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

For 9 months, 38 transfusion-dependent patients with beta-thalassemia, ranging in age from 3.4 to 19.1 years, were observed for serologic evidence of viral infections, by the collection of serial serum samples. Seventy-six age-matched healthy subjects, two for each patient, were followed as controls. Samples taken at the beginning, middle, and end of the study were tested against 18 viral antigens by complement fixation (CF). In addition, tests for antibodies to HIV, Epstein-Barr virus, hepatitis A virus, and markers for hepatitis B virus were performed. When changes in the antibody titer on CF tests (greater than or equal to 2-fold increase or decrease) or persistently high titers (greater than or equal to 64) were revealed, specific enzyme immunoassays (EIAs) for IgM and IgA antibodies were performed concomitant with CF tests in all sera. When symptomatic infections occurred, viral cultures and/or direct detection of antigens were carried out by immunofluorescence methods, EIA, or latex agglutination tests. Thalassemic patients and controls had similar (p greater than 0.05) overall rates of serologically confirmed viral infections (53 versus 132), but the former group had a higher (p less than 0.01) incidence of cytomegalovirus (CMV) infections (9 versus 4). CMV infections were associated in the thalassemic patients with hepatitis (2 cases), lymphadenitis (2 cases), and upper respiratory tract infection (1 case), while the remaining cases of CMV had a subclinical course. Moreover, the thalassemic patients had a lower (p less than 0.01) incidence of symptomatic infections (27 versus 110) than controls. Therefore, this study showed that both symptomatic and subclinical CMV infections may occur often in thalassemic patients, who otherwise have subclinical viral infections at an overall rate similar to that in healthy subjects.
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PMID:Viral infections in transfusion-dependent patients with beta-thalassemia major: the predominant role of cytomegalovirus. 217 79

Pure fetal blood samples, obtained fetoscopically from 30 patients with unexplained fetal hydrops at 16 to 32 weeks gestation were investigated for cytogenetic, haematological, biochemical and virological properties. In two patients with oligohydramnios, the fetoscope was introduced transabdominally into the fetal peritoneal cavity and sampling was undertaken from the intra-abdominal portion of the umbilical vein; in all the other patients an umbilical cord vessel was sampled. Ten (33%) of the fetuses had chromosomal abnormalities, one an erythroblastic process, possibly erythroleukaemia, one alpha-thalassaemia and one cytomegalovirus infection. Blood-film abnormalities were seen in 23 (88%) of 26 fetuses that had this examination. Biochemical analysis of fetal plasma was undertaken in 18 fetuses and hypoproteinaemia was found in all cases. One fetus was subsequently found to have a paroxysmal tachyarrhythmia that responded to digitilization. Three (10%) of the fetuses survived.
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PMID:Fetoscopy in the assessment of unexplained fetal hydrops. 241 12

The prevalence of total as well as IgM antibodies against cytomegalovirus (CMV) was determined by enzyme immunoassay in a group of 287 multitransfused thalassemia major patients aged 5-39 years and in another group of 1,220 healthy controls. A significantly higher prevalence of CMV antibodies was observed in thalassemic patients of all age-groups compared with controls. The prevalence among splenectomized thalassemia patients was higher than among nonsplenectomized thalassemics. It is concluded concluded that patients with thalassemia, especially if splenectomized, are at high risk for transfusion-transmitted CMV infections. The high prevalence of CMV infections might be responsible, at least in part, for the immunological disturbances and the susceptibility to other infections observed in thalassemic patients. On the basis of these results, it is suggested that safe blood should be provided for anti-CMV-negative thalassemics, with priority to anti-HIV-positives and those who are to receive bone marrow transplantation.
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PMID:Thalassemic patients are at high risk for transfusion-transmitted cytomegalovirus infections. 255 29

Fourteen thalassemia patients (aged 1.6-13.5 years; median age of 6 years) underwent allogeneic bone marrow transplantation (BMT) between March 1984 and May 1987. The preconditioning regimens consisted of oral busulfan, intravenous cyclophosphamide, with or without irradiation. Two of the patients, who received maternal transplants, failed to engraft but experienced autologous recovery. Of the 12 patients who received sibling marrow, two experienced autologous recovery and one developed marrow chimerism. Five patients died of complications of bone marrow transplantation: two died of intracranial hemorrhage, two died of sepsis, and one succumbed to acute graft-versus-host disease associated with cytomegalovirus infection. Six patients engrafted and have been followed for 1-4 years (median of 2.6 years) without intoward events. The overall survival rate was 64% (nine out of 14) with follow-up of 1-4 years. These results demonstrate that bone marrow transplantation can cure thalassemia but infection, graft-versus-host disease, and hemorrhage were major causes of morbidity and mortality in this group of patients. Other factors of importance include the unfavorable influence of engraftment of prior multiple transfusions and sex-mismatched transplantation. In patients who fail to engraft, autologous recovery usually occurs within 2 months of transplantation.
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PMID:Allogeneic bone marrow transplantation for thalassemia in Taiwan: factors associated with graft failure. 261 76

The major barriers to successful bone marrow transplantation (BMT) are graft-versus-host disease (GVHD), infection, rejection and relapse. The combination of methotrexate and cyclosporin is significantly better than either alone in controlling GVHD. Removal of T cells from donor marrow prior to BMT has also decreased GVHD significantly, but a 5-10% rejection rate occurs and an increased relapse risk is being reported by some centres. Cyclosporin is valuable in the treatment of both acute and chronic GVHD. Interstitial pneumonitis due to cytomegalovirus (CMV) is a major cause of mortality. Protection can be provided with CMV hyperimmune globulin and also by the avoidance of blood donors who are CMV antibody positive. Fractionated total body irradiation is associated with decreased toxicity compared to single dose. There is a 75% 4 year disease-free survival following BMT for acute non-lymphoblastic leukemia in first remission, a 50% survival for acute lymphoblastic leukemia in second remission and an 88% survival for chronic myeloid leukemia in chronic phase. BMT for beta-thalassaemia major in young patients without organ dysfunction cures 80% of patients and identical results are achieved for severe aplastic anaemia when BMT is undertaken prior to blood product transfusion.
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PMID:Recent advances in bone marrow transplantation. 332 11

Cytomegalovirus (CMV) pneumonia frequently occurs in immunocompromised hosts. Unlike encapsulated bacteria and Mycoplasma, CMV pneumonia has not been reported in sickle cell disease. We describe a case of a healthy young man with sickle cell thalassemia who died with CMV pneumonia.
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PMID:Cytomegalovirus pneumonia in sickle cell disease. 608 44

Autoimmune hemolytic anemia may occur in the course of some viral diseases such as Coxsackie virus, cytomegalovirus, Epstein Barr virus, Influenza A, herpes simplex virus, and rarely hepatitis B virus infection. The role of being heterozygous for beta-thalassemia in hemolysis during acute viral hepatitis is not known. In this report, we present an eight-year-old boy with jaundice and anemia. The diagnosis of hepatitis B virus infection and hemolytic anemia were made on the basis of physical and laboratory findings. A hemoglobin electrophoresis revealed that the child was heterozygous for beta-thalassemia. No specific etiology could be found for hemolytic anemia. It remained unclear whether hemolytic anemia in this patient was merely a coincidental finding or whether hepatitis B virus infection and beta-thalassemia trait had played a role in causing hemolysis.
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PMID:Acute hemolysis in association with hepatitis B infection in a child with beta-thalassemia trait. 797 18

Translational stop mutations of the human beta-globin gene cause a reduction of cytoplasmic mRNA accumulation in thalassemia patients and in transfection models. The exact mechanism underlying this phenomenon has remained enigmatic but is known to be post-transcriptional. We have used transfected HeLa cells to study the expression of beta-globin mRNAs with nonsense or frameshift mutations within the three exons of this gene. Mutations in exons 1 or 2 reduce cytoplasmic mRNA accumulation whereas a mutation in exon 3 permits essentially normal expression. We report here that the post-transcriptional fate of mutated beta-globin mRNAs is differentially affected by the type of RNA polymerase II promoter driving expression. Replacement of the beta-globin promoter with the herpes simplex virus type 1 thymidine kinase gene promoter but not the cytomegalovirus immediate early promoter rescues the cytoplasmic accumulation of mutated mRNA to wild-type levels. This effect is shown to be independent of the absolute quantity and the kinetics of accumulation of mutated mRNA synthesized, and primer-extension analyses confirm that both viral promoters accurately utilize identical transcription start sites. These data thus reveal an unexpected property of RNA polymerase II promoters: determination of the post-transcriptional fate of the maturing mRNA, presumably by influencing alternative choices between as yet undefined processing and/or transport pathways.
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PMID:Determination of mRNA fate by different RNA polymerase II promoters. 823 61

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


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