Thalassemia Center

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Thalassemia Center

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Saiviroonporn P.,Siriraj Hospital | Viprakasit V.,Thalassemia Center | Khuhapinant A.,Mahidol University | Wood J.C.,University of Southern California
Journal of Computer Assisted Tomography | Year: 2011

Purpose: To compare the observer variability of the conventional region-based (RB) to the typical and proposed pixel-wise (PW) methods for cardiac T2* analysis in thalassemia patients. Design AND Methods: Fifty thalassemia major patients were enrolled for the study. Short-axis bright- and black-blood sequences were acquired and analyzed using the RB and PW methods. Regions were defined using the whole septum (WS) or partial septum (PS). From the same PS region, results were reported by mean (PS-PW) and median (MPS-PW). Intraobserver and interobserver variabilities were investigated on all data set by 2 independent observers blinded to the result. Results: The T2* values from the PS-PW and MPS-PW methods were comparable to the conventional WS-RB method on both scanning techniques. When comparing the interobserver variability from the WS-RB to the PS-PW method, the coefficient of variation of the PS-PW method was equivalent (4.5% vs 4.7%, P = NS) for the bright-blood technique but 31% lower (4.0% vs 2.8%, P = 0.21) for the black-blood technique. The proposed MPS-PW method performed even better with respect to the conventional WS-RB method, decreasing interobserver coefficient of variation by 24% (4.5% vs 3.5%, P = 0.08) and 42% (4.0% vs 2.4%, P = 0.02), respectively. Intraobserver reproducibility followed the same trend. Conclusions: The proposed PW method using the median of T2* values calculated from partial interventricular septum region provided lower intraobserver and interobserver variabilities compared with the conventional RB or typical PW methods. Copyright © 2011 by Lippincott Williams & Wilkins.


Carpenter J.-P.,Royal Brompton and Harefield NHS Foundation Trust | Carpenter J.-P.,Imperial College London | Roughton M.,Royal Brompton and Harefield NHS Foundation Trust | Pennell D.J.,Royal Brompton and Harefield NHS Foundation Trust | And 50 more authors.
Haematologica | Year: 2013

Accumulation of myocardial iron is the cause of heart failure and early death in most transfused thalassemia major patients. T2* cardiovascular magnetic resonance provides calibrated, reproducible measurements of myocardial iron. However, there are few data regarding myocardial iron loading and its relation to outcome across the world. A survey is reported of 3,095 patients in 27 worldwide centers using T2* cardiovascular magnetic resonance. Data on baseline T2* and numbers of patients with symptoms of heart failure at first scan (defined as symptoms and signs of heart failure with objective evidence of left ventricular dysfunction) were requested together with more detailed information about patients who subsequently developed heart failure or died. At first scan, 20.6% had severe myocardial iron (T2*≤10ms), 22.8% had moderate myocardial iron (T2* 10-20ms) and 56.6% of patients had no iron loading (T2*>20ms). There was significant geographical variation in myocardial iron loading (24.8-52.6%; P<0.001). At first scan, 85 (2.9%) of 2,915 patients were reported to have heart failure (81.2% had T2* <10ms; 98.8% had T2* <20ms). During follow up, 108 (3.8%) of 2,830 patients developed new heart failure. Of these, T2* at first scan had been less than 10ms in 96.3% and less than 20ms in 100%. There were 35 (1.1%) cardiac deaths. Of these patients, myocardial T2* at first scan had been less than 10ms in 85.7% and less than 20ms in 97.1%. Therefore, in this worldwide cohort of thalassemia major patients, over 43% had moderate/severe myocardial iron loading with significant geographical differences, and myocardial T2* values less than 10ms were strongly associated with heart failure and death. © 2013 Ferrata Storti Foundation.


Murad H.,Syrian Atomic Energy Commission | Moassas F.,Syrian Atomic Energy Commission | Jarjour R.,Syrian Atomic Energy Commission | Mukhalalaty Y.,Thalassemia Center | Al-Achkar W.,Syrian Atomic Energy Commission
Hemoglobin | Year: 2014

Our objective was to evaluate the prenatal diagnosis (PND) of β-thalassemia (β-thal) and sickle cell anemia in Syria. Mutations detected from blood of at-risk couples and 55 amniotic fluid samples collected at the second trimester of pregnancy (14-22 weeks' gestation) were characterized. Molecular screening and direct DNA sequencing of the HBB gene was carried out. DNA analyses showed 14 affected fetuses (25.45%), 32 (58.18%) carriers and eight (14.54%) normal fetuses. It appears that 20.0% of individuals carried the sickle cell anemia mutation and 80.0% carried the β-thal mutation. Thirteen different known mutations were detected in the fetuses. The most common mutations were: IVS-II-1 (G>A), codon 39 (C>T)], IVS-I-110 (G>A), IVS-I-1 (G>A) and IVS-I-5 (G>C). The Hb S [β6(A3)Glu→Val; HBB: c.20A>T] mutation was the only abnormal hemoglobin (Hb) that was found. The results point to a successful future for PND of β-thal and sickle cell anemia in Syria, using a rapid and accurate molecular method. We hope that this method will be used as a common application approach to decrease the incidence of β-thal major (β-TM). © 2014 Informa Healthcare USA, Inc. All rights reserved.


Samimi-Rad K.,Tehran University of Medical Sciences | Asgari F.,Centers for Disease Control and Prevention | Nasiritoosi M.,Tehran University of Medical Sciences | Esteghamati A.,Tehran University of Medical Sciences | And 6 more authors.
Hepatitis Monthly | Year: 2013

Background: Hepatitis C is prevalent among thalassemia patients in Iran. It is mainly transfusion mediated, in particular among patients treated before 1996 when blood screening was introduced. Objectives: The current study aimed to investigate why patients still seroconvert to anti-HCV in Iranian thalassemia centers. Patients and Methods: During 2006-2007 sera were sampled from 217 anti-HCV positive thalassemia patients at nine thalassemia centers in Tehran and Amol city, where 34 (16%) patients had been infected after 1996. The HCV subtype could be determined by sequencing and phylogenetic analysis of partial NS5B and/or 5?NCR-core region in 130 strains. Results: 1a (53%) was predominant followed by 3a (30%), 1b (15%), and one strain each of 2k, 3k and 4a. Phylogenetic analysis revealed 19 clades with up to five strains diverging with less than six nucleotides from each other within subtypes 1a and 3a. Strains in seven clades were from nine patients infected between 1999 and 2005 and similar to strains from eight patients infected before 1996, indicating ongoing transmission at the centers. Further epidemiological investigation revealed that 28 patients infected with strains within the same clade had frequently been transfused at the same shift sitting on the same bed. An additional eight patients with related strains had frequently been transfused simultaneously in the same room. Conclusions: The results suggest nosocomial transmission at these thalassemia centers both before and after the introduction of blood screening. Further training of staff and strict adherence to preventive measures are thus essential to reduce the incidence of new HCV infections. © 2013, Kowsar Corp.


Wankhade V.,University of Pune | Andhale R.B.,University of Pune | Lodha S.,Thalassemia Center
Trends in Medical Research | Year: 2013

In the present study, some cellular and crystalline components of urine of Sickle Cell Anemia (SCA) were studied. In total 67 samples were investigated. The normal and abnormal cellular components like presence of Red blood cells, White blood cells Epithelial cells, Renal tubules cells, Bacteria, Yeast and Protozoan were observed and counted. Noncellular components/crystals like Tyrosine, Cholesterol, A/T Phosphate, Leucine, B-Granule, Uric Acid, Oxalate and Cystine were counted. It was observed that RBCs, WBCs and Epithelial cells, Renal tubules cells, Bacteria, Yeast and Protozoan were in high number indicating various complications occurring in the body of sickle cell anemic patients. It was observed that RBCs were 13918.18/cumm±SE 4057.90 indicating tremendous hematourea, number of WBC were 224.59±50.002 SE, Sqaumous Epithelial cells were 35.68±10.989 SE, Renal cells were 110.68±74.15 SE. This indicates that SCA patients suffer from kidney damage, hematourea, Urinary Tract Inflammation, Interstitial nephritis, Glomerulonephritis and Pylonephritis. © 2013 Academic Journals Inc.


PubMed | Thalassemia Center, Tehran University of Medical Sciences and Payame Noor University
Type: Journal Article | Journal: Archives of virology | Year: 2016

Occult hepatitis C virus infection (OCI) is a new pathological form of chronic hepatitis virus (HCV) infection characterized by the presence of HCV RNA in liver biopsy and/or peripheral blood mononuclear cell (PBMC) specimens and the absence of HCV RNA and anti-HCV antibodies (Abs) in plasma samples. -thalassemia major is a hereditary recessive blood disease with deficiency in the hemoglobin beta chain. Thalassemic patients need blood transfusion therapy; repeated blood transfusion increases the risk of viral blood-borne infection. The aim of this study was to determine the prevalence of OCI in Iranian patients with -thalassemia major. From February 2015 to November 2015, a total of 147 Iranian patients with -thalassemia major were enrolled in this cross-sectional study. After extraction of viral RNA from the plasma and PBMC samples, HCV genomic RNA in the specimens was amplified by RT-nested PCR using primers from the 5-UTR. The HCV genotypes of the positive specimens were tested using the RFLP assay. To confirm the HCV genotypes, the 5-UTR fragment was amplified and cloned into the pJET1.2/blunt cloning vector and then sequenced. Out of 147 patients, 106 (72.1%) were negative for anti-HCV Abs and HCV RNA. HCV RNA was found in PBMC specimens of six (5.7%) patients, from a total of 106 patients with undetectable plasma HCV RNA and anti-HCV Abs. Therefore, six out of 106 patients had OCI. HCV genotyping revealed that three patients were infected with HCV subtype 1b, two patients were infected with HCV subtype 3a, and one patient was infected with HCV subtype 1a. These results revealed that Iranian patients with beta-thalassemia major might have OCI. Therefore, it seems that the design of a study to identify this infection in patients with -thalassemia major would provide valuable information.

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