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)

Hepatitis C virus is responsible for the majority of cases of post-transfusion non-A non-B hepatitis in patients with thalassemia major. Interferon alfa is an effective treatment for patients with chronic hepatitis C. Response to therapy is related to the duration of treatment, the viral load in serum, and the hepatitis C virus genotype. The purpose of this study was to estimate the response of multitransfused children with beta-thalassemia and chronic hepatitis C to interferon alfa-2b therapy. Thirteen patients with beta-thalassemia and chronic hepatitis C, (mean age+/-SD, 14.1 +/- 1.7 years) participated in the study. Liver biopsy, estimation of HCV RNA, and virus genotyping were performed before onset of treatment. All patients were positive for HCV RNA in a low concentration; two patients carried the la genotype, four had genotype 3, and seven had genotype 4. Patients were treated with 3 x 10(6) U of subcutaneous interferon alfa-2b three times weekly. Eleven of 13 patients received therapy for 18 months; the remaining two underwent therapy for 6 months. Six of 13 patients responded completely to therapy, four responded partially, and three did not respond at all. The grade of inflammation and stage of fibrosis was lower in complete responders. Complete responders had lower ferritin values compared with the values for partial and nonresponders before starting therapy. The results suggest that interferon therapy should be recommended for children with beta-thalassemia major complicated by a low viral concentration of hepatitis C.
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PMID:Response to interferon alfa-2b therapy in mutitransfused children with beta-thalassemia and chronic hepatitis C. 1058 97

In previous trials, the orally active iron chelator deferiprone (L1) has been associated with sporadic agranulocytosis, milder forms of neutropenia and other side-effects. To determine the incidence of these events, we performed a multicentre prospective study of the chelator. Blood counts were performed weekly, and confirmed neutropenia mandated discontinuation of therapy. Among 187 patients with thalassaemia major, the incidence of agranulocytosis (neutrophils < 0.5 x 109/l) was 0.6/100 patient-years, and the incidence of milder forms of neutropenia (neutrophils 0.5-1.5 x 109/l) was 5.4/100 patient-years. All cases of neutropenia resolved after interruption of therapy. Neutropenia occurred predominantly in non-splenectomized patients. Nausea and/or vomiting occurred early in therapy, was usually transient and caused discontinuation of deferiprone in three patients. Mild to moderate joint pain and/or swelling did not require permanent cessation of deferiprone and occurred more commonly in patients with higher ferritin levels. Mean alanine transaminase (ALT) levels rose during therapy. Increased ALT levels were generally transient and occurred more commonly in patients with hepatitis C. Persistent changes in immunological studies were infrequent, although sporadic abnormalities occurred commonly. Mean zinc levels decreased during therapy. Ferritin levels did not change in the overall group but decreased in those patients with baseline levels > 2500 microgram/l. This study characterized the safety profile of deferiprone, and, under the specific conditions of monitoring, demonstrated that agranulocytosis is less common than previously predicted.
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PMID:Safety profile of the oral iron chelator deferiprone: a multicentre study. 1069 60

Regular blood transfusions for patients with thalassemia have improved their overall survival although these transfusions carry a definite risk of the transmission of certain viruses. Infection with hepatitis B virus (HBV), hepatitis C virus (HCV), cytomegalovirus (CMV) and human immunodeficiency virus (HIV) leads to complications which contribute to the morbidity and mortality of patients with thalassemia. We analyzed the blood samples taken from 85 transfusion dependent thalassemics receiving treatment at the day care center in Hospital Universiti Kebangsaan Malaysia and found that the seroprevalence rates for HBV, HCV and CMV were 2.4%, 22.4% and 91.8% respectively. None of the patients tested positive for HIV. Those positive for HBV and HCV will require further tests and treatment if chronic hepatitis is confirmed.
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PMID:Seroprevalence of hepatitis B, hepatitis C, CMV and HIV in multiply transfused thalassemia patients: results from a thalassemia day care center in Malaysia. 1077 66

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

To date the true prevalence of hepatitis C virus (HCV) mixed-genotype infections has not been established mainly because currently available methods are not suitable for the detection of mixed genotypes in a viral population. A novel semiautomated genotyping method, primer-specific and mispair extension analysis (S-PSMEA), which is more reliable than other genotyping assays was developed for detection of HCV mixed-genotype infections. A genotype present at levels as low as 0.8% in a defined mix of HCV genotypes was detected, showing a 20-fold increase in sensitivity over that of direct DNA sequencing. A total of 434 HCV isolates were genotyped and analyzed for a comparative study of the accuracy between S-PSMEA and four current genotyping methods. The results showed that viruses in approximately 40% of the samples from this group determined to be infected with mixed genotypes by S-PSMEA were undetected by direct DNA sequencing due to its low sensitivity. Type-specific PCR, line probe assay, and restriction fragment length polymorphism analysis performed poorly, being able to identify only 38.5, 16.1, and 15.4% of mixed-genotype infections, respectively, that were detected by direct DNA sequencing. The prevalence of mixed-genotype infections detected by S-PSMEA was 7.9% (12 of 152 donors) among HCV-infected blood donors, 14.3% (15 of 105) among patients with chronic hepatitis C, and 17.1% (6 of 36) among thalassemia patients who had received multiple transfusions. The data lead us to conclude that HCV mixed-genotype infections are more common than previously estimated and that S-PSMEA may be the method of choice when detection of genotypes present at low levels in mixed-genotype infections is required due to its higher level of sensitivity.
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PMID:Comparison and application of a novel genotyping method, semiautomated primer-specific and mispair extension analysis, and four other genotyping assays for detection of hepatitis C virus mixed-genotype infections. 1092 31

All patients presenting with hereditary hemolytic anemia, (n = 143) over a period of 18 months were enrolled in a study to evaluate the prevalence of hepatitis B, hepatitis C and HIV in multi-transfused patients in Jordan, and to identify possible related risk factors. All patients were treated in the Thalassemia Unit at Princess Rahma Teaching Hospital. Relevant clinical data were collected. Blood specimens were taken from these patients and tested for HbsAg, HbsAb, hepatitis core IgMAb, hepatitis core IgGAb, HCVAb, and ELISA for HIV. Fifty-eight (40.5 per cent) of the specimens were HCVAb positive, while only five (3.5 per cent) of them were positive for HBsAg. None of the specimens were positive for HIV. The frequency of blood transfusion and the time of diagnosis before or after 1995, were investigated as possible risk factors for viral seropositivity. Only the time of diagnosis was a statistically significant risk factor for HCVAb positivity (OR = 4.49; p = 0.005). In conclusion, hepatitis C acquisition is a serious risk for multi-transfused patients in Jordan. Hepatitis B is relatively less common. Blood screening initiated after 1995 in Jordan has significantly reduced the risk of hepatitis C associated with blood transfusion.
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PMID:The prevalence of hepatitis B, hepatitis C and human immune deficiency virus markers in multi-transfused patients. 1152 66

Hepatitis C virus (HCV) infection is common in transfusion-dependent thalassaemia. The clinical usefulness of 12-month treatment, using interferon alpha 3 MIU/m2 thrice weekly and oral ribavirin 16 mg/kg/d, was evaluated in 18 previously untreated thalassaemia patients. The median age at start of treatment was 16 years (range 7-29). Fourteen were infected with genotype 1b and 4 with genotype 6a. The sustained biochemical and virological response rates 6 months after stopping treatment were both 72.2%. Blood consumption was temporarily increased by 30% due to ribavirin-associated haemolysis. This study demonstrated a high, sustained response rate to combination treatment despite infection with genotype 1b.
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PMID:Interferon and ribavirin as frontline treatment for chronic hepatitis C infection in thalassaemia major. 1202 54

Exposure to hepatitis C virus (HCV), hepatitis G virus (HGV) and the carrier 'rate' for hepatitis B virus (HBsAg) were investigated in thalassaemia patients in Lebanon, a group that has not been studied in the past. The HCV genotypes and their distribution in the 395 thalassaemics, all of whom had been registered at the Chronic Care Center (CCC) in Hazmieh since 1996, were also studied. Of the 55 samples (14%) found positive for anti-HCV, 19 were also positive for HCV RNA. The 19 samples of HCV RNA were mostly of genotype 4 (37%), followed by 1a and 3a (21% each), lb (16%) and 2b (5%). Most (14; 74%) of the 19 HCV-RNA-positive samples, but only 13 (36%) of the 36 samples that were negative for HCV RNA although anti-HCV-positive, were positive for anti-HGV. Among 100 anti-HCV-negative samples, eight (8%) were anti-HGV positive. Only one (0.28%) of all 395 patients investigated was found to be HBsAg-positive. All of the HBV- and HCV-positive patients had initially been found positive in 1996, when they were first registered at the CCC, and none of the remaining patients had seroconverted since. As none of the patients had been checked for anti-HGV until the present study, the history of their exposure to HGV was unknown. These results emphasise the importance of screening all blood donations collected in Lebanon for HBsAg and anti-HCV. This and stringent infection-control measures are necessary steps to limit the spread of HBV, HCV and perhaps HGV to thalassaemics.
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PMID:Hepatitis-C-virus genotypes and hepatitis-G-virus infection in Lebanese thalassaemics. 1208 Sep 81

Patients with thalassemia major require lifelong chelation therapy to prevent iron-induced organ damage. The orally active chelator deferiprone has been proposed as an alternative for patients unable or unwilling to use deferoxamine. One report has concluded that deferiprone may worsen hepatic fibrosis in patients with thalassemia, whereas others have found no detrimental effect. A panel of 3 pathologists evaluated 112 coded liver biopsies obtained from 56 patients before and after deferiprone therapy. Fibrosis was scored with the Laennec and Ishak systems. The mean interval between liver biopsies was 3.1 years (range, 1.2-4.9 years). In 11 patients seronegative for hepatitis C, fibrosis scores before and after therapy were 1.12 +/- 1.07 and 0.97 +/- 0.84 (P =.42) with the use of the Ishak system, and 0.71 +/- 0.65 and 0.70 +/- 0.53 (P =.91) with the Laennec system. Among 45 patients seropositive for hepatitis C, fibrosis scores before and after therapy were 1.91 +/- 1.13 and 2.04 +/- 1.30 (P =.43) with the use of the Ishak system and 1.26 +/- 0.73 and 1.35 +/- 0.90 (P =.41) with the Laennec system. When the data set was limited to biopsies that each contained 6 or more portal tracts (31 patients), analysis still showed no significant change in fibrosis with time. With the use of the Laennec system, the fibrosis score did not increase by more than one level in any patients without hepatitis C; it increased by more than one level in 1 patient with hepatitis C; and it did not decrease by more than one level in any of the 56 patients. This analysis of the largest collection of liver biopsies reported to date in patients receiving deferiprone demonstrates no evidence of deferiprone-induced progression of hepatic fibrosis during long-term therapy.
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PMID:Lack of progressive hepatic fibrosis during long-term therapy with deferiprone in subjects with transfusion-dependent beta-thalassemia. 1278 94

Hepatitis C virus (HCV) seroprevalence and risk factors in north Iran were investigated in 105 thalassemia sufferers, 93 haemodialysis patients and 5976 blood donors by second generation ELISA. Our study showed that haemodialysis patients and thalassemia sufferers were at higher risk of having HCV infection; the prevalence being 55.9% and 63.8% respectively in comparison to the prevalence of blood donors (0.5%). A confirmatory immunoblotting was employed using HCV-positive cases (54 thalassemia sufferers and 19 blood donors). The result showed that 92.6% of samples of the first group and 10.5% of the latter were positive. Thus, it can be suggested that ELISA in low-risk cases may produce considerable false positives. In HCV-positive patients with thalassemia, the incidence of HCV among different age groups and genders was similar but a strong correlation in respect to the number of blood transfusion (P=0.008) was observed. In HCV-positive haemodialysis patients, it was found that there was no correlation with liver function tests (alanine aminotransferase and aspartate aminotransferase: ALT and AST), but a significant correlation was observed in respect to the duration of dialysis(P=0.000) and the number of units transfused (P=0.000). Consequently, it still seems blood transfusion is the main factor for increasing the incidence of HCV in thalassemia sufferers and haemodialysis patients.
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PMID:Prevalence of hepatitis C virus infection in thalassemia and haemodialysis patients in north Iran-Rasht. 1222 35


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