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)

Forty-three patients with hematopoietic disease were treated with intensive chemotherapy and radiotherapy, followed by allogeneic bone marrow transplantation (BMT) from 28 HLA-identical and 10 one to two antigen haploidentical sibling donors and autologous BMT (5 cases). Of these cases, there were 21 with acute nonlymphocytic leukemia (ANLL), 5 with acute lymphocytic leukemia (ALL), 6 with chronic myelocytic leukemia (CML), 2 with Hodgkin's disease (HD), 8 with severe-form aplastic anemia (SAA) and 1 with thalassemia. Complications of BMT were evaluated including acute graft-versus-host disease (GVHD), interstitial pneumonia (IP), veno-occlusive liver disease (VOD), abnormalities of liver function (LF), and alteration of hepatitis B virus (HBV) markers. In thirty-three patients who were followed up for more than 3 months, we found that the incidence of moderate to severe acute GVHD (9.1%) and IP (two cases, 4.7%) were low. No VOD occurred in our series. During the follow-up period, 27 out of 35 patients (77%) had high alanine aminotransferase (ALT)/aspartate aminotransferase (AST) levels, even up to 1000 U/liter; however, only one patient succumbed to a hepatitis-related complication. Previous hepatic damage from HBV infection before BMT does not appear to increase the risk of posttransplant morbidity and mortality.
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PMID:Complications of bone marrow transplantation in Chinese. 232 72

Sera from 40 children with beta-thalassaemia major, 54 children with sickle-cell disease and 120 matched controls were examined by radio-immunoassay for hepatitis B and A markers. The overall prevalence of hepatitis B virus markers was highest in children with thalassaemia followed by children with sickle-cell disease and lowest in the control group. The hepatitis A marker showed a reversed pattern, being lowest in the thalassaemic group. The high incidence of hepatitis B infection was presumably due to frequent blood transfusions, frequent exposure to hospital environment and the high carrier rate in the community. Frequent blood transfusions, however, seem to protect against hepatitis A virus infection.
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PMID:Hepatitis B and A markers in children with thalassaemia and sickle-cell disease in Riyadh. 242 95

During the years 1982-1987, 66 patients with homozygous beta-thalassaemia were treated at the blood transfusion centre of Algiers. The patients, aged from 1 to 23 years in 1982, came from 48 families, 30 of which were issued from consanguinous unions. The patients fell into three groups according to the early institution and quality of treatment (blood transfusions, antibiotic therapy, desferrioxamine given when available). The beneficial clinical effects observed (satisfactory growth and development, reduction of splenomegaly and hypersplenism, attenuation of craniofacial malformations, performance at school) seemed to be directly related to the mean haemoglobin level prior to transfusion and to the early institution of treatment. Four patients died of anaemia and haemochromatosis. The incidence of viral contamination was 27.5 per cent for the hepatitis B virus and nil for the human immunodeficiency virus.
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PMID:[Treatment of homozygote beta thalassemia in Algiers. A 5-year follow-up of 66 patients]. 252 93

Thirty two patients with beta thalassaemia and sickle cell disease who were having regular blood transfusions were selected to test the efficacy and immunogenicity of low dose (2 micrograms or 0.1 ml) intradermal hepatitis B vaccine compared with the standard (20 micrograms or 1 ml) intramuscular dose. There was no significant difference in the rates of seroconversion, seroconversion had occurred in all patients by seven months. There were no significant differences in antibody titres between the intramuscular and intradermal groups at 1, 2, and 6 months. Although the titres were significantly higher in the intramuscular group at seven months and at 12-18 months, the antibody titre in the intradermal group did not fall below 10 IU/l. The results of this study suggest that low dose intradermal hepatitis B vaccination is an effective and economical way of stimulating an immune response in patients with beta thalassaemia and sickle cell disease.
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PMID:Intradermal hepatitis B vaccine in thalassaemia and sickle cell disease. 252 22

Sera from 101 children with thalassemia, aged between 6 months to 15 years, were examined for detection of HBV infection. Of these 101 children, 18 negative for all HBV markers were vaccinated against HB with "Hevac B" vaccine from the Pasteur Institute. Our results show that 3 inocculations at one-month intervals have induced an excellent antibody (anti-HBs) response in all vaccinated children, affording full protection against HBV infection. There were no noticeable local or general reactions to "Hevac B" vaccine. Our results, in accordance with other reports, have revealed that hepatitis B vaccine is highly immunogenic for children with thalassemia and is particularly well tolerated.
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PMID:Hepatitis B vaccination in children with thalassemia in northern Greece. 281 40

Frequencies of hepatitis B, delta and human immune deficiency virus markers were investigated in multitransfused thalassaemic (212) and sickle-cell disease (173) patients from four different regions of Saudi Arabia. Irrespective of the region, thalassaemics had higher exposure rate to HBV (14.7-31.6% HBsAg; 79.4-97.9% all markers) than sickle-cell disease patients (9.4-27.5% HBsAg; 45.3-85% all markers) and controls (5.8-21.3% HBsAg; 30.8-72.5% all markers). Evidence of delta infection was found in thalassaemics from all regions (7.1-21.0%); also, the prevalence of anti-delta was higher in thalassaemics than in sickle-cell disease patients and controls. Only five thalassaemics were positive for antibody to the human immune deficiency virus (HIV). In the light of these results, screening of all donated blood for HBsAg and for anti-HIV by the most sensitive techniques is essential. Furthermore, early vaccination of Saudi children with thalassaemia and sickle-cell disease against HBV is recommended.
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PMID:Frequencies of hepatitis B, delta and human immune deficiency virus markers in multitransfused Saudi patients with thalassaemia and sickle-cell disease. 291 72

Liver biopsies were performed on 51 regularly transfused patients with beta thalassaemia, age range 5-36 (mean 18.6) years, who had received regular subcutaneous desferrioxamine (DFX) treatment for periods between one and eight years (40 for eight years). The biopsy specimens were examined by light microscopy and immunofluorescence for hepatitis B virus surface and core antigens (HBsAg and HBcAg), and the iron content was determined chemically. The results were compared with serum ferritin concentration and aspartate transaminase (AST) activity and with hepatitis B virus serology. Biopsy specimens, in which chemical liver iron had been determined in 12, were also available from 17 patients. Mean serum ferritin (+/- SD) had fallen from 5885 (3245) micrograms/l to 1638 (976) micrograms/l in 36 patients after eight years' chelation, while mean (+/- SD) liver iron concentration had fallen from 2945 (900) micrograms/100 mg dry weight to 857 (435) micrograms/100 mg dry weight in 12 of them. All biopsy specimens examined were negative for HBs and HBc antigens. The presence of histological features of hepatitis was associated with increased liver iron content, increased fibrosis, and with progression of fibrosis between the two biopsies. Procollagen III peptide was assayed in 28 patients but did not correlate with the degree of hepatitis, fibrosis, or with chemical liver iron content. We conclude that with regular subcutaneous DFX, mean concentrations of serum ferritin and liver iron are maintained in these patients at about five and 10 times the normal value, respectively, and that progression of liver damage is more likely to be due to viral hepatitis, presumably related to the parenterally transmitted non-A, non-B agents than to iron overload.
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PMID:Iron state and hepatic disease in patients with thalassaemia major, treated with long term subcutaneous desferrioxamine. 312 79

Preliminary results of a trial involving a yeast-derived hepatitis B vaccine administered to 41 transfusion-dependent thalassaemic patients and 2 patients with spherocytosis are reported. Twenty-microgram doses of HBsAg were administered according to either a 0, 1, and 6 month or 0, 1, and 2 month schedule. Serum specimens collected prior to vaccination, one month after each vaccine dose, and again at 5 and 15 months, were tested for HBV markers and ALT. To date, seroconversion (anti-HBs titres greater than 10 IU/l) was observed in 15%, 67%, and 86% of patients one month following the three vaccine doses, respectively. Although the study is still in progress, a comparison of these results with those previously obtained using plasma-derived vaccine indicates that seroconversion to the recombinant yeast-derived vaccine is at least as high as that obtained by plasma-derived vaccines in patients affected by thalassaemia major.
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PMID:Yeast-derived hepatitis B vaccine in thalassaemic patients: a preliminary report. 331 53

During the period of 1978 to 1986, 66 patients (31 men, 35 women) with a mean age of 28.4 years and various sickle cell hemoglobinopathies underwent 82 surgical procedures; 28 were emergencies. Fifty of the 66 patients had HbSS, 13/66 had HbSC, and 3/66 had HbS-thalassemia. All 66 patients received transfusions, although not for all procedures. In 48 patients, transfusion therapy was only administered preoperatively. Simple transfusions (1 to 10 units) were administered in 31 of 48 procedures. Exchange transfusions (1 to 6 units) were performed in nine of 48 procedures. Preoperative hematocrit ranged from 7.0% to 54.2%; of those receiving transfusions the hematocrit ranged from 22.6% to 53.7%. Intraoperative transfusions (1 to 10 units) were performed in 14 of 82 procedures; postoperative transfusions (1 to 6 units) were performed in 13 of 82 procedures. No advantage was noted in preoperative exchange transfusion as measured by a decrease in postoperative complications; a slight increase was seen in atelectasis in this group of patients with preoperative transfusions. An increase was reported in the complication rate of patients with an hematocrit of less than 30%. The type of transfusion (preoperative, intraoperative, or postoperative) administered did not appear to be related to postoperative morbidity rates. The complication rate for simple transfusions was 51.6% and for multiple transfusions, 55.6%. HbSS hemoglobinopathy had the higher complication rate. The hepatitis B surface antigen was demonstrated in four of 66 (6.1%) patients; ten of 66 (15.2%) developed alloantibodies. The benefits of transfusion therapy should be judged according to clinical needs; not all sickle cell patients need exchange or preoperative transfusion.
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PMID:Assessment of the use of transfusion therapy perioperatively in patients with sickle cell hemoglobinopathies. 335 66

We studied 29 patients with thalassaemia major who had received intensive chelation for between 6.2 and 8.8 years. All patients had normal oral glucose tolerance tests before subcutaneous chelation therapy was introduced and 22 of 29 patients had normal liver function tests. At the end of the period of study 12 patients still had normal oral glucose tolerance (7 with normal liver function tests and 5 with chronic active hepatitis). On the other hand, 11 patients had developed impaired glucose tolerance tests (3 patients had normal liver function tests, 5 with chronic active hepatitis and 3 with cirrhosis), and 6 patients had developed frank diabetes mellitus (one with chronic active hepatitis and 5 with cirrhosis). Patients with chronic active hepatitis showed 91% positivity for one or more hepatitis B markers whilst all patients with cirrhosis were positive. Ferritin levels before subcutaneous chelation in patients with normal oral glucose tolerance tests were lower than in those patients with abnormal oral glucose tolerance or diabetes (P less than 0.05) but none had normal serum ferritin levels. In addition, a positive correlation was found between glucose area under the curve after chelation therapy and serum ferritin levels (r = 0.47, P less than 0.01). It is apparent that long term chelation therapy does not prevent the development of abnormal oral glucose tolerance in chronically transfused patients. More intensive chelation therapy is needed to prevent tissue damage. Chronic liver disease may have an important role to play in the deterioration of glucose tolerance.
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PMID:The development of diabetes mellitus and chronic liver disease in long term chelated beta thalassaemic patients. 354 13


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