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)

Iron overload was found in 3 patients who had undergone partial gastrectomy: a 61-year-old woman developed iron overload because she may have had idiopathic haemochromatosis and had also been given parenteral iron; in a 62-year-old man with thalassaemia minor, iron overload may have developed because of increased oral iron ingestion, low serum folate, increased, albeit ineffective, erythropoiesis and sideroblastic anaemia; a 74-year-old man with thalassaemia minor developed iron overload without exogenous therapy and died from a hepatoma. These cases illustrate that partial gastrectomy fails to protect patients from developing iron overload, particularly if given uncontrolled iron therapy.
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PMID:Iron overload despite partial gastrectomy. 53 65

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

The occurrence of a hepatocellular adenoma is described in a young woman with beta-thalassemia and secondary iron overload. This patient had no history of oral contraceptive use, and in fact was hypogonadal as a result of iron deposition in pituitary and gonadal tissue. Although hepatocellular carcinoma frequently occurs in diseases associated with iron overload, this is the first report of a benign liver tumor associated with secondary iron overload.
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PMID:Hepatocellular adenoma in a young woman with beta-thalassemia and secondary iron overload. 626 45

23 descendents of a 74--year-old Englishman who had beta-thalassaemia trait, and died of hepatoma, were studied to discover whether thalassaemia minor alone could predispose to iron overload. Serum ferritin and HLA antigens were assessed in all members, and adults underwent radioiron investigations and liver biopsy. 2 members of the second generation and 1 of the third generation, all of whom had thalassaemia trait, had elevated liver iron concentration, indicating preclinical iron overload. This was not associated with any HLA type. None of the subjects had been treated with exogenous iron. The one member of the second generation who had thalassaemia minor but not iron overload was female, and the 5 members of the third generation with the trait, but with normal serum ferritin levels, were all under the age of 15 years. Members of the family without beta-thalassaemia minor had normal iron metabolism. It is possible that the development of iron overload in 4 members of this family was related to the presence of thalassaemia minor, and not to the inheritance of another abnormal gene causing idiopathic haemochromatosis.
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PMID:Iron overload in beta-thalassaemia minor. A family study. 734 95

An 18-year-old woman had a hepatocellular adenoma of 4 cm across in the right lobe of the liver which was severely hemosiderotic because of beta-thalassemia hemoglobin E disease with numerous blood transfusions. The lesion was an accidental postmortem finding. To our knowledge, this is the second example of liver-cell adenoma occurring in a patient with beta-thalassemia hemoglobin E disease with secondary iron overload. It is suggested that this is an association between hepatocellular adenoma and secondary iron overload of liver cells, a similar event to the relation observed in hepatocellular carcinoma and secondary iron overload of hepatocytes.
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PMID:Hepatocellular adenoma in a beta-thalassemic woman having secondary iron overload. 779 35

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

No experience has been reported to date in treating chronic hepatitis C virus (HCV) infection with interferon (IFN) therapy after BMT, mainly due to concerns related to the impact of an immunomodulatory drug in patients who are immunologic and haematologic chimeras. However, chronic inflammatory activity related to HCV infection results in a chronic fibrogenous mechanism potentially leading to liver cirrhosis and hepatocellular carcinoma. Moreover, patients transplanted for beta-thalassemia could be at greater risk because of concomitant iron overload and pre-existing fibrous liver damage. Eleven patients with serological, biochemical, histological and molecular biological evidence of HCV infection were included in the study and treated for 6-12 months with recombinant IFN 24-65 months following BMT. The serum alanine aminotransferase (ALT) was persistently elevated (range 85-1242 U/l; mean 416) for at least 1 year prior to IFN treatment. Ten patients completed the protocol; five were considered as responders to treatment. In these five patients the liver histology showed an overall reduction of inflammation and necrosis: histological inflammatory activity improved from chronic active hepatitis (CAH) to chronic persistent hepatitis (three patients) or minimal residual inflammatory activity (two patients). The Knodell total activity score varied from 5.4 (range 3-9) to 1.4 (range 1-2; P = 0.05). All responding patients revealed negativization of serum HCV-RNA, that has been persistent in four (follow-up 1-3 years). ALT level fell to 15-80 U/l (mean 52; P = 0.0027). No major complications occurred during the therapy and no influence on marrow engraftment parameters were noted. We conclude that IFN therapy does not adversely interfere with engraftment and that it is a feasible therapy for treatment of chronic hepatitis C virus after BMT.
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PMID:Alpha-interferon treatment of chronic hepatitis C after bone marrow transplantation for homozygous beta-thalassemia. 938 79

The novel hepatitis TT virus first described by a Japanese group has been reported to be parenterally transmitted and furthermore, to have been detected in patients with hepatitis of unknown etiology. Hence, in the present study its prevalence was investigated within groups at high risk for contracting blood-borne viruses, such as individuals with chronic liver disease, intravenous drug users and recipients of blood and blood products, as compared to voluntary blood donors and pregnant women. To that end, DNA was extracted from sera obtained from the respective patients and subjected to PCR using semi-nested primers. The frequency of TTV DNA detected within high risk groups, such as nine out of 50 patients with chronic non-A-to-G liver disease (18%), nine out of 98 hepatocellular carcinoma cases (9.2%), 17 out of 52 intravenous drug users (32.7%), 15 out of 80 thalassemia patients with multiple blood transfusions (18.8%) and three out of 31 prostitutes (9.7%) exceeded that among voluntary blood donors and pregnant women, which amounted to 14 out of 200 (7%) and seven out of 103 (6.8%), respectively. Additional molecular research should be performed in order to determine its short-, as well as long-term clinical significance.
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PMID:Hepatitis TT virus infection in high-risk groups. 986 59

Hepatitis C virus (HCV) infection may occur in infants and children, although it is much less common than it is in adults. The main transmission routes include mother-to-infant transmission, use of HCV infected blood products, unsterile needles or syringes and other invasive procedures. The natural course of HCV infection in children is variable: some (20-40%) develop an acute resolving infection and spontaneous regression occurs in approximately one-third of infants of HCV infected mothers before 2 years of age. Approximately 60-80% of HCV infected children develop a chronic infection with varying degrees of activity and fibrosis, mostly mild during childhood. However, the potential risks of liver cirrhosis and hepatoma during later life are obvious. Interferon is the main agent used to treat HCV infection in children. The response to interferon at the end of 4-12 months of therapy ranges from 25-90%. A sustained response was found in 36-56% of children 6-36 months after the end of therapy. The duration of therapy is recommended to be 12 months. At the end of 3 months, an evaluation of the response is indicated in the majority of children, except those with thalassemia, in whom evaluation of response should be conducted at the end of 6 months of therapy. The benefit of other therapies, such as combination therapy with interferon and ribavirin in children with hepatitis C is currently under investigation.
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PMID:Treatment of chronic hepatitis C virus infection in children. 1089 Mar 26

Hepatocellular carcinoma (HCC) frequently complicates hepatic cirrhosis secondary to viral infection or iron overload. Therefore, patients affected by thalassaemia syndromes have a theoretically high risk of developing the tumour. We collected data on patients attending Italian centres for the treatment of thalassaemia. Twenty-two cases of HCC were identified; 15 were male. At diagnosis, the mean age was 45 +/- 11 years and the mean serum ferritin was 1764 +/- 1448 microg/l. Eighty-six percent had been infected by hepatitis C virus. Nineteen of 22 cases were diagnosed after 1993, suggesting that this problem is becoming more frequent with the aging population of thalassaemia patients.
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PMID:Hepatocellular carcinoma in the thalassaemia syndromes. 1467 16


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