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thalassemia major

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https://www.readbyqxmd.com/read/28221264/efficacy-and-safety-of-combined-oral-chelation-with-deferiprone-and-deferasirox-in-children-with-%C3%AE-thalassemia-major-an-experience-from-north-india
#1
Nupur Parakh, Jagdish Chandra, Sunita Sharma, Bhavna Dhingra, Rajesh Jain, DeoNath Mahto
OBJECTIVE: A combination of desferrioxamine with either deferiprone (DFP) or deferasirox (DFX) for patients with β-thalassemia major who do not achieve negative iron balance with monotherapy has been studied widely. However, poor compliance resulting from the need for parentral administration of desferrioxamine and its cost necessicitates combining 2 oral chelators. METHODS: A prospective study was conducted in patients with transfusion-dependent β-thalassemia major in a tertiary care center over 2 years...
February 17, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28218005/evaluation-of-cardiac-and-hepatic-iron-overload-in-thalassemia-major-patients-with-t2-magnetic-resonance-imaging
#2
Pustika Amalia Wahidiyat, Felix Liauw, Damayanti Sekarsari, Siti Ayu Putriasih, Vasili Berdoukas, Dudley J Pennell
OBJECTIVES: Recent advancements have promoted the use of T2* magnetic resonance imaging (MRI) in the non-invasive detection of iron overload in various organs for thalassemia major patients. This study aims to determine the iron load in the heart and liver of patients with thalassemia major using T2* MRI and to evaluate its correlation with serum ferritin level and iron chelation therapy. METHODS: This cross-sectional study included 162 subjects diagnosed with thalassemia major, who were classified into acceptable, mild, moderate, or severe cardiac and hepatic iron overload following their T2* MRI results, respectively, and these were correlated to their serum ferritin levels and iron chelation therapy...
February 20, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28215056/an-analysis-of-blood-utilization-for-stem-cell-transplant-patients-in-a-tertiary-care-hospital
#3
Natasha Ali
Background and Objective: Haematopoietic stem cell transplant is a potentially curative treatment option in various benign and malignant haematological diseases. Patients undergoing stem cell transplant procedure require blood transfusion on a daily basis. Currently, there is paucity of data from developing countries on transfusion practices. This audit was undertaken to determine the consumption of packed red blood cells (PRBCs) transfusion in the bone marrow transplant unit of the Aga Khan University Hospital...
February 28, 2017: International Journal of Stem Cells
https://www.readbyqxmd.com/read/28203323/the-effects-of-nutrition-exercise-and-a-praying-program-on-reducing-iron-overload-in-patients-with-beta-thalassemia-major-a-randomized-clinical-trial
#4
Zahra Molazem, Roghaye Noormohammadi, Roya Dokouhaki, Maryam Zakerinia, Zahra Bagheri
BACKGROUND: Excessive iron accumulation in the visceral organs creates problems for patients with beta-thalassemia major. Despite chelation therapy, mortality rate from the complications of this disease is still quite high. OBJECTIVES: This study aimed to investigate the effectiveness of nutrition, exercise, and a praying program at reducing iron overload in patients with beta-thalassemia major. PATIENTS AND METHODS: This randomized clinical trial assessed the effect of the designed care program on iron overload...
October 2016: Iranian Journal of Pediatrics
https://www.readbyqxmd.com/read/28200076/prediction-of-cardiac-complications-for-thalassemia-major-in-the-widespread-cardiac-magnetic-resonance-era-a-prospective-multicentre-study-by-a-multi-parametric-approach
#5
Alessia Pepe, Antonella Meloni, Giuseppe Rossi, Massimo Midiri, Massimiliano Missere, Gianluca Valeri, Francesco Sorrentino, Domenico Giuseppe D'Ascola, Anna Spasiano, Aldo Filosa, Liana Cuccia, Nicola Dello Iacono, Gianluca Forni, Vincenzo Caruso, Aurelio Maggio, Lorella Pitrolo, Angelo Peluso, Daniele De Marchi, Vincenzo Positano, John C Wood
No abstract text is available yet for this article.
February 14, 2017: European Heart Journal Cardiovascular Imaging
https://www.readbyqxmd.com/read/28192253/hematopoietic-stem-cell-transplantation-using-preimplantation-genetic-diagnosis-and-hla-typing-for-hla-matched-sibling-donor-a-turkish-multicenter-study
#6
Emin Kurekci, Alphan Küpesiz, Sema Anak, Gülyüz Öztürk, Orhan Gürsel, Serap Aksoylar, Talia Ileri, Barış Kuşkonmaz, Ibrahim Eker, Mualla Cetin, Gülsün Tezcan Karasu, Zühre Kaya, Tunç Fışgın, Mehmet Ertem, Savaş Kansoy, Mehmet Akif Yeşilipek
Preimplantation genetic diagnosis involves the diagnosis of a genetic disorder in embryos obtained through in vitro fertilization, selection of healthy embryos, and transferring them to the mother's uterus. Preimplantation genetic diagnosis has been used not only to avoid the risk of having an affected child, but also by using HLA matching together, it offers preselection of potential HLA-genoidentical healthy donor progeny for an affected sibling, who requires bone marrow transplantation. Here, we share the hematopoietic stem cell transplantation results of 52 patients with different benign and malign hematological or metabolic diseases or immunodeficiencies, whose donors were their siblings borned with this technique in Turkey since 2008...
February 10, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28168351/circulating-microparticles-and-the-risk-of-thromboembolic-events-in-egyptian-beta-thalassemia-patients
#7
Ilham Youssry, Nohair Soliman, Mona Ghamrawy, Rania Mohamed Samy, Amal Nasr, Mohamed Abdel Mohsen, Mohamed ElShahaat, Rayan Bou Fakhredin, Ali Taher
The presence of elevated numbers of circulating microparticles (MPs) has been hypothesized to be responsible for the occurrence of thromboembolic events (TEEs) in thalassemic patients. Our aim is to evaluate the presence and the thrombotic risk of circulating MPs in thalassemia patients and to determine the difference in MPs between β-thalassemia major (β-TM) and thalassemia intermedia (TI). The percentage of the annexin-labeled MPs, platelet-derived MPs (PMPs), erythrocyte-derived MPs (RMPs), and endothelial-derived MPs (EMPs) was measured by flow cytometry, in 87 thalassemia patients (39 β-TM and 48 TI)...
February 7, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28149147/craniofacial-characteristics-of-thalassemia-major-patients
#8
Sacide Karakas, Ayfer Metin Tellioglu, Mehmet Bilgin, Imran Kurt Omurlu, Sercin Caliskan, Salih Coskun
OBJECTIVE: Thalassemias major are the most common autosomal recessive disorders; they are characterized by anomalies in the synthesis of the beta chains of hemoglobin and are often associated with varying degrees of craniofacial anomalies. The purpose of this study was to evaluate the craniofacial dimensions of β-thalassemia patients and to identify differences by comparing them to those of a control group. MATERIALS AND METHODS: The study comprised 43 thalassemia major patients and 26 age- and sex- matched healthy control subjects...
October 2016: Eurasian Journal of Medicine
https://www.readbyqxmd.com/read/28141745/deferasirox-iron-complex-formation-ratio-as-an-indicator-of-long-term-chelation-efficacy-in-%C3%AE-thalassemia-major
#9
Meng-Yao Lu, Ting-Hao Lin, Po-Hung Chiang, Pei-Hsin Kuo, Ning Wang, Wen-Hsin Wu, Kai-Hsin Lin, Tzu-Hua Wu
BACKGROUND: β-Thalassemia major patients with higher total drug levels (deferasirox [DEFR] plus its iron complex) do not yield better serum ferritin (SF) control. This study aimed to determine the concentrations of DEFR and its iron complex (Fe-[DEFR]2) in thalassemia patients to predict the chelation efficacy in terms of SF and cardiac T2* values. METHODS: Patients' steady-state drug levels at trough (Ctrough) and 2 hours post-dose (C2h) were determined. Since iron deposition may cause changes in the hepatic metabolism of amino acids, the concentrations of 40 amino acids in plasma were also assayed at 2 hours post-dose...
January 30, 2017: Therapeutic Drug Monitoring
https://www.readbyqxmd.com/read/28139073/assessment-of-left-ventricular-functions-and-myocardial-iron-load-with-tissue-doppler-and-speckle-tracking-echocardiography-and-t2-mri-in-patients-with-%C3%AE-thalassemia-major
#10
Mehmet Emre Ari, Filiz Ekici, İbrahim İlker Çetin, Emine Betül Tavil, Neşe Yaralı, Pamir Işık, Tuncay Hazırolan, Bahattin Tunç
BACKGROUND: The purpose of this study is to determine early myocardial dysfunction in β-thalassemia major (BTM) patients. Where the myocardial dysfunction cannot be detected by conventional echocardiography, it could be detected by tissue Doppler imaging (TDI) or speckle tracking echocardiography (STE). METHODS: In this study, we analyzed 60 individuals, 30 of whom were BTM patients and the other 30 of whom were the control group. T2* magnetic resonance imaging (MRI) was used to measure cardiac iron deposition...
January 31, 2017: Echocardiography
https://www.readbyqxmd.com/read/28135306/thalassemia-in-the-united-arab-emirates-why-it-can-be-prevented-but-not-eradicated
#11
Sehjeong Kim, Abdessamad Tridane
Thalassemia is a genetic blood disorder that causes abnormal hemoglobin. Hemoglobin is a protein in red blood cells that carries oxygen and is made of two proteins from four α-globin genes and two β-globin genes. A defect in one or more of these genes causes thalassemia. The treatment of thalassemia mostly depends on life-long blood transfusions and removal of excessive iron from the blood stream. Such tremendous blood consumption puts pressure on the national blood stock in many countries. In particular, in the United Arab Emirates (UAE), various forms of thalassemia prevention have been used and hence, the substantial reduction of the thalassemia major population has been achieved...
2017: PloS One
https://www.readbyqxmd.com/read/28109403/total-antioxidant-capacity-in-beta-thalassemia-a-systematic-review-and-meta-analysis-of-case-control-studies
#12
REVIEW
Husseen Manafikhi, Gregor Drummen, Maura Palmery, Ilaria Peluso
Total Antioxidant Capacity (TAC), a biomarker measuring the antioxidant potential of body fluids, including redox synergistic interactions, is influenced by the presence of products of catabolism such as bilirubin (BR) and uric acid (UA). Hyperuricaemia and increased BR levels were observed in thalassemia. In order to evaluate the differences in TAC values between thalassemic patients and healthy subjects, we performed a systematic review and meta-analysis of case-control studies. After the exclusion of data deemed unsuitable for meta-analysis inclusion and a study imputed of bias by Trim-and-fill analysis, mean difference (MD) and confidence intervals 95% (CI 95%) were calculated by the random effect model for beta-thalassemia major (BTM) (1351 subjects: 770 thalassemic and 581 controls, from 15 studies) and Trait (BTT) or Hemoglobin E (BTE) (475 subjects: 165 thalassemic and 310 controls, from 5 studies)...
February 2017: Critical Reviews in Oncology/hematology
https://www.readbyqxmd.com/read/28101307/review-and-recommendations-on-management-of-adult-female-thalassemia-patients-with-hypogonadism-based-on-literature-review-and-experience-of-icet-a-network-specialists
#13
REVIEW
Vincenzo De Sanctis, Ashraf T Soliman, Heba Elsedfy, Alice Albu, Soad Al Jaouni, Salvatore Anastasi, Maria Grazia Bisconte, Duran Canatan, Soteroula Christou, Shahina Daar, Salvatore Di Maio, Mohamed El Kholy, Doaa Khater, Mohamed Elshinawy, Yurdanur Kilinc, Roberto Mattei, Hala H Mosli, Alessandra Quota, Maria Grazia Roberti, Praveen Sobti, Saif Al Yaarubi, Saveria Canpisi, Christos Kattamis
BACKGROUND: Multi-transfused thalassemia major (TM) patients frequently develop severe endocrine complications, mainly due to iron overload, anemia, and chronic liver disease, which require prompt diagnosis, treatment and follow-up by specialists. The most common endocrine complication documented is hypogonadotropic hypogonadism which increases with age and associated comorbidities. It is thus important for physicians to have a clear understanding of the pathophysiology and management of this disorder...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28099398/utilizing-a-novel-mobile-health-selfie-application-to-improve-compliance-to-iron-chelation-in-pediatric-patients-receiving-chronic-transfusions
#14
Sarah Leonard, Lindsay M Anderson, Jude Jonassaint, Charles Jonassaint, Nirmish Shah
Iron chelation therapy can prevent iron overload for pediatric patients with sickle cell disease and β-thalassemia major; however, adherence is suboptimal. Therefore, we developed an intensive training program (ITP), to improve medication management and disease knowledge. The objectives were to determine feasibility of the ITP and its preliminary impact on adherence, disease knowledge, and health outcomes. Pediatric patients were recruited to participate in the ITP over a 90-day period and were followed for 6 months...
January 17, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28078791/effects-of-stem-cell-transplantation-on-bone-mineral-density-and-vitamin-d-status-in-children-with-thalassemia-major
#15
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
#16
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-for-children-with-sickle-cell-disease-and-thalassemia-a-consensus-statement-from-the-second-pediatric-blood-and-marrow-transplant-consortium-international
#17
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 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 because of 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
#18
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
#19
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
#20
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
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