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

Transfusion of whole blood or blood components is the mainstay of treatment in patients with beta-thalassemia and hemophilia. Owing to the scarcity of reports regarding the frequency of transfusion-transmitted hepatitis virus infections in thalassemia patients, the frequency of such infections was studied in India in 40 multi-transfused thalassemia patients (26 males, 14 females; mean age 8.1 +or- 5.3 years, range 1-35) with no clinical or biochemical evidence of liver disease. The enzyme-linked immunosorbent assay (ELISA) technique (Abbott) was used for all tests. The patients had received an average of 80 units (range 10-250) of blood. A majority of these units had been screened for hepatitis B surface antigen (HBsAg) using RPHA. HBsAg antibodies were present in 18 (45%), antihepatitis C virus (HCV) in 7 (17.5%), and antihuman immunodeficiency virus in 1 (2.5%) case, respectively. Of 18 HBsAg positive patients, antidelta and anti-HCV antibodies were present in 3 and 4 patients, respectively; 1 patient had both the antibodies. 4 of 40 (10%) patients had evidence of both hepatitis B virus (HBV) and HCV infection. In a US study, the frequencies of HBsAg and anti-HBs positively among thalassemics were 4.5% and 43.5%, respectively. In contrast, 90% of hemophiliacs show serological evidence of HBV infection. Routine screening of blood donors by CEP or RPHA technique was started in the hospital blood bank 7 years ago. The sensitivity of these techniques is much lower than that of RIA and ELISA and a majority of the patients has received initial blood transfusions before HBsAg screening was started. The study indicated that more than 50% of multi-transfused thalassemia patients showed serological evidence of one or more HBV, HCV, HDV, and HIV infection. Thus, screening of blood units for HBV, HCV, and HIV infections to be used for thalassemic patients and vaccination of thalassemic patients against hepatitis B is imperative.
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PMID:Frequency of hepatitis B, C and D and human immunodeficiency virus infections in multi-transfused thalassemics. 142 37

HIV is efficiently transmitted through transfusion with HIV-infected blood. Accordingly, 203 multitransfused children with thalassemia attending the thalassemia clinic of the Charak Palika Hospital in New Delhi were screened for antibodies to HIV using ELISA and Western blot tests. 8.37% of the sample tested HIV-seropositive (HIV+). These 17 children were joined by 3 others referred from a neighboring state to constitute a group to be matched against 20 HIV-children for the purpose of comparing psychosocial aspects. The control group was matched for age, sex, educational level, and socioeconomic status with mean age 10.8 years ranging over 1-16 years. 4 members of the HIV+ sample were diagnoses as having clinical AIDS according to WHO criteria. The remaining 14 boys and 2 girls were HIV+, but asymptomatic. 25% were of lower class, 63.5% middle class, and 12.5% upper class. Of those with AIDS, 50% were diagnosed in their first year of life and 82% were diagnosed by year 3. Symptoms generally developed after 4-6 months of life. Lymphadenopathy and hepatomegaly tend to be visible at birth, while chronic diarrhea, prolonged fever, oral thrush, recurrent bacterial infections, and hepatosplenomegaly may also be presented. 7.1% of cases aged 2-3 years exhibited rocking and head banging problems worse than did control subjects. Furthermore, 28.5% had temper tantrums and 21.5% ground teeth. These children may have delayed developmental milestones as well as behavioral problems. The small sample size, however, precludes concluding that psychosocial differences exist between those with HIV/AIDS and those with thalassemia major. In fact, behavioral problems in these children were due to child illness and not of HIV-positivity, for children tend to be unaware of HIV/AIDS infections and its consequences. The author recommends that HIV+ children continue to attend school unless they can not control bodily secretions, have uncoverable oozing lesions, have unacceptable behaviors, or if there is extreme possibility of contracting infectious diseases at school. The author also stresses parents' and families' need for long-term medical and psychological care.
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PMID:Psycho-social aspects of HIV infection and AIDS in multiple transfused thalassemic children. 145 60

To estimate the cumulative incidence of acquired immunodeficiency syndrome (AIDS) in thalassaemia major patients (TMP) human immunodeficiency virus (HIV-1) infected through transfusion, 79 seropositive TMP were studied. At inclusion, mean age was 12.4 +/- 6.6 years; 40 were men; 21 were splenectomized. Centers for Disease Control, 1986 (CDC) stages and prescription of zidovudine were noted at least once a year. Cumulative incidence of AIDS and standard error were calculated using non parametric life table method. Age, sex, acute infection and splenectomy associations with progression to AIDS were tested using Breslow statistic. The median follow-up period was 4 years 11 months. At the end of the study period, 43 TMP were in CDC stage II, 23 in CDC stage III and 13 in CDC stage IV, including seven AIDS cases, of whom three had died. Four subjects died of other causes. Only two patients were treated with AZT prior to the occurrence of AIDS. Rate of progression to AIDS was not associated with acute infection, splenectomy, age, or sex. A cumulative AIDS incidence rate of 1.4% (SE 1.3%) was observed at 3 years and of 9% (SE 4%) at 5 years.
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PMID:Incidence of AIDS in HIV-1 infected thalassaemia patients. European and Mediterranean W.H.O. Working Group on Haemoglobinopathies and Cooleycare. 152 Jun 8

A free radical is any species capable of independent existence that contains one or more unpaired electrons. Free radical reactions have been implicated in the pathology of more than 50 human diseases. Radicals and other reactive oxygen species are formed constantly in the human body, both by deliberate synthesis (e.g. by activated phagocytes) and by chemical side-reactions. They are removed by enzymic and nonenzymic antioxidant defence systems. Oxidative stress, occurring when antioxidant defences are inadequate, can damage lipids, proteins, carbohydrates and DNA. A few clinical conditions are caused by oxidative stress, but more often the stress results from the disease. Sometimes it then makes a significant contribution to the disease pathology, and sometimes it does not. Several antioxidants are available for therapeutic use. They include molecules naturally present in the body [superoxide dismutase (SOD), alpha-tocopherol, glutathione and its precursors, ascorbic acid, adenosine, lactoferrin and carotenoids] as well as synthetic antioxidants [such as thiols, ebselen (PZ51), xanthine oxidase inhibitors, inhibitors of phagocyte function, iron ion chelators and probucol]. The therapeutic efficacy of SOD, alpha-tocopherol and ascorbic acid in the treatment of human disease is generally unimpressive to date although dietary deficiencies of the last two molecules should certainly be avoided. Xanthine oxidase inhibitors may be of limited relevance as antioxidants for human use. Exciting preliminary results with probucol (antiatherosclerosis), ebselen (anti-inflammatory), and iron ion chelators (in thalassaemia, leukaemia, malaria, stroke, traumatic brain injury and haemorrhagic shock) need to be confirmed by controlled clinical trials. Clinical testing of N-acetylcysteine in HIV-1-positive subjects may also be merited. A few drugs already in clinical use may have some antioxidant properties, but this ability is not widespread and drug-derived radicals may occasionally cause significant damage.
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PMID:Drug antioxidant effects. A basis for drug selection? 172 62

A total of 124 Indian patients with haemophilia and 185 multiple transfused patients with thalassaemia, haemoglobinopathies, patients on chronic haemodialysis and others were screened for HIV-1 infection by a commercially available competitive ELISA test and supplementary Western Blot (WB) test. The results showed that HIV-1 infection was mostly confined to the haemophiliacs where 15 (12.1%) were confirmed to be positive for HIV infection. All except one had received both foreign and Indian cryoprecipitate. However, one haemophilic seroconverted within 4 months of receiving a cryoprecipitate manufactured in India.
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PMID:HIV infection in Asian Indian patients with haemophilia & those who had multiple transfusions. 202 94

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

Seropositivity to HBV (HBsAg) in multi-transfused patients of haemophilia A, haemophilia B, B thalassaemia and EB thalassaemia from Eastern India, was found to be 9, 0, 22.1 and 13 per cent respectively. HIV seropositivity was detected in patients of haemophilia A (4.4%) and B thalassaemia (0.8%) who received plasma components and packed cells periodically. Seropositivity to both HBsAg and HIV was found in one patient of haemophilia A. Serum alanine amino transferase (ALT), raised in multi-transfused thalassaemics suggests concurrent hepatitis which might have enhanced the transmission of viruses due to disturbed immune status. The universal voluntary blood donation programme, screening of blood for HBV and HIV by sensitive tests, early immunisation and periodic monitoring of HBV and HIV status are prerequisites for the management of transfusion dependent thalassaemia and haemophilia.
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PMID:HBV & HIV seropositivity in multi-transfused haemophilics & thalassaemics in eastern India. 234 33

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

The umbilical cord blood from 109 consecutive Zambian neonates (excluding those found to be anti-HIV positive) were analysed for haemoglobin (Hb) Bart's and for alpha thalassaemia by restriction endonuclease analysis. This showed that 52.3% had the genotype alpha alpha/alpha alpha, 38.5% had -alpha 3.7/alpha alpha, 7.3% had -alpha 3.7/-alpha 3.7 and 1.8% had alpha alpha alpha/alpha alpha. The alpha thalassaemia gene (-alpha) frequency was 0.27. There were no apparent differences in gene frequency between six major Zambian ethnic groups. Detection of Hb Bart's identified all alpha-thalassaemia homozygotes (-alpha/-alpha), but fewer than 10% of heterozygotes (-alpha/alpha alpha). alpha-thalassaemia was associated with slight but significant anaemia and microcytosis.
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PMID:Alpha thalassaemia in Zambian newborn. 270 99


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