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https://www.readbyqxmd.com/read/28801997/prevalence-of-low-bone-mass-among-adolescents-with-nontransfusion-dependent-hemoglobin-e-%C3%AE-thalassemia-and-its-relationship-with-anemia-severity
#1
Pairunyar Nakavachara, Jaturat Petchkul, Krittha Jeerawongpanich, Pornpimol Kiattisakthavee, Teerarat Manpayak, Parichat Netsakulnee, Katharee Chaichanwattanakul, Julaporn Pooliam, Somdet Srichairatanakool, Vip Viprakasit
BACKGROUND: Low bone mass is common among adolescents with transfusion-dependent β-thalassemia despite adequate transfusion and iron chelation. However, there are few reports regarding bone mineral density (BMD) among adolescents with nontransfusion-dependent thalassemia (NTDT). Indeed, only BMD data in patients with nontransfusion-dependent (NTD) β-thalassemia intermedia have been reported. No previous study has investigated BMD among adolescents with NTD hemoglobin (Hb) E/β-thalassemia...
August 12, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28759343/epidemiologic-study-of-major-complications-in-adolescent-and-adult-patients-with-thalassemia-in-northeastern-thailand-the-e-saan-study-phase-i
#2
Nattiya Teawtrakul, Arunee Jetsrisuparb, Saranya Pongudom, Chittima Sirijerachai, Kanchana Chansung, Chinadol Wanitpongpun, Supan Fucharoen
INTRODUCTION: Thalassemia-related complications are one of the main factors that increase morbidity and mortality in aging patients with thalassemia. This study was aimed to report the prevalence and clinical risk factors for the complications in thalassemia. METHODS: A multi-center prospective cohort study was conducted in patients with thalassemia aged ≥10 years old. Thalassemia-related complications were heart failure, pulmonary hypertension, extramedullary hematopoiesis, endocrine disorders, infections, thrombosis and leg ulcers...
July 31, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28707012/soluble-form-of-transferrin-receptor-1-level-is-associated-with-the-age-at-first-diagnosis-and-the-risk-of-therapeutic-intervention-and-iron-overloading-in-patients-with-non-transfusion-dependent-thalassemia
#3
Paolo Ricchi, Antonella Meloni, Silvia Costantini, Anna Spasiano, Tiziana Di Matola, Alessia Pepe, Patrizia Cinque, Aldo Filosa
We retrospectively evaluated the relationship between serum transferrin receptor-1 (sTfR1) and some fundamental events in the life and the management (the age at diagnosis, the age at the first red blood cells transfusion, the age at splenectomy, and the overall need of chelation therapy) of 111 patients with non-transfusion-dependent thalassemia (NTDT) subdivided in four genetic entities: patients with homozygous or compound heterozygous state for β-thalassemia, patients with triplicated α genotype associated with β heterozygosity, patients with deletional HbH, and patients with the combination of a β defect plus a β chain variant...
September 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28633041/study-of-platelet-activation-hypercoagulable-state-and-the-association-with-pulmonary-hypertension-in-children-with-%C3%AE-thalassemia
#4
REVIEW
Mahmoud Alhosiny Fayed, Hesham El-Sayed Abdel-Hady, Mona Mohammed Hafez, Osama Saad Salama, Youssef Abdelhalim Al-Tonbary
BACKGROUND: The increased survival rate of thalassemic patients has led to unmasking of management related complications which were infrequently encountered. OBJECTIVE: Study the increased coagulation and platelet activation in children with β-thalassemia, to analyze the factors that lead to such hypercoagulable state and to study pulmonary hypertension (PH) in conjunction with platelet activation and hypercoagulable state in children with β-thalassemia. METHODS: 36 Egyptian children with β-thalassemia with a mean age of 9...
June 15, 2017: Hematology/oncology and Stem Cell Therapy
https://www.readbyqxmd.com/read/28561822/synthesis-characterization-and-oxygen-atom-transfer-reactivity-of-a-pair-of-mo-iv-o-and-mo-vi-o2-enedithiolate-complexes-a-look-at-both-ends-of-the-catalytic-transformation
#5
Ashta C Ghosh, Prinson P Samuel, Carola Schulzke
Two new molybdenum complexes (Bu4N)2[Mo(IV)O(ntdt)2] (1) and (Ph4P)2[Mo(VI)O2(ntdt)2] (2) (ntdt = 2-naphthyl-1,4-dithiolate) were synthesized using asymmetric dithiolene precursors and were characterized as structural models for the active site of arsenite oxidase, a molybdopterin bearing enzyme. The ligand was obtained readily by a two-step synthesis starting from 2-bromo-2'-acetonapthone. Complexes 1 and 2 were obtained by reaction of the resulting 4-naphthyl-1,3-dithiol-2-one with metal precursors trans-[MoO2(CN)4](4-) and cis-[MoO2(NCS)4](2-) respectively...
June 13, 2017: Dalton Transactions: An International Journal of Inorganic Chemistry
https://www.readbyqxmd.com/read/28405327/iron-overload-correlates-with-serum-liver-fibrotic-markers-and-liver-dysfunction-potential-new-methods-to-predict-iron-overload-related-liver-fibrosis-in-thalassemia-patients
#6
Man Wang, Rongrong Liu, Yuzhen Liang, Gaohui Yang, Yumei Huang, Chunlan Yu, Kaiqi Sun, Yongrong Lai, Yang Xia
BACKGROUND: Early detection of liver fibrosis in thalassemia patients and rapid initiation of treatment to interfere with its progression are extremely important. OBJECTIVE: This study aimed to find a sensitive, easy-to-detect and noninvasive method other than liver biopsy for early detection of liver fibrosis in thalassemia patients. METHODS: A total of 244 Chinese Thalassemia patients with non-transfusion-dependent thalassemia (NTDT, n = 105) or thalassemia major (TM, n = 139) and 120 healthy individuals were recruited into the present study, and blood collagen type IV (C IV), precollagen type III (PIIINPC) and hyaluronic acid (HA), aspartate aminotransferase (AST), alanine aminotransferase (ALT) and ferritin were measured...
February 2017: United European Gastroenterology Journal
https://www.readbyqxmd.com/read/27917462/iron-overload-across-the-spectrum-of-non-transfusion-dependent-thalassaemias-role-of-erythropoiesis-splenectomy-and-transfusions
#7
RANDOMIZED CONTROLLED TRIAL
John B Porter, Maria Domenica Cappellini, Antonis Kattamis, Vip Viprakasit, Khaled M Musallam, Zewen Zhu, Ali T Taher
Non-transfusion-dependent thalassaemias (NTDT) encompass a spectrum of anaemias rarely requiring blood transfusions. Increased iron absorption, driven by hepcidin suppression secondary to erythron expansion, initially causes intrahepatic iron overload. We examined iron metabolism biomarkers in 166 NTDT patients with β thalassaemia intermedia (n = 95), haemoglobin (Hb) E/β thalassaemia (n = 49) and Hb H syndromes (n = 22). Liver iron concentration (LIC), serum ferritin (SF), transferrin saturation (TfSat) and non-transferrin-bound iron (NTBI) were elevated and correlated across diagnostic subgroups...
January 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/27710960/molecular-understanding-of-non-transfusion-dependent-thalassemia-associated-with-hemoglobin-e-%C3%AE-thalassemia-in-northeast-thailand
#8
Supawadee Yamsri, Naruwat Pakdee, Goonnapa Fucharoen, Kanokwan Sanchaisuriya, Supan Fucharoen
Non-transfusion-dependent thalassemia (NTDT) is associated with various forms of thalassemia and genetic modifiers. We report the molecular basis of NTDT in hemoglobin (Hb) E-β-thalassemia disease. This study was done in 73 adult patients encountered at the prenatal diagnosis center of Khon Kaen University, Northeast Thailand. Hematological parameters and Hb patterns were collected, and α- and β-globin gene mutations were determined. Multiple single-nucleotide polymorphisms (SNPs) including the rs7482144/Gγ-XmnI polymorphism, rs2297339, rs2838513, rs4895441, and rs9399137 in the HBS1L-MYB gene, rs4671393 and rs11886868 in the BCL11A gene, and G176AfsX179 in the KLF1 gene were examined...
2016: Acta Haematologica
https://www.readbyqxmd.com/read/27186941/morbidities-in-non-transfusion-dependent-thalassemia
#9
REVIEW
Antoine N Saliba, Ali T Taher
Patients with non-transfusion-dependent thalassemia (NTDT) experience a wide array of clinical complications despite their independence from frequent, regular red blood cell (RBC) transfusions. According to the current understanding of NTDT, these clinical complications stem from the interaction of multiple pathophysiological factors: ineffective erythropoiesis, iron overload, and hypercoagulability. The state of chronic anemia and hypoxia-resulting from ineffective erythropoiesis and hemolysis-leads to the expansion of the erythroid marrow and extramedullary hematopoiesis...
March 2016: Annals of the New York Academy of Sciences
https://www.readbyqxmd.com/read/27108419/cortical-bone-invasion-in-non-transfusion-dependent-thalassemia-tumefactive-extramedullary-hematopoiesis-reviewed
#10
Karim Z Masrouha, Joelle Wazen, Anthony Haddad, Fadi Saadeh, Ali Taher, Nabil J Khoury
OBJECTIVE OF THE STUDY: To assess the prevalence of cortical bone invasion (CBI) with secondary extramedullary hematopoiesis (EMH) in patients with non-transfusion-dependent thalassemia (NTDT), to determine its predilection sites on thoracic and abdominal imaging, to determine whether there is an association between various clinical and hematological parameters, and to evaluate its various findings mainly on magnetic resonance imaging (MRI), in addition to computed tomography (CT) scans...
August 2016: La Radiologia Medica
https://www.readbyqxmd.com/read/27052211/iron-overload-in-non-transfusion-dependent-thalassemia-association-with-genotype-and-clinical-risk-factors
#11
Adisak Tantiworawit, Pimlak Charoenkwan, Sasinee Hantrakool, Worawut Choeyprasert, Chate Sivasomboon, Torpong Sanguansermsri
In the present study, we sought to determine the prevalence of iron overload in patients with non-transfusion-dependent thalassemia (NTDT) and its association with genotype and other clinical risk factors, and to evaluate the correlation between serum ferritin (SF) and liver iron concentration (LIC). Myocardial and liver iron concentration was measured by MRI using a T2* gradient multi-echo sequence in NTDT patients, aged 10-50 years. Of 91 patients, 54 (59 %) had hepatic iron overload. None had cardiac iron overload...
June 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/26852651/optimising-iron-chelation-therapy-with-deferasirox-for-non-transfusion-dependent-thalassaemia-patients-1-year-results-from-the-thetis-study
#12
MULTICENTER STUDY
Ali T Taher, M Domenica Cappellini, Yesim Aydinok, John B Porter, Zeynep Karakas, Vip Viprakasit, Noppadol Siritanaratkul, Antonis Kattamis, Candace Wang, Zewen Zhu, Victor Joaquin, Marie José Uwamahoro, Yong-Rong Lai
Efficacy and safety of iron chelation therapy with deferasirox in iron-overloaded non-transfusion-dependent thalassaemia (NTDT) patients were established in the THALASSA study. THETIS, an open-label, single-arm, multicentre, Phase IV study, added to this evidence by investigating earlier dose escalation by baseline liver iron concentration (LIC) (week 4: escalation according to baseline LIC; week 24: adjustment according to LIC response, maximum 30mg/kg/day). The primary efficacy endpoint was absolute change in LIC from baseline to week 52...
March 2016: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/26810455/the-long-term-and-extensive-efficacy-of-low-dose-thalidomide-in-a-case-of-an-untransfusable-patient-with-non-transfusion-dependent-thalassemia
#13
Paolo Ricchi, Silvia Costantini, Anna Spasiano, Gianfranco De Dominicis, Tiziana Di Matola, Patrizia Cinque, Massimiliano Ammirabile, Maria Marsella, Aldo Filosa
Patients with Non-Transfusion-Dependent Thalassemia may require regular transfusion therapy. However, these patients are at risk of developing irregular antibodies, making them untransfusable. Second line treatment usually includes hydroxyurea, which however is not effective in all patients. Other treatment options include thalidomide, which has been reported to be safe and effective in selected patients. We report the case of a patient who experienced improvement of hemoglobin levels and of a part of NTDT related complications, following 36months of continuous therapy with low doses of thalidomide...
March 2016: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/26664743/prevalence-and-risk-factors-for-complications-in-patients-with-nontransfusion-dependent-alpha-and-beta-thalassemia
#14
Poramed Winichakoon, Adisak Tantiworawit, Thanawat Rattanathammethee, Sasinee Hantrakool, Chatree Chai-Adisaksopha, Ekarat Rattarittamrong, Lalita Norasetthada, Pimlak Charoenkwan
Background. Nontransfusion dependent thalassemia (NTDT) is a milder form of thalassemia that does not require regular transfusion. It is associated with many complications, which differ from that found in transfusion-dependent thalassemia (TDT). Currently available information is mostly derived from beta-NTDT; consequently, more data is needed to describe complications found in the alpha-NTDT form of this disease. Methods. We retrospectively reviewed the medical records of NTDT patients from January 2012 to December 2013...
2015: Anemia
https://www.readbyqxmd.com/read/26545830/development-of-a-new-disease-severity-scoring-system-for-patients-with-non-transfusion-dependent-thalassemia
#15
M Domenica Cappellini, John B Porter, Khaled M Musallam, Antonis Kattamis, Vip Viprakasit, Renzo Galanello, Ali T Taher
BACKGROUND: Patients with non-transfusion-dependent thalassemia (NTDT) present with a spectrum of disease severities. Since there are multiple pathophysiologies in such patients, tailoring treatment remains essential. Therefore, one simple, reliable tool would be beneficial to assess disease severity and tailor therapy, particularly for internal medicine specialists who may treat a variety of NTDT patients with a multitude of complications. This would allow for standardization of assessments leading to timely interventions and prevention of complications...
March 2016: European Journal of Internal Medicine
https://www.readbyqxmd.com/read/26479125/non-transfusion-dependent-thalassemia-and-thalassemia-intermedia-epidemiology-complications-and-management
#16
REVIEW
Elliott Vichinsky
OBJECTIVE: The non-transfusion-dependent thalassemias (NTDTs), including thalassemia intermedia (TI), hemoglobin E beta thalassemia, and hemoglobin H disease, have sometimes been regarded as less severe than their transfusion-dependent variants; however, these disorders carry a substantial disease burden (e.g., splenomegaly, iron overload, skeletal effects, and cardiopulmonary disease). The aim of this review is to increase clinician awareness of the growing global problem of NTDT and TI, and discuss the current management strategies for these conditions...
2016: Current Medical Research and Opinion
https://www.readbyqxmd.com/read/26460260/study-of-alpha-hemoglobin-stabilizing-protein-expression-in-patients-with-%C3%AE-thalassemia-and-sickle-cell-anemia-and-its-impact-on-clinical-severity
#17
Hanan Mohamed Mahmoud, Ahmed Al-Saiid Hamed Shoeib, Shereen Mohamed Abd El Ghany, Marwa Mohamed Reda, Iman Ahmed Ragab
UNLABELLED: The α hemoglobin stabilizing protein (AHSP) binds α-Hb and prevents its precipitation limiting free α-Hb toxicities. Our aim was to study AHSP expression in β thalassemia syndromes in relation to their clinical severity and to compare it with its level in sickle cell anemia. We compared patients with β-thalassemia (n=37) (β-thalassemia major (BTM) (n=19) and β-thalassemia intermedia (BTI) (n=18)) with 12 patients with sickle cell anemia as regards clinical severity, age at presentation, transfusion dependency, mean pre-transfusion hemoglobin level, use of hydroxyurea and AHSP expression by real time quantitative PCR...
December 2015: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/26406992/tissue-iron-distribution-assessed-by-mri-in-patients-with-iron-loading-anemias
#18
Lucía Gutiérrez, Michael J House, Nisha Vasavda, Emma Drašar, Isabel Gonzalez-Gascon Y Marin, Austin G Kulasekararaj, Tim G St Pierre, Swee L Thein
Bone marrow, spleen, liver and kidney proton transverse relaxation rates (R2), together with cardiac R2* from patients with sickle cell disease (SCD), paroxysmal nocturnal hemoglobinuria (PNH) and non-transfusion dependent thalassemia (NTDT) have been compared with a control group. Increased liver and bone marrow R2 values for the three groups of patients in comparison with the controls have been found. SCD and PNH patients also present an increased spleen R2 in comparison with the controls. The simultaneous measurement of R2 values for several tissue types by magnetic resonance imaging (MRI) has allowed the identification of iron distribution patterns in diseases associated with iron imbalance...
2015: PloS One
https://www.readbyqxmd.com/read/26358855/severe-bacterial-infections-in-patients-with-non-transfusion-dependent-thalassemia-prevalence-and-clinical-risk-factors
#19
Nattiya Teawtrakul, Arunee Jetsrisuparb, Chittima Sirijerachai, Kanchana Chansung, Chinadol Wanitpongpun
INTRODUCTION: Bacterial infection is one of the major causes of death in patients with thalassemia. Clinical predictive factors for severe bacterial infection were evaluated in patients with non-transfusion-dependent thalassemia (NTDT). METHODS: A retrospective study was conducted of patients with NTDT aged ≥ 10 years at Srinagarind Hospital, Khon Kaen University, Thailand. Clinical characteristics and potential clinical risk factors for bacterial infection were collected...
October 2015: International Journal of Infectious Diseases: IJID
https://www.readbyqxmd.com/read/26303193/phenotype-and-genotype-in-a-cohort-of-312-adult-patients-with-nontransfusion-dependent-thalassemia-in-northeast-thailand
#20
MULTICENTER STUDY
Patcharawadee Prayalaw, Nattiya Teawtrakul, Arunee Jetsrisuparb, Saranya Pongudom, Goonnapa Fucharoen, Supan Fucharoen
Patients with nontransfusion-dependent thalassemia (NTDT) do not require regular blood transfusion for survival but may encounter several complications that contribute to morbidity and mortality. We report the molecular heterogeneity and hematological features of NTDT in 312 adult patients in northeast Thailand. Hemoglobin (Hb) and DNA analyses identified 177 subjects with Hb E-β-thalassemia, 1 with homozygous β0-thalassemia and 134 with Hb H, AEBart's and EEBart's diseases. For β-thalassemia, 12 different mutations including both β0- and β+-thalassemias were detected...
2016: Acta Haematologica
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