Database : MEDLINE
Search on : thalassemia [Words]
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[PMID]: 29428552
[Au] Autor:De Luna G; Ranque B; Courbebaisse M; Ribeil JA; Khimoud D; Dupeux S; Silvera J; Offredo L; Pouchot J; Arlet JB
[Ad] Address:Internal Medicine Department, Sickle Cell Referral Center, Georges Pompidou European Hospital, AP-HP, Paris, France; Faculté de Médecine Paris Descartes, Sorbonne Paris-Cité, Paris, France. Electronic address: gonzalo.deluna@aphp.fr.
[Ti] Title:High bone mineral density in sickle cell disease: Prevalence and characteristics.
[So] Source:Bone;110:199-203, 2018 Feb 08.
[Is] ISSN:1873-2763
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:BACKGROUND: Osteosclerosis (OSC) is a rarely studied complication of sickle cell disease (SCD). The objective of our study was to determine the prevalence and characteristics of high bone mineral density (BMD) and its radiological features in adult SCD patients. METHODS: This prospective observational study was conducted from May 2007 to May 2016 in consecutive patients with steady-state SCD at two university hospitals. The BMD of the lumbar spine (L1-L4) and right femoral neck was determined by dual energy X-ray absorptiometry. Clinical, laboratory and radiographic data were recorded. High BMD was defined as a BMD Z-score of at least +2.5 standard deviations at the lumbar spine or hip. The characteristics of the patients with high BMD were compared to those of individuals with low or middle BMD, using multivariate ordinal logistic regression. RESULTS: 135 patients (86 women and 49 men) with a median age of 27 (IQR 23-33) years were included. High BMD was diagnosed in 20 (15%) patients with a median age of 33.5 (IQR 28-45) years. The SCD genotypes of these patients were SS in 11, SC in 5, S/beta+ in 3, and S/beta0 in 1. High BMD patients more frequently harbored the S/beta SCD genotype (21% vs 5% in non-high BMD patients; p=0.047) and were older (p=0.0007). Compared to patients with low or middle BMD, after adjustment for age and SCD genotype, high BMD patients had a higher prevalence of avascular necrosis history (p=0.009), higher BMI (p=0.007), and lower serum resorption marker CTX (p=0.04), bilirubin (p=0.02) and parathyroid hormone levels (p=0.02). There were no differences between groups regarding fracture history, H-shaped vertebrae or other biological variables. CONCLUSION: High-BMD values is a common manifestation in SCD patients, especially in those with the S/beta-thalassemia genotypes. The prevalence of high-BMD in SCD is associated with older age, suggesting that it will be more common in the future because the life span of patients with SCD is increasing thanks to significant progress in SCD treatment.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180310
[Lr] Last revision date:180310
[St] Status:Publisher

  2 / 21559 MEDLINE  
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[PMID]: 29523504
[Au] Autor:Casu C; Nemeth E; Rivella S
[Ad] Address:Department of Pediatrics, Division of Hematology, Children's Hospital of Philadelphia, United States.
[Ti] Title:Hepcidin agonists as therapeutic tools.
[So] Source:Blood;, 2018 Mar 09.
[Is] ISSN:1528-0020
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Hepcidin agonists are a new class of compounds that regulate blood iron levels and limit iron absorption, and could improve the treatment of hemochromatosis, ß-thalassemia, polycythemia vera, and other disorders where disrupted iron homeostasis causes disease or contributes to it. Hepcidin agonists also have the potential to prevent severe complications of siderophilic infections in patients with iron overload or chronic liver disease. This review highlights the preclinical studies that support the development of hepcidin agonists for the treatment of these disorders.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180310
[Lr] Last revision date:180310
[St] Status:Publisher

  3 / 21559 MEDLINE  
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[PMID]: 29318368
[Au] Autor:Mirlohi MS; Yaghooti H; Shirali S; Aminasnafi A; Olapour S
[Ad] Address:Hyperlipidemia Research Center, School of Allied Medical Sciences, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran.
[Ti] Title:Increased levels of advanced glycation end products positively correlate with iron overload and oxidative stress markers in patients with ß-thalassemia major.
[So] Source:Ann Hematol;97(4):679-684, 2018 Apr.
[Is] ISSN:1432-0584
[Cp] Country of publication:Germany
[La] Language:eng
[Ab] Abstract:The impaired biosynthesis of the ß-globin chain in ß-thalassemia leads to the accumulation of unpaired alpha globin chains, failure in hemoglobin formation, and iron overload due to frequent blood transfusion. Iron excess causes oxidative stress and massive tissue injuries. Advanced glycation end products (AGEs) are harmful agents, and their production accelerates in oxidative conditions. This study was conducted on 45 patients with major ß-thalassemia who received frequent blood transfusions and chelation therapy and were compared to 40 healthy subjects. Metabolic parameters including glycemic and iron indices, hepatic and renal functions tests, oxidative stress markers, and AGEs (carboxymethyl-lysine and pentosidine) levels were measured. All parameters were significantly increased in ß-thalassemia compared to the control except for glutathione levels. Blood glucose, iron, serum ferritin, non-transferrin-bound iron (NTBI), MDA, soluble form of low-density lipoprotein receptor, glutathione peroxidase, total reactive oxygen species (ROS), and AGE levels were significantly higher in the ß-thalassemia patients. Iron and ferritin showed a significant positive correlation with pentosidine (P < 0.01) but not with carboxymethyl-lysine. The NTBI was markedly increased in the ß-thalassemia patients, and its levels correlated significantly with both carboxymethyl-lysine and pentosidine (P < 0.05). Our findings confirm the oxidative status generated by the iron overload in ß-thalassemia major patients and highlight the enhanced formation of AGEs, which may play an important role in the pathogenesis of ß-thalassemia major.
[Mh] MeSH terms primary: Blood Transfusion
Glycation End Products, Advanced/blood
Iron Overload/etiology
Oxidative Stress
Transfusion Reaction/physiopathology
beta-Thalassemia/blood
[Mh] MeSH terms secundary: Adolescent
Adult
Biomarkers/blood
Chelation Therapy/adverse effects
Combined Modality Therapy/adverse effects
Cross-Sectional Studies
Deferoxamine/therapeutic use
Female
Humans
Iran
Iron Overload/prevention & control
Male
Pyridones/therapeutic use
Scavenger Receptors, Class E/blood
Young Adult
beta-Thalassemia/therapy
[Pt] Publication type:JOURNAL ARTICLE
[Nm] Name of substance:0 (Biomarkers); 0 (Glycation End Products, Advanced); 0 (OLR1 protein, human); 0 (Pyridones); 0 (Scavenger Receptors, Class E); 2BTY8KH53L (deferiprone); J06Y7MXW4D (Deferoxamine)
[Em] Entry month:1803
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[Js] Journal subset:IM
[Da] Date of entry for processing:180111
[St] Status:MEDLINE
[do] DOI:10.1007/s00277-017-3223-3

  4 / 21559 MEDLINE  
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[PMID]: 29521164
[Au] Autor:Carlos AM; Souza BMB; Souza RAV; Resende GAD; Pereira GA; Moraes-Souza H
[Ad] Address:a Department of Medicine , Universidade Federal do Triângulo Mineiro (UFTM) , Uberaba , Minas Gerais , Brazil.
[Ti] Title:Causes of microcytic anaemia and evaluation of conventional laboratory parameters in the differentiation of erythrocytic microcytosis in blood donors candidates.
[So] Source:Hematology;:1-7, 2018 Mar 09.
[Is] ISSN:1607-8454
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:CONTEXT AND OBJECTIVE: Microcytic anaemia results from defective synthesis of haemoglobin in the erythroid precursors, causing a reduction in its mean corpuscular volume (MCV). The most common causes of microcytosis, without the increase in HbA levels, are iron deficiency anaemia (IDA) and α-thalassemia. The aim of this study was to identify the causes of microcytic anaemia and evaluate the haematological parameters from blood donors deemed ineligible (due to the low haematocrit level) that would differentiate the IDA and α-thal, whether isolated or in association. METHODS: Genomic DNA was submitted to the polymerase chain reaction multiplex for the diagnosis of the most common allele deletions of α-thal and erythrogram and in order to verify haematological parameters. Iron deficiency (ID) was determined through the measurement of serum ferritin. RESULTS: Of the 204 samples, 82 (40.2%) were identified with ID, 24 (17.8%) with α-thal and 10 (4.9%) with ID associated with α-thal. In the α-thal with ID group haemoglobin (Hb), MCV, mean corpuscular Hb concentration (MCHC) and mean corpuscular Hb (MCH) values were significantly lower compared to the isolated α-thal. In the group with ID Hb, MCV, MCHC and MCH values were significantly lower compared to those with isolated α-thal. The α-thal with ID group, showed Hb, MCV, MCHC and MCH significantly reduced when compared to those with IDA. CONCLUSIONS: This study showed that the values of haematological parameters, especially haematocrit, Hb, MCV, MCH, MCHC and red blood cell distribution width (RDW), are lower in patients with IDA, especially when associated with α-thal and therefore it may be useful to discriminate between the different types of microcytic anaemia.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[St] Status:Publisher
[do] DOI:10.1080/10245332.2018.1446703

  5 / 21559 MEDLINE  
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[PMID]: 29521137
[Au] Autor:Vosper J; Evangeli M; Porter JB; Shah F
[Ad] Address:a Department of Clinical Psychology , Royal Holloway University of London , Egham , UK.
[Ti] Title:Psychological Factors Associated with Episodic Chelation Adherence in Thalassemia.
[So] Source:Hemoglobin;:1-7, 2018 Mar 09.
[Is] ISSN:1532-432X
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:ß-Thalassemia major (ß-TM) is a life-long genetic hemoglobin (Hb) disorder requiring intensive treatment regimens, including frequent blood transfusions and daily chelation therapy. Understanding psychosocial correlates of chelation adherence is important for developing interventions to improve adherence. This study investigated within-participant correlates of oral chelation adherence on a daily (episodic) basis. Thirty-seven adult participants with ß-TM were recruited from clinics at two hospitals (22 males, 9 females, mean age 34.5 years, range 19-54 years). A structured interview was used to assess behavioral and psychological situational variables related to an adherent and a nonadherent episode for each participant. Positive outcome expectancies and higher self-efficacy were both significantly associated with adherent episodes. Behavioral variables, including difficulty in accessing medication, location, and whether alone or with others, were also associated with nonadherent episodes. Findings suggested that situational psychological factors are important for chelation adherence. Adherence interventions should consider focusing on potentially modifiable situational variables (psychological and behavioral).
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[St] Status:Publisher
[do] DOI:10.1080/03630269.2018.1433686

  6 / 21559 MEDLINE  
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[PMID]: 29519807
[Au] Autor:Antoniani C; Meneghini V; Lattanzi A; Felix T; Romano O; Magrin E; Weber L; Pavani G; El Hoss S; Kurita R; Nakamura Y; Cradick TJ; Lundberg AS; Porteus M; Amendola M; El Nemer W; Cavazzana M; Mavilio F; Miccio A
[Ad] Address:Laboratory of chromatin and gene regulation during development, Imagine Institute, INSERM UMR1163, Paris, France.
[Ti] Title:Induction of fetal hemoglobin synthesis by CRISPR/Cas9-mediated editing of the human ß-globin locus.
[So] Source:Blood;, 2018 Mar 08.
[Is] ISSN:1528-0020
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Naturally occurring, large deletions in the ß-globin locus result in hereditary persistence of fetal hemoglobin, a condition that mitigates the clinical severity of sickle-cell disease (SCD) and ß-thalassemia. We designed a CRISPR/Cas9 strategy to disrupt a 13.6-kb genomic region encompassing the δ- and ß-globin genes and a putative γ-δ intergenic fetal hemoglobin (HbF) silencer. Disruption of just the putative HbF silencer results in a mild increase in γ-globin expression, whereas deletion or inversion of a 13.6-kb region causes a robust re-activation of HbF synthesis in adult erythroblasts, associated with epigenetic modifications and changes in the chromatin contacts within the ß-globin locus. In primary, SCD patient-derived hematopoietic stem/progenitor cells, targeting the 13.6-kb region results in high proportion of γ-globin expression in erythroblasts, increased HbF synthesis, and amelioration of the sickling cell phenotype. Overall, this study provides clues for a potential genome editing approach to the therapy of ß-hemoglobinopathies.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[St] Status:Publisher

  7 / 21559 MEDLINE  
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[PMID]: 29518866
[Au] Autor:Li WX; Ma QW; Zeng FY
[Ti] Title:[The effect of DNA hydroxymethylase Tet2 on γ globin activation in the treatment of ß-thalassemia].
[So] Source:Zhonghua Nei Ke Za Zhi;57(3):206-211, 2018 Mar 01.
[Is] ISSN:0578-1426
[Cp] Country of publication:China
[La] Language:chi
[Ab] Abstract:To study the function of ten-eleven translocation 2 (Tet2) in γ globin gene expression in patients with ß- thalassemia. Gamma globin expression was induced by 5-azacytidine and Tet2 gene expression was knocked down by short hairpin RNA (shRNA) in a human immortalized myelogenous leukemia K562 cell line. The global 5-hydroxymethylcytosine (5hmC) level was measured by an ELISA kit. 5hmC level of γ globin gene was quantified by sulfite sequencing. The mRNA level of Tet2, γ globin, and related transcription factors Nfe4 and Klf1 were quantified by real-time PCR. Tet2 knockdown resulted in a decreased global 5hmC level from 0.14% to 0.03% as of the control group in K562 cells. The expression of γ globin was enhanced after 5-azacytidine treatment in vitro. However, γ globin mRNA level in Tet2 knockdown cells was only 55% as that in control group. The CG sites on γ globin gene were unmethylated. As Tet2 was down-regulated, the expression levels of Nfe4 and Klf1 decreased by about 80% and increased to 3.5 folds, respectively. Tet2 appears to maintain 5hmC level and facilitates γ globin gene activation. Moreover, Tet2 more likely regulates γ globin expression via affecting transcription factors rather than the gene itself. Thus, Tet2 could be a potential therapeutic target for ß thalassemias.
[Pt] Publication type:ENGLISH ABSTRACT; JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[St] Status:In-Process
[do] DOI:10.3760/cma.j.issn.0578-1426.2018.03.011

  8 / 21559 MEDLINE  
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[PMID]: 29516854
[Au] Autor:Sookaromdee P; Wiwanitkit V
[Ti] Title:Glomerular and Tubular Functions in Transfusion-Dependent Thalassemia.
[So] Source:Turk J Haematol;, 2018 Mar 08.
[Is] ISSN:1308-5263
[Cp] Country of publication:Turkey
[La] Language:eng
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180308
[Lr] Last revision date:180308
[St] Status:Publisher
[do] DOI:10.4274/tjh.2018.0083

  9 / 21559 MEDLINE  
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[PMID]: 29516378
[Au] Autor:Choudhry VP
[Ad] Address:Department of Hematology, Fortis Escorts Hospital, Neelam-Bata Road, Neelam Chowk, Faridabad, Haryana, 121001, India. vedpchoudhry@yahoo.co.in.
[Ti] Title:Economic Burden of Transfusion Dependent Thalassemia.
[So] Source:Indian J Pediatr;, 2018 Mar 08.
[Is] ISSN:0973-7693
[Cp] Country of publication:India
[La] Language:eng
[Pt] Publication type:JOURNAL ARTICLE; REVIEW
[Em] Entry month:1803
[Cu] Class update date: 180308
[Lr] Last revision date:180308
[St] Status:Publisher
[do] DOI:10.1007/s12098-018-2642-z

  10 / 21559 MEDLINE  
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[PMID]: 29346671
[Au] Autor:Pornprasert S; Salaeh NA; Tookjai M; Punyamung M; Pongpunyayuen P; Treesuwan K
[Ad] Address:Department of Medical Technology, Faculty of Associated Medical Sciences, Chiang Mai University, Chiang Mai, Thailand.
[Ti] Title:Hematological Analysis in Thai Samples With Deletional and Nondeletional HbH Diseases.
[So] Source:Lab Med;, 2018 Jan 13.
[Is] ISSN:1943-7730
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Objectives: To compare hematological parameters between deletional and nondeletional HbH diseases, and to investigate the correlation between HbH levels and hematological parameters within these 2 groups. Methods: Samples of 43 deletional HbH diseases, which included 39 --SEA/-α3.7, 4 - -SEA/-α4.2, and 22 nondeletional HbH diseases (- -SEA/αcsα), were used in this study. Correlations between HbH levels and hematological parameters within these 2 groups were analyzed. Results: The deletional HbH disease had higher levels of RBC counts, total Hb, pack cell volume (PCV), mean corpuscular Hb (MCH), mean corpuscular Hb concentration (MCHC), HbA, and HbA2 than did the nondeletional HbH disease. A negative correlation between HbH and RBC counts was detected in the group of deletional HbH disease, while a positive correlation between HbH and RBC counts, total Hb, and PCV was found in the group of nondeletional HbH disease. Conclusions: These results reflected that samples with nondeletional HbH showed more anemic features than those with the deletional HbH.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180308
[Lr] Last revision date:180308
[St] Status:Publisher
[do] DOI:10.1093/labmed/lmx068


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