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thalassemia major

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https://www.readbyqxmd.com/read/28078791/effects-of-stem-cell-transplantation-on-bone-mineral-density-and-vitamin-d-status-in-children-with-thalassemia-major
#1
Dilek Gürlek Gökçebay, Namik Ozbek, Arzu Yazal Erdem, Vildan Culha, Nese Yarali, Pamir Isik, Zekai Avci, Fatih Azik, Fatma Demirel, Bahattin Tunc
HSCT is a curative treatment in TM, but conditioning and immunosuppressive treatment may affect bone metabolism. In this retrospective study, we aimed to compare BMD, vitamin D status, and growth in children with TM who underwent HSCT to those in children with TD TM. Twenty-three children with TM who underwent HSCT (mean age 7.1 years [1.03-14.7]) and 24 children with TD thalassemia (mean age 9.8 years [1.6-14]) were recruited. Lumbar spine BMD of TD thalassemia patients was higher than those in patients who had HSCT at both baseline and second-year assessments (P=...
January 12, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28068960/nutritional-status-among-primary-school-children-in-rural-sri-lanka-a-public-health-challenge-for-a-country-with-high-child-health-standards
#2
N P G C R Naotunna, M Dayarathna, H Maheshi, G S Amarasinghe, V S Kithmini, M Rathnayaka, L Premachandra, N Premarathna, P C Rajasinghe, G Wijewardana, T C Agampodi, S B Agampodi
BACKGROUND: Nutritional status of pre adolescent children is not widely studied in Sri Lanka. The purpose of this study was to determine the nutritional status among pre-adolescent school children in a rural province of Sri Lanka. METHODS: A school based cross sectional study was carried out in North Central Province in 100 rural schools, selected using multi stage cluster sampling with probability proportionate to size. Children in grade one to five were enrolled with a maximum cluster size of fifty...
January 10, 2017: BMC Public Health
https://www.readbyqxmd.com/read/28065838/current-results-and-future-research-priorities-in-late-effects-after-hematopoietic-stem-cell-transplantation-hct-for-children-with-sickle-cell-disease-and-thalassemia-a-consensus-statement-from-the-second-pediatric-blood-and-marrow-transplant-consortium-international
#3
Shalini Shenoy, Emanuele Angelucci, Staci D Arnold, K Scott Baker, Monica Bhatia, Dorine Bresters, Andrew C Dietz, Josu De La Fuente, Christine Duncan, Javid Gaziev, Allison A King, Michael A Pulsipher, Angela Smith, Mark C Walters
Sustained donor engraftment after allogeneic hematopoietic cell transplantation (HCT) converts to healthy donor hemoglobin synthesis and halts disease symptoms in patients with sickle cell disease (SCD) and thalassemia major. A disease free survival probability that exceeds 90% has been reported when HCT using an HLA-matched sibling donor is performed in young patients with low risk disease or treatment related risk factors. Alternate donor HCT and HCT in adults is performed infrequently due to a higher risk profile...
January 5, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28057638/an-international-registry-of-survivors-with-hb-bart-s-hydrops-fetalis-syndrome
#4
Duantida Songdej, Christian Babbs, Douglas R Higgs
Hb Bart's Hydrops Fetalis Syndrome (BHFS) resulting from α(0)-thalassemia is considered a universally fatal disorder. However, over the last three decades improvements in intrauterine interventions and perinatal intensive care have resulted in increasing numbers of BHFS survivors. We have initiated an international registry containing information on 69 patients, of which 31 are previously unpublished. In this perspective we analyze the available clinical information to document the natural history of BHFS...
January 5, 2017: Blood
https://www.readbyqxmd.com/read/28028293/potential-use-of-cord-blood-for-hb-e-hemoglobinopathy-screening-programme-using-capillary-electrophoresis
#5
W A Wan Mohd Saman, R Hassan, S Mohd Yusoff, C A Che Yaakob, N A F Abdullah, S Ghazali, M A R Mohd Radzi, R Bahar
BACKGROUND: Thalassemia and hemoglobinopathies are inherited red blood cell disorders found worldwide. Hemoglobin (Hb) E disorder is one of the hemoglobinopathies known to have the high prevalence in South East Asia. Most of transfusion-dependent thalassemias were genotypically compound heterozygous Hb E/ β-thalassemia. In Malaysia, the national screening program for thalassemia was implemented for early pregnancy or secondary school girls; however many participants do not turn-up and missed the screening test...
December 2016: Malaysian Journal of Pathology
https://www.readbyqxmd.com/read/28019032/severe-cardiac-iron-toxicity-in-two-adults-with-sickle-cell-disease
#6
Hellen Oduor, Caterina P Minniti, Alessandra Brofferio, Ahmed M Gharib, Khaled Z Abd-Elmoniem, Matthew M Hsieh, John F Tisdale, Courtney D Fitzhugh
BACKGROUND: Use of chronic blood transfusions as a treatment modality in patients with blood disorders places them at risk for iron overload. Since patients with β-thalassemia major (TM) are transfusion-dependent, most studies on iron overload and chelation have been conducted in this population. While available data suggest that compared to TM, patients with sickle cell disease (SCD) have a lower risk of extrahepatic iron overload, significant iron overload can develop. Further, previous studies have demonstrated a direct relationship between iron overload and morbidity and mortality rates in SCD...
December 26, 2016: Transfusion
https://www.readbyqxmd.com/read/27999484/effects-of-home-care-training-on-the-self-efficacy-of-patients-with-beta-thalassemia-major
#7
Mahdieh Poodineh Moghadam, Hajar Nourisancho, Hossein Shahdadi, Sohila Shahraki, Batoul Azarkish, Abbas Balouchi
BACKGROUND: The self-efficacy of thalassemia patients is an important factor in creating behavioral changes in such patients. Home-care training reduces hospitalization duration as well as relevant costs and improves disease outcomes. This study was designed to assess the effect of home-care training on the self-efficacy of patients with beta thalassemia major. METHODOLOGY: This was a quasi-experimental, case-control, before and after intervention study conducted on 136 thalassemia cases from January 2014 to October 2015...
October 2016: Materia Socio-medica
https://www.readbyqxmd.com/read/27998866/-nosocomial-septicemia-in-the-early-stage-after-stem-cell-transplantation-in-children-with-major-%C3%AE-thalassemia
#8
Yan-Jun Xu, Chun-Fu Li, Yue-Lin He, Xiao-Qin Feng, Fu-Yu Pei, Xue-Dong Wu
OBJECTIVE: To explore the clinical characteristics of nosocomial septicemia in the early stage after hematopoietic stem cell transplantation (HSCT) in children with major β-thalassemia. METHODS: The clinical data were retrospectively analyzed of 55 consecutive children with major β-thalassemia who developed septicemia early after HSCT between January, 2011 and June, 2016. RESULTS: Among the total of 416 consecutive children with major β-thalassemia undergoing allogeneic HSCT, the incidence of nosocomial infection early after transplantation was 77...
December 20, 2016: Nan Fang Yi Ke da Xue Xue Bao, Journal of Southern Medical University
https://www.readbyqxmd.com/read/27987208/poor-stem-cell-harvest-may-not-always-be-related-to-poor-mobilization-lessons-gained-from-a-mobilization-study-in-patients-with-%C3%AE-thalassemia-major
#9
Varnavas C Constantinou, Asimina Bouinta, Garyfalia Karponi, Fani Zervou, Penelope-Georgia Papayanni, George Stamatoyannopoulos, Achilles Anagnostopoulos, Evangelia Yannaki
BACKGROUND: Hematopoietic stem cell mobilization and leukapheresis in adult patients with β-thalassemia have recently been optimized in the context of clinical trials for obtaining hematopoietic stem cells for thalassemia gene therapy. In some patients, however, the yield of cluster of differentiation 34-positive (CD34+) cells was poor despite successful mobilization, and a modification of apheresis settings was mandatory for harvest rescue. STUDY DESIGN AND METHODS: Data were analyzed from 20 adult patients with β-thalassemia who were enrolled in a clinical trial of optimizing mobilization strategies for stem cell gene therapy...
December 17, 2016: Transfusion
https://www.readbyqxmd.com/read/27981643/ugt1a1-ta-n-genotype-is-not-the-major-risk-factor-of-cholelithiasis-in-sickle-cell-disease-children
#10
Philippe Joly, Céline Renoux, Philippe Lacan, Yves Bertrand, Giovanna Cannas, Nathalie Garnier, Daniella Cuzzubbo, Kamila Kebaïli, Cécile Renard, Alexandra Gauthier, Vincent Pialoux, Cyril Martin, Marc Romana, Philippe Connes
Because of the increased hemolytic rate, a significant proportion of patients with sickle cell disease (SCD) are prone to develop cholelithiasis. The present study investigated the role of several genetic factors (UGT1A1 promoter (TA)n repeat polymorphism, alpha-globin status), hematological parameters, clinical severity and hydroxyurea (HU) therapy on the occurrence of cholelithiasis in SCD METHODS: One hundred and fifty eight children (2-18 years old) and regularly followed at the university hospital of Lyon (France) were included...
December 16, 2016: European Journal of Haematology
https://www.readbyqxmd.com/read/27956899/body-composition-in-adult-patients-with-thalassemia-major
#11
Marianna Vlychou, Evangelos Alexiou, Paschalis Thriskos, Ioannis Fezoulidis, Katerina Vassiou
Objective. To assess body composition in adult male and female patients with thalassemia major by dual-energy X-ray absorptiometry (DXA) and to compare the findings with a group of healthy age-matched controls. Methods. Our study group included sixty-two patients (27 males, mean age 36 years, and 35 females, mean age 36.4 years) and fifteen age-matched healthy controls. All patients had an established diagnosis of thalassemia major and followed a regular blood transfusion scheme since childhood and chelation treatment...
2016: International Journal of Endocrinology
https://www.readbyqxmd.com/read/27928477/health-related-quality-of-life-and-health-utility-values-in-beta-thalassemia-major-patients-receiving-different-types-of-iron-chelators-in-iran
#12
Meysam Seyedifar, Farid Abedin Dorkoosh, Amir Ali Hamidieh, Majid Naderi, Hossein Karami, Mehran Karimi, Masoomeh Fadaiyrayeny, Masoumeh Musavi, Sanaz Safaei, Mohammad Mahdi Ahmadian-Attari, Molouk Hadjibabaie, Abdol Majid Cheraghali, Ali Akbari Sari
Background: Thalassemia is a chronic, inherited blood disorder, which in its most severe form, causes life-threatening anemia. Thalassemia patients not only engage with difficulties of blood transfusion and iron chelating therapy but also have some social challenges and health threatening factors. There are some reports on quality of life in thalassemia patients around the world from southeast of Asia to Italy in Europe and United States. In this study, we tried to evaluate and compare Health Related Quality of life (HRQoL) and the health utility in beta thalassemia major patients receiving different types of iron chelators and living in different socio-economical situations...
October 1, 2016: International Journal of Hematology-oncology and Stem Cell Research
https://www.readbyqxmd.com/read/27928257/evaluating-the-correlation-between-serum-nt-probnp-level-and-diastolic-dysfunction-severity-in-beta-thalassemia-major-patients
#13
Behzad Alizadeh, Zahra Badiee, Mahmoud Mahmoudi, Mahsa Mohajery
Background: N-terminal pro-brain natriuretic peptide (NT-proBNP) is a sensitive biomarker for the detection of asymptomatic left ventricular (LV) dysfunction. Since β-thalassemia major patients suffer from early diastolic dysfunction due to iron deposition of chronic blood transfusion, we tried to evaluate the correlation between the serum NT-proBNP level and the severity of LV diastolic dysfunction determined by echocardiography in these patients. Methods: Fifty β-thalassemia major patients with normal LV systolic function were studied by tissue Doppler echocardiography, and blood samples were taken at the same time to measure the serum NT-proBNP level...
April 13, 2016: Journal of Tehran Heart Center
https://www.readbyqxmd.com/read/27924466/quality-of-life-in-children-with-thalassemia-and-their-caregivers-in-india
#14
Sapna Sharma, Bageshree Seth, Prashant Jawade, Madhavi Ingale, Maninder Singh Setia
OBJECTIVES: To assess and compare the Quality of Life (QOL) of children with beta-thalassemia major on regular transfusion therapy with normal children, and of the caregivers of children with beta-thalassemia major to that of caregivers of normal children. METHODS: A cross-sectional comparison of QOL in 75 thalassemic and 80 non-thalassemic children was conducted using the PedsQL™ 4.0 generic core scale. Also self-rated health was assessed in their caregivers using Short Form-36 Health Survey...
December 7, 2016: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/27923273/decision-points-in-the-treatment-of-transfusional-iron-overload-in-patients-with-myelodysplastic-syndromes-why-when-and-how-to-chelate
#15
Farhan Imran, Pradyumna Phatak
Patients with myelodysplastic syndromes (MDS) differ from those with other transfusion-dependent conditions (eg, thalassemia) as they are typically older, have comorbid conditions, and a generally shorter life expectancy. The underlying disease process in MDS and frequent use of red blood cell transfusions lead to iron accumulation and ultimately organ damage. Whether iron-reducing interventions such as chelation therapy can improve outcomes in this population is currently under investigation. Areas covered: We reviewed published English-language articles from PubMed on the topic of iron overload (IO) in MDS, and the use of iron chelation therapies (ICTs) to alleviate iron burden...
January 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/27909215/cure-for-thalassemia-major-from-allogeneic-hematopoietic-stem-cell-transplantation-to-gene-therapy
#16
Alok Srivastava, Ramachandran V Shaji
Allogeneic hematopoietic stem cell transplantation has been established for several decades as a gene replacement therapy for patients with thalassemia major and now offers very high rates of cure to those who are able to access this therapy. Outcomes have improved tremendously over the last decade even in high-risk patients. The limited data available suggests that the long-term outcome is also excellent with >90% survival but for best results, hematopoietic stem cell transplantation should be offered early before any end organ damage occurs...
December 1, 2016: Haematologica
https://www.readbyqxmd.com/read/27900772/hematopoietic-stem-cell-transplantation-for-people-with-%C3%A3-thalassaemia-major
#17
REVIEW
Vanitha A Jagannath, Zbys Fedorowicz, Amani Al Hajeri, Akshay Sharma
BACKGROUND: Thalassemia is an inherited autosomal recessive blood disorder, caused by mutations in globin genes or their regulatory regions. This results in a reduced rate of synthesis of one of the globin chains that make up haemoglobin. In ß-thalassaemia major there is an underproduction of ß-globin chains combined with excess of free α-globin chains. The excess free α-globin chains precipitate in red blood cells, leading to their destruction (haemolysis) and ineffective erythropoiesis...
November 30, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27900452/microparticles-from-splenectomized-%C3%AE-thalassemia-hbe-patients-play-roles-on-procoagulant-activities-with-thrombotic-potential
#18
Phatchanat Klaihmon, Kunwadee Phongpao, Wasinee Kheansaard, Egarit Noulsri, Archrob Khuhapinant, Suthat Fucharoen, Noppawan Phumala Morales, Saovaros Svasti, Kovit Pattanapanyasat, Pornthip Chaichompoo
Thromboembolic events including cerebral thrombosis, deep vein thrombosis, and pulmonary embolism are major complications in β-thalassemia. Damaged red blood cells and chronic platelet activation in splenectomized β-thalassemia/HbE patients were associated with increased microparticles (MPs) releases into blood circulation. MPs are small membrane vesicles, which play important roles on coagulation. However, the role of MP in thalassemia is poorly understood. In this study, the effects of splenectomized-MPs on platelet activation and aggregation were investigated...
November 29, 2016: Annals of Hematology
https://www.readbyqxmd.com/read/27891555/the-epidemiologic-transition-of-thalassemia-and-associated-hemoglobinopathies-in-southern-taiwan
#19
Hui-Ching Wang, Li-Ling Hsieh, Yi-Chang Liu, Hui-Hua Hsiao, Shu-Kai Lin, Wen-Chan Tsai, Ta-Chih Liu
Since 1993, following the National Thalassemia Major Prevention Program and an increase in immigration and interracial marriages, especially in southern Taiwan, the distribution of hemoglobinopathies may have changed. This study investigates the epidemiologic transition of hemoglobinopathies. We analyzed 1870 specimens collected between 2003 and 2012 in southern Taiwan, used gap-polymerase chain reaction and PCR-restriction fragment length polymorphism-based methods, and confirmed genotypes of hemoglobinopathies by DNA sequencing...
November 28, 2016: Annals of Hematology
https://www.readbyqxmd.com/read/27891535/gene-therapy-for-hemoglobin-disorders-a-mini-review
#20
Parul Rai, Punam Malik
Gene therapy by either gene insertion or editing is an exciting curative therapeutic option for monogenic hemoglobin disorders like sickle cell disease and β-thalassemia. The safety and efficacy of gene transfer techniques has markedly improved with the use of lentivirus vectors. The clinical translation of this technology has met with good success, although key limitations include number of engraftable transduced hematopoietic stem cells and adequate transgene expression that results in complete correction of β0 thalassemia major...
2016: Journal of Rare Diseases Research & Treatment
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