtheria, and hepatitis B has been lost in 74%, 47%, 78%, and 44%, respectively. After BMT a careful follow-up is needed to monitor and treat late transplant-related and thalassemia-related complications.
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PMID:Late effects of bone marrow transplantation for thalassemia. 966 51


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