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)

We studied the immune function of 33 long-term survivors of thalassemia after hematopoietic stem cell transplantation. Lymphocyte subsets, lymphoproliferative response and immunoglobulin were normal but the level of natural killer cells was low. Five and seven patients had suboptimal antibody response at 4 week after pneumococcal and hepatitis B vaccine, respectively, but this response returned to normal by 6 months.
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PMID:Immunological reconstitution in long-term survivors of thalassemia major patients after hematopoietic stem cell transplantation. 1455 20

Hepatitis C virus (HCV) infection is a common cause of liver disease in thalassemia major patients in Western, especially Mediterranean, countries. Its significance in thalassemic patients from Southeast Asia has not been critically evaluated. In this report, we describe our study of the prevalence of HCV infection among Thai patients with thalassemia. The relationships of the infection to blood transfusion and the infection's effects on liver function have also been determined. Of the 104 patients studied, 21 (20.2%) tested positively by enzyme immunoassay for anti-HCV antibody, whereas only 2 patients (2%) had the hepatitis B surface antigen. There was no significant relationship between the presence of anti-HCV antibodies and the number and frequency of blood transfusions. In fact, 2 patients (10%) who tested positive for anti-HCV antibodies had never received transfusions. Patients with anti-HCV antibodies had significantly abnormal liver functions, such as higher levels of serum aspartate aminotransferase (SGOT) and alanine aminotransferase (SGPT) and lower levels of serum albumin, compared with patients without anti-HCV antibodies (P = .021, .017, and .004, respectively). However, there were also significant correlations between iron status as indicated by transferrin saturation or serum ferritin levels and SGOT, SGPT, and gamma-glutamyltransferase (GGT) levels. Moreover, abnormal liver function as represented by elevated levels of SGOT, SGPT, GGT, and serum alkaline phosphatase was observed more frequently in patients with iron overload than in patients with a lower degree of iron burden. The presence of HCV did not alter the effects of iron overload on liver function. The findings suggest that both HCV and iron overload are the main causes of abnormal liver function in Thai patients with thalassemia. The treatment of both problems, if coexisting in patients with thalassemia, is required to prevent progression to chronic liver disease.
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PMID:Prevalence and clinical significance of hepatitis C virus infection in Thai patients with thalassemia. 1468 98

Hepatitis C virus is considered to be the main aetiological agent responsible for the occurrence of post-transfusion hepatitis. Patients with thalassaemia acquire hepatitis most often from viruses contracted through blood transfusions. The present study was undertaken to evaluate the prevalence of hepatitis C virus (HCV) in thalassaemic patients with multiple blood transfusions. The association of HCV seropositivity with number of blood transfusions and liver enzyme profile was also analysed. The study group consisted of fifty patients (40 males and 10 females) attending the thalassaemic unit of Lok Nayak Hospital, a tertiary care hospital at Delhi, within the age group of 1-25 years. Thirty patients (60%) were found to be seropositive for HCV antibodies while one patient (2%) was co-infected with HCV antibodies and hepatitis B surface antigen. Study of liver enzyme profile showed aspartate aminotransferase levels to be significantly higher, although the level of serum alanine aminotransferase, alkaline phosphatase, total protein, bilirubin and albumin were not significantly altered in these patients. It is inferred from this study that 60% of the thalassaemics were infected with HCV and this was directly related to the number of blood transfusions received by them. The regularised national blood policy followed by blood banks for providing safe blood along with better screening method of donated blood in blood banks would bring down the incidence of hepatitis C in such high risk group.
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PMID:Anti-HCV seropositivity among multiple transfused patients with beta thalassaemia. 1600 15

Hepatocellular carcinoma (HCC) is a complication of cirrhosis. Due to blood transfusions, patients with beta-thalassemia (thal) are often infected with either hepatitis C virus (HCV) or hepatitis B virus (HBV). In the past, many patients did not survive long enough to develop HCC. The recent improvements in prognosis have helped in the diagnosis of HCC that has developed. The aim of this study was to evaluate HCC incidence in beta-thal. We performed liver ultrasound (US) on all adults without a previous diagnosis of HCC. Risk factors (iron overload, HCV infection, HBV infection, cirrhosis) were evaluated. One hundred and eight thalassemia patients have been evaluated; of whom three were excluded (two patients as they were under the age of 18 years and one patient because he had a previous history of HCC). Seventy-two patients [31 had thalassemia major (TM), 41 had thalassemia intermedia (TI)] with risk factors (iron overload in 72, HCV infection in 46, HBV infection in two, cirrhosis in 10) and 33 (four with TM and 29 with TI) without risk factors underwent liver US. Overall, two patients were found to have a newly developed HCC. Of these two patients, one was treated with surgery and the other with percutaneous radiofrequency. Further follow-up did not show any evidence of recurrence after 23 and 15 months, respectively. Ultrasound screening can allow early detection and treatment of HCC in thalassemia patients.
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PMID:A prospective study of hepatocellular carcinoma incidence in thalassemia. 1654 Apr 24

In hematology patients on chronic transfusion regimes, liver diseases are frequent, and mostly related to the agents transmitted by blood products and concominant iron deposition in liver. Besides hepatitis B (HBV) and C (HCV) viruses, new viral agents like hepatitis G virus (HGV) and TorqueTeno virus (TTV) are identified in these patients, although their association with any pathology or disease is not yet proved. In the present work, the authors studied the clinical importance of TTV in Turkish multitransfused patients with thalassemia. Forty-six healthy and 57 thalassemic patients were enrolled in the study. TTV was detected in serum samples by 3'-UTR nested PCR. Transaminase and ferritin levels, hepatitis B and C virus markers and number of transfusions were interpreted for possible association with TTV infection. As a result, TTV was detected in 63% of the thalassemia and 54% of the control patients. Prevalence of TTV infection, clinical features, laboratory data, and annual transfusion numbers of TTV-positive and -negative patients were not observed to be statistically significant. In conclusion, in Turkish patients with thalassemia, TTV infection cannot be considered as a risk factor for liver disease.
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PMID:Transfusion-transmitted virus prevalence in Turkish patients with thalassemia. 1662 77

Deferiprone (L1) is an orally active iron-chelation agent that is being evaluated as a treatment for iron overload in thalassemia major. Although some reports have concluded that LI may exacerbate hepatic fibrosis and the deterioration of liver function in thalassemia patients, other studies have reported no detrimental effects. In view of these serious concerns regarding the hepatic toxicity of LI, a Taiwanese group of beta-thalassemia (thal) patients with the longest known duration of LI therapy and who had provided liver biopsies, were enrolled in this study. From April 1999 to July 2004, the 17 enrolled thalassemia major patients had been on L1 therapy for as long as 19 to 60 months. Two liver biopsies from each of the 17 patients were received at the China Medical University Hospital, Taichung, Taiwan. Serum alanine aminotransferase (ALT), viral serological studies for hepatitis B and hepatitis C, iron scores and fibrosis scores were available at the beginning of the study and at the time of the second biopsy. Overall, the 17 patients received L1 therapy continuously for a mean period of 3.3 years. With the exception of two patients, fibrosis scores decreased in all patients after LI therapy. Three patients had increased iron scores after therapy of L1 and 11 patients had increased ALT levels; increased ALT levels occurred more frequently in hepatitis C positive patients. In this study, most thalassemia major patients had no progression of hepatic fibrosis or increased liver iron stores during long-term LI therapy.
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PMID:Liver fibrosis and iron levels during long-term deferiprone treatment of thalassemia major patients. 1679 46

Along with hepatitis B virus (HBV) and human immunodeficiency virus (HIV), Hepatitis C virus (HCV) is emerging as a major transfusion hazard. 22 cases of haemophilia (A 19, B 3) and 20 cases of thalassaemia (2 16, E(2) 4) constituted the study group. Patients tested for anti HCV (using third generation ELISA), HBsAg and antibodies to HIV I and II. Prevalence of anti HCV was 54.5% in haemophilics and 5% in thalassaemics. HBsAg was detected in 9.09% haemophilics and 5% thalassaemics. No anti HIV was detected in this cohort. Anti HCV seropositivity in haemophilics has increased compare to previous studies.
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PMID:Prevalence of anti HCV, HBsAg and HIV antibodies in high risk recipients of blood and blood products. 1719 61

Increasing numbers of successful pregnancies are reported in recipients of allogeneic hemopoietic stem cell transplantation (HSCT). These may occur naturally, or more commonly, through assisted reproduction. The pregnancy outcomes are usually normal. There are currently no guidelines on the prenatal management of pregnancies involving HSCT recipients. HSCT recipients are unique in that their red cells, lymphocytes and even the DNA in the circulation are donor derived. As a result, typical prenatal screening tests in parents, including mean cell volume (MCV), hemoglobin pattern, blood group, infective serology and DNA screening, are all affected. The MCV cannot be used as guide for iron and folate supplements, or for thalassemia and sickle cell anemia screening. Such screening must be based on pre-HSCT indices and pre-HSCT DNA samples. The risks for hemolytic disease of newborn and hepatitis B virus transmission have to be re-evaluated, based on both pre- and post-HSCT patient as well as donor blood group and serology results. Good communication between obstetricians and the HSCT physician is paramount to promote successful pregnancies in this distinct patient population.
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PMID:Challenges and pitfalls in prenatal screening in pregnancies involving allogeneic stem cell transplantation recipients. 1731 Jan 36

In a two-year retrospective review of the thalassemia program in our hospital, relevant clinical and laboratory items of information were extracted and analyzed. There were 12 regular attendants; seven males and five females, with a mean age of 6.9 +/- 3.3 years and a mean age at the time of diagnosis of 18.6 +/- 8.7 months. Mean hemoglobin at diagnosis was 6.5 +/- 1.1 g/dL and MCV was 67.9 +/- 4.6 fL. Predominant hemoglobin was HbF (30% to 98%). Presenting features included pallor, abdominal distention, hepatomegaly, splenomegaly and occasional fever. Mean pretransfusion hemoglobin was 8.3 +/- 1.5 g/dL and mean post-transfusion hemoglobin was 12.2 g/dL. Only two (16.7%) had weight less than the 5th percentile; six (50%) had weight >/=25th percentile while eight (66.7%) had height >/=10th percetile. Mean weight gain per year was 1.3 +/- 0.82 kg and linear growth rate was 4.27 +/- 1.66 cm. One patient was positive for hepatitis C virus (detected by antibody) and another was positive for both hepatitis B (both antigen and antibody) and C. Apart from admissions for routing blood transfusion, none of the patients were hospitalzed for any other morbid events in two years. High serum ferritin level (1482 +/- 766 mg/L) despite subcutaneous desferrioxamine remains a problem.
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PMID:Experience with thalassemia major in Al Baha. 1758 16

We report 2 cases of ground-glass hepatocyte inclusions occurring in pediatric patients. Case 1 had alpha-thalassaemia major and was receiving iron chelation therapy, whereas case 2 had trisomy 21 with a history of bone marrow transplantation for acute myeloid leukemia. The liver sections in both cases showed eosinophilic, periodic acid-Schiff diastase-positive intracytoplasmic inclusions that were negative for hepatitis B surface antigen. Immunohistochemically the inclusions showed positive staining with KM279, a monoclonal antibody against polyglucosan derived from Lafora inclusions. On electron microscopy, in case 1, intracytoplasmic inclusions were composed of degenerate organelles, glycogen, and irregular fibrillar structures; in case 2, they were composed of vesicular structures containing granular material. Ultrastructural changes in both cases differed from classical Lafora inclusions and ruled out hepatitis B surface antigen, glycogenosis type IV, and fibrinogen storage disease. Genetic analysis of the Lafora's disease genes performed in case 2 revealed no mutations. The development of hepatocyte cytoplasmic inclusions in both our cases could be related to medication effects, because similar inclusions were reported in patients using cyanamide. Drug-induced inclusions, mimicking Lafora's disease, should be included in the differential diagnosis of hepatocyte ground-glass inclusions.
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PMID:Lafora-like ground-glass inclusions in hepatocytes of pediatric patients: a report of two cases. 1792 93


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