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https://www.readbyqxmd.com/read/29543340/plgf-and-sflt-1-levels-in-patients-with-non-transfusion-dependent-thalassemia-correlations-with-markers-of-iron-burden-and-endothelial-dysfunction
#1
Charikleia Kelaidi, Antonis Kattamis, Filia Apostolakou, Christos Poziopoulos, Christina Lazaropoulou, Polyxeni Delaporta, Ino Kanavaki, Ioannis Papassotiriou
BACKGROUND: Levels of the angiogenic cytokines Placental Growth Factor (PlGF) and soluble Fms-Like Tyrosine Kinase-1 (sFlt-1) and the angiogenic balance, expressed by sFlt-1/PlGF ratio, are perturbed in Sickle Cell Disease and iron overload, but they have not been evaluated in Non-Transfusion-Dependent Thalassemia (NTDT). PATIENTS AND METHODS: We measured levels of PlGF, sFlt-1 and vWF:antigen in patients with NTDT of beta thalassemia genotype, and correlated them with erythrocytic indices and markers of iron overload, inflammation and tissue hypoxia...
March 15, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29458728/clinical-complications-and-their-management
#2
REVIEW
Alessia Marcon, Irene Motta, Ali T Taher, Maria Domenica Cappellini
The hallmarks of thalassemias are ineffective erythropoiesis and peripheral hemolysis leading to a cascade of events responsible for several clinical complications. This pathophysiologic mechanism can be partially controlled by blood transfusions or by correction of the severity of ineffective erythropoiesis. Thalassemias include a spectrum of phenotypes. Two main groups can be clinically distinguished: transfusion-dependent (TDT) and non-transfusion-dependent (NTDT) thalassemia. Both conditions are characterized by several clinical complications along life; some are shared, whereas some have higher prevalence in one group over the other...
April 2018: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/29363758/the-impact-of-liver-steatosis-on-the-ability-of-serum-ferritin-levels-to-be-predictive-of-liver-iron-concentration-in-non-transfusion-dependent-thalassaemia-patients
#3
Paolo Ricchi, Antonella Meloni, Anna Spasiano, Silvia Costantini, Alessia Pepe, Patrizia Cinque, Aldo Filosa
This study analysed the impact of liver steatosis (LS) on the parameters of iron overload in 110 patients with non-transfusion dependent thalassaemia (NTDT). LS was diagnosed by ultrasound. Liver iron concentration (LIC) measurements were available for 64 patients who underwent a magnetic resonance imaging (MRI) scan. LS was frequent (35·5%) and was significantly more prevalent in males than in females (49·0% vs. 24·6%, P = 0·008). Patients with LS had significant higher levels of alanine aminotransferase (ALT), aspartate aminotransferase (AST), ALT/AST ratio and ferritin than those without, but LIC values were comparable...
March 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29359464/a-prospective-analysis-for-prevalence-of-complications-in-thai-nontransfusion-dependent-hb-e-%C3%AE-thalassemia-and-%C3%AE-thalassemia-hb-h-disease
#4
Supachai Ekwattanakit, Noppadol Siritanaratkul, Vip Viprakasit
Recently, complications in patients with nontransfusion-dependent thalassemia (NTDT), in particular those with β-thalassemia intermedia (β-TI), were found to be significantly different from those in patients with transfusion dependent thalassemia (TDT), mainly β-thalassemia major (β-TM). However, this information is rather limited in other forms of NTDT. In this prospective study, adult Thai NTDT patients were interviewed and clinically evaluated for thalassemia related complications. Fifty-seven NTDT patients (age 18-74 years), 59...
May 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/29316681/non-transfusion-dependent-thalassemia-an-update-on-complications-and-management
#5
REVIEW
Joseph Sleiman, Ali Tarhini, Rayan Bou-Fakhredin, Antoine N Saliba, Maria Domenica Cappellini, Ali T Taher
Patients with non-transfusion-dependent thalassemia (NTDT) experience many clinical complications despite their independence from frequent transfusions. Morbidities in NTDT stem from the interaction of multiple pathophysiological factors: ineffective erythropoiesis, iron overload (IOL), and hypercoagulability. Ineffective erythropoiesis and hemolysis are associated with chronic hypoxia and a hypercoagulable state. The latter are linked to a high prevalence of thromboembolic and cerebrovascular events, as well as leg ulcers and pulmonary hypertension...
January 8, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29261151/iron-overload-and-chelation-therapy-in-non-transfusion-dependent-thalassemia
#6
REVIEW
Rayan Bou-Fakhredin, Abdul-Hamid Bazarbachi, Bachar Chaya, Joseph Sleiman, Maria Domenica Cappellini, Ali T Taher
Iron overload (IOL) due to increased intestinal iron absorption constitutes a major clinical problem in patients with non-transfusion-dependent thalassemia (NTDT), which is a cumulative process with advancing age. Current models for iron metabolism in patients with NTDT suggest that suppression of serum hepcidin leads to an increase in iron absorption and subsequent release of iron from the reticuloendothelial system, leading to depletion of macrophage iron, relatively low levels of serum ferritin, and liver iron loading...
December 20, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29244749/serum-levels-of-s100b-and-nse-proteins-in-patients-with-non-transfusion-dependent-thalassemia-as-biomarkers-of-brain-ischemia-and-cerebral-vasculopathy
#7
Aikaterini Kanavaki, Konstantinos Spengos, Maria Moraki, Polyxeni Delaporta, Catherine Kariyannis, Ioannis Papassotiriou, Antonis Kattamis
Patients with non-transfusion-dependent thalassemia (NTDT) are at risk of developing brain ischemia. Transcranial Doppler (TCD) has been established as a useful screening tool of cerebrovascular disease in patients with sickle cell disease. Proteins neuron specific enolase (NSE) and S100B are biomarkers that reflect CNS injury. The purpose of this study is to evaluate cerebral vessel vasculopathy and brain damage in NTDT patients using non-invasive methods as TCD and measurement serum levels of NSE and S100B...
December 15, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29222267/new-therapeutic-targets-in-transfusion-dependent-and-independent-thalassemia
#8
REVIEW
M Domenica Cappellini, Irene Motta
β-Thalassemias are characterized by reduced production of β-globin chain, resulting in α/β-chain unbalance and precipitation of α-globin-heme complexes and determining ineffective erythropoiesis. Ineffective erythropoiesis, chronic hemolytic anemia, and compensatory hematopoietic expansion are the disease hallmarks, and they are related to the severity of the chain unbalance. Several clinical forms of β-thalassemia, including the coinheritance of β-thalassemia with hemoglobin E resulting in hemoglobin E/β-thalassemia, have been described...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29222265/iron-overload-in-thalassemia-different-organs-at-different-rates
#9
REVIEW
Ali T Taher, Antoine N Saliba
Thalassemic disorders lie on a phenotypic spectrum of clinical severity that depends on the severity of the globin gene mutation and coinheritance of other genetic determinants. Iron overload is associated with increased morbidity in both patients with transfusion-dependent thalassemia (TDT) and non-transfusion-dependent thalassemia (NTDT). The predominant mechanisms driving the process of iron loading include increased iron burden secondary to transfusion therapy in TDT and enhanced intestinal absorption secondary to ineffective erythropoiesis and hepcidin suppression in NTDT...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
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
#10
MULTICENTER STUDY
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...
January 2018: 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
#11
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...
January 2018: 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
#12
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
#13
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-o-2-enedithiolate-complexes-a-look-at-both-ends-of-the-catalytic-transformation
#14
Ashta C Ghosh, Prinson P Samuel, Carola Schulzke
Two new molybdenum complexes (Bu4 N)2 [MoIV O(ntdt)2 ] (1) and (Ph4 P)2 [MoVI 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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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