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thalassemia major

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https://www.readbyqxmd.com/read/28440111/quality-of-life-outcomes-in-a-pediatric-thalassemia-population-in-egypt
#1
Soheir Adam, Hanan Afifi, Manal Thomas, Phoebe Magdy, Ghada El-Kamah
Thalassemia is a disorder of hemoglobin (Hb) synthesis characterized by chronic hemolysis. In β-thalassemias major (β-TM), patients require regular transfusion at an early age due to severe anemia. Subsequently, intensive chelation therapy is initiated to mitigate the effects of the resultant iron overload. Clinical disease burden and the demanding treatment can affect health-related quality of life (HRQoL) outcomes in this population. The aim of this study was to assess HRQoL outcomes in Egyptian pediatric thalassemia patients...
April 25, 2017: Hemoglobin
https://www.readbyqxmd.com/read/28435994/thalassemia-minor-and-major-%C3%A2-current-management
#2
REVIEW
Ved Prakash Choudhry
Thalassemia is a common genetic disorder. It has been estimated that in India nearly 5 crore people are thalassemia carriers. They are asymptomatic and are detected on blood tests. These people are at same risk of developing iron deficiency anemia as general population and need iron therapy in the presence of iron deficiency anemia. Nearly 12,000 children with thalassemia major (Homozygous state) are born every year. These children often present with significant anemia along with hepatosplenomegaly during infancy and require early diagnosis and institution of therapy with repeated blood transfusions and chelation therapy...
April 24, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28428899/watershed-infarct-in-beta-thalassemia-major-patient
#3
Behnaz Ansari, Mohammad Saadatnia, Ali Asghar Okhovat
Background. The mechanism of stroke in beta-thalassemia was reported previously as cardioembolic and hypercoagulable state. However, there is no report of watershed infarct in beta-thalassemia anemia. Method. We present an adult β-thalassemia major patient with manifest asymptomatic chronic left carotid occlusion who suffered watershed infarct. Result. In the presence of asymptomatic chronic left internal carotid occlusion, we assumed that severe anemia (hemoglobin = 3) at admission leads to watershed infarct...
2017: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/28419488/interferon-%C3%AE-3-gene-il28b-is-associated-with-spontaneous-or-treatment-induced-viral-clearance-in-hepatitis%C3%A2-c-virus-infected-multitransfused-patients-with-thalassemia
#4
Aritra Biswas, Rushna Firdaus, Debanjali Gupta, Monika Ghosh, Kallol Saha, Prosanto Chowdhury, Maitreyee Bhattacharyya, Provash C Sadhukhan
BACKGROUND: Hepatitis C virus (HCV) is the major posttransfusion infection in multitransfused individuals in India with thalassemia major. To our knowledge, this study is the first conducted to correlate and comprehend the effects of the host interleukin (IL)28B gene polymorphism at loci rs12979860 and rs8099917 in spontaneous or interferon (IFN)-induced treatment response in the HCV-seroreactive individuals with thalassemia major. STUDY DESIGN AND METHODS: A total of 557 HCV-seroreactive individuals with thalassemia were processed for HCV viral genotyping and host IL28B single-nucleotide polymorphism analysis at loci rs12979860 and rs8099917...
April 16, 2017: Transfusion
https://www.readbyqxmd.com/read/28408108/busulfan-drug-monitoring-is-needed-in-patients-undergoing-allogeneic-stem-cell-transplantation
#5
Bushra Salman, Mohammed Al-Zaabi, Mohammed Al-Huneini, David Dennison, Abdulhakeem Al-Rawas, Salam Al-Kindi, Khalil Al-Farsi, Melanie Tauro, Murtadha Al-Khabori
Busulfan (Bu)-based preparative regimens in hematopoietic stem cell transplantation are commonly used. Previous studies have shown that Bu at a fixed dose of 3.2mg/kg/day (FBD) given intravenously decreases variability in drug pharmacokinetics and this decreases the dependency on therapeutic drug monitoring (TDM) of Bu. We compared the Bu dose given using TDM with the FBD of 3.2mg/kg/day. Seventy-three patients with acute leukemia, myelodysplasia, chronic myeloid leukemia, thalassemia major, and sickle cell disease were included...
April 6, 2017: Hematology/oncology and Stem Cell Therapy
https://www.readbyqxmd.com/read/28408107/hydroxyurea-for-nontransfusion-dependent-%C3%AE-thalassemia-a-systematic-review-and-meta-analysis
#6
Ali H Algiraigri, Nicola A M Wright, Elizabeth Oddone Paolucci, Aliya Kassam
Nontransfusion-dependent β-thalassemia (NTDβT) syndromes consist of β-thalassemia intermedia and moderate hemoglobin E/β thalassemias. They are characterized by varying degrees of chronic anemia and a wide spectrum of complications due to ineffective erythropoiesis and iron overload from chronic transfusions. Hydroxyurea (HU), an oral chemotherapeutic drug, is anticipated to decrease disease severity. We performed a meta-analysis to evaluate the clinical efficacy and safety of HU in NTDβT patients of any age...
April 6, 2017: Hematology/oncology and Stem Cell Therapy
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
#7
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/28404539/a-national-registry-of-thalassemia-in-turkey-demographic-and-disease-characteristics-of-patients-achievements-and-challenges-in-prevention
#8
Yeşim Aydınok, Yeşim Oymak, Berna Atabay, Gönül Aydoğan, Akif Yeşilipek, Selma Ünal, Yurdanur Kılınç, Banu Oflaz, Mehmet Akın, Canan Vergin, Melike Sezgin Evim, Ümran Çalışkan, Şule Ünal, Ali Bay, Elif Kazancı, Dilber Talia İleri, Didem Atay, Türkan Patıroğlu, Selda Kahraman, Murat Söker, Mediha Akcan, Aydan Akdeniz, Mustafa Büyükavcı, Güçhan Alanoğlu, Özcan Bör, Nur Soyer, Nihal Özdemir Karadaş, Ezgi Uysalol, Meral Türker, Arzu Akçay, Süheyla Ocak, Adalet Meral Güneş, Hüseyin Tokgöz, Zümrüt Uysal, Naci Tiftik, Zeynep Karakaş
Turkish Society of Pediatric Hematology set up a National Hemoglobinopathy Registry to demonstrate demographic and disease characteristics of patients, and assessed the efficacy of hemoglobinopathy control program (HCP) over 10 years in Turkey. A total of 2046 patients from 27 Thalassemia Centers were registered in which 1988 were eligible for analysis. This cohort mainly comprised patients with β-thalassemia major (n=1658, 83.4%) and intermedia (n=215, 10.8%). The majority of patients were accumulated into the costal areas of Turkey...
April 13, 2017: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
https://www.readbyqxmd.com/read/28402168/performance-evaluation-of-automated-impedance-and-optical-fluorescence-platelet-counts-compared-with-international-reference-method-in-patients-with-thalassemia
#9
Chaicharoen Tantanate, Ladawan Khowawisetsut, Kovit Pattanapanyasat
CONTEXT: - Spurious platelet counts from automated methods have been reported in patients with abnormal red blood cells. However, there is no specific study regarding performance of platelet counts by automated methods in patients with thalassemia. OBJECTIVE: - To investigate the performance of automated platelet counts, including impedance (PLT-I) and optical fluorescent (PLT-O and PLT-F) methods, and compare them with the international reference method (IRM) for platelet counting in patients with thalassemia...
April 12, 2017: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/28401102/human-parvovirus-b19-in-patients-with-beta-thalassemia-major-from-tehran-iran
#10
Seyed Ali Mohammad Arabzadeh, Farideh Alizadeh, Ahmad Tavakoli, Hamidreza Mollaei, Farah Bokharaei-Salim, Gharib Karimi, Mohammad Farahmand, Helya Sadat Mortazavi, Seyed Hamidreza Monavari
BACKGROUND: Due to the tropism of human parvovirus B19 to erythroid progenitor cells, infection in patients with an underlying hemolytic disorder such as beta-thalassemia major leads to suppression of erythrocyte formation, referred to as transient aplasia crisis (TAC), which may be life-threatening. We investigated the prevalence of parvovirus B19 among patients with beta thalassemia major attending the Zafar Adult Thalassemia Clinic in Tehran, Iran. METHODS: This cross-sectional study was performed to determine the presence of parvovirus B19 DNA in blood samples and parvovirus B19 genotypes in plasma samples of patients with thalassemia major...
March 2017: Blood Research
https://www.readbyqxmd.com/read/28401070/simple-and-easy-to-perform-preimplantation-genetic-diagnosis-for-%C3%AE-thalassemia-major-using-combination-of-conventional-and-fluorescent-polymerase-chain-reaction
#11
Rasoul Salehi, Sharifeh Khosravi, Mansour Salehi, Majid Kheirollahi, Hossein Khanahmad
BACKGROUND: Thalassemias are the most common monogenic disorders in many countries throughout the world. The best practice to control the prevalence of the disease is prenatal diagnosis (PND) services. Extensive practicing of PND proved effective in reducing new cases but on the other side of this success high abortion rate is hided, which ethically unfair and for many couples, especially with a previous experience of a therapeutic abortion, or moral concerns, is not a suitable choice...
2017: Advanced Biomedical Research
https://www.readbyqxmd.com/read/28399703/value-of-speckle-tracking-echocardiography-for-detection-of-clinically-silent-left-ventricular-dysfunction-in-patients-with-%C3%AE-thalassemia
#12
Mozhgan Parsaee, Sedigheh Saedi, Pegah Joghataei, Azita Azarkeivan, Zahra Alizadeh Sani
OBJECTIVE: β-Thalassemia is an inherited hemoglobin disorder resulting in chronic hemolytic anemia requiring chronic transfusion therapy. Cardiac involvement is the main cause of death in patients with thalassemia major. The narrow border is between overt myocardial dysfunction and clinically silent left ventricular (LV) dysfunction in patients with thalassemia. Therefore, we need novel parameters in different imaging techniques to discover cardiac involvement in an early and subtle stage...
April 12, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28399542/presence-of-the-ivs-i-6-mutated-allele-in-beta-thalassemia-major-patients-correlates-with-extramedullary-hematopoiesis-incidence
#13
Nikolaos Sousos, Despoina Adamidou, Philippos Klonizakis, Alexandra Agapidou, Stamatia Theodoridou, Georgios Spanos, Kyriakos Psarras, Evaggelia Vetsiou, Timoleon-Achilleas Vyzantiadis, Efthymia Vlachaki
Extramedullary hematopoiesis (EMH) results from the extension of hematopoietic tissue beyond the confines of the bones. Since the initiation of regular transfusion programs from an early age for all thalassemia major (ΤΜ) patients, EMH has not been considered a clinical issue anymore. The present study aims to record the prevalence of EMH in chronically transfused ΤΜ patients followed at our institution and to investigate possible risk factors associated with its occurrence. The project was designed as a retrospective, nonexperimental, descriptive, exploratory study...
April 12, 2017: Acta Haematologica
https://www.readbyqxmd.com/read/28386676/attitudes-and-beliefs-among-high-and-low-risk-population-groups-towards-%C3%AE-thalassemia-prevention-a-cross-sectional-descriptive-study-from-india
#14
Swati Chawla, Rajnish Kumar Singh, Bhaskar V K S Lakkakula, Raghavendra Rao Vadlamudi
β-thalassemia is an autosomal recessive blood disorder caused by gene mutations that affect all aspects of β-globin production. In majority of Asian countries including India, the frequency of β-thalassemia is closely intertwined with social, cultural, and religious issues of the respective country. Several national level screening programs imparted education regarding β-thalassemia, but follow-up evaluation revealed that education was not effective. It has been hypothesized that the beliefs and attitudes, carrier screening, and education among "high risk communities" will have far-reaching implications towards β-thalassemia prevention in the country...
April 6, 2017: Journal of Community Genetics
https://www.readbyqxmd.com/read/28381365/alpha-thalassemia-major-and-restrictive-foramen-ovale-in-a-preterm-neonate-without-severe-hydrops-fetalis
#15
Shih-Yun Hsu, Shih-Hsiang Cheng, Ren-Huei Fu
No abstract text is available yet for this article.
March 18, 2017: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/28362936/high-frequency-of-blackwater-fever-among-children-presenting-to-hospital-with-severe-febrile-illnesses-in-eastern-uganda
#16
Peter Olupot-Olupot, Charles Engoru, Sophie Uyoga, Rita Muhindo, Alex Macharia, Sarah Kiguli, Robert O Opoka, Samuel Akech, Carolyne Ndila, Richard Nyeko, George Mtove, Julius Nteziyaremye, Martin Chebet, Elizabeth C George, Abdel G Babiker, Diana M Gibb, Thomas N Williams, Kathryn Maitland
Background: In the Fluid Expansion as a Supportive Treatment (FEAST) trial, an unexpectedly high proportion of participants from eastern Uganda presented with blackwater fever (BWF). Methods: We describe the prevalence and outcome of BWF among trial participants and compare the prevalence of 3 malaria-protective red blood cell polymorphisms in BWF cases vs both trial (non-BWF) and population controls. Results: Of 3170 trial participants, 394 (12...
April 1, 2017: Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America
https://www.readbyqxmd.com/read/28361596/a-case-of-fatal-agranulocytosis-that-developed-in-a-patient-with-%C3%AE-thalassemia-major-treated-with-deferiprone
#17
Maria Mainou, Aggeliki Kotsiafti, Philippos Klonizakis, Vasiliki Soulountsi, Chrysoula Apostolou, Kiriakos Psarras, Efthymia Vlachaki
A 29-year-old male with transfusion-dependent β-thalassemia major (β-TM), splenectomized and on chelation therapy with deferiprone (DFP or L1) due to heart and liver hemosiderosis, presented with high fever and agranulocytosis. Deferiprone was discontinued and a broad spectrum antibiotic therapy was started intravenously. The patient remained febrile and showed no recovery of neutrophil count even after the initiation of granulocyte colony-stimulation factor (G-CSF). After 12 days at the hospital, he developed respiratory failure and was transferred to the intensive care unit (ICU) where he developed multi-organ failure and died 3 days later...
November 2016: Hemoglobin
https://www.readbyqxmd.com/read/28361176/electrophysiological-assessment-for-early-detection-of-retinal-dysfunction-in-%C3%AE-thalassemia-major-patients
#18
Maria Dettoraki, Antonis Kattamis, Ioannis Ladas, Konstantinos Maragkos, Chryssanthi Koutsandrea, Klio Chatzistefanou, Konstantinos Laios, Dimitrios Brouzas, Marilita M Moschos
PURPOSE: The purpose of this study was to assess the role of various diagnostic tests in early detection of retinal changes in β-thalassemia major patients. METHODS: Thirty-eight visually asymptomatic β-thalassemia major patients receiving regular blood transfusions and iron-chelation therapy with deferoxamine (group A, n = 13), deferasirox (group B, n = 11) or deferoxamine with deferiprone (group C, n = 14) and fourteen age- and sex- matched healthy individuals were included in the study...
March 30, 2017: Graefe's Archive for Clinical and Experimental Ophthalmology
https://www.readbyqxmd.com/read/28351935/bidirectional-immune-tolerance-in-non-myeloablative-mhc-mismatched-bmt-for-murine-%C3%AE-thalassemia
#19
Shuyu E, Aman Seth, Peter Vogel, Matt Sommers, Taren Ong, Asha B Pillai
Non-myeloablative conditioning using total lymphoid irradiation (TLI) and rabbit anti-thymocyte serum (ATS) (the murine pre-clinical equivalent of anti-thymocyte globulin/ATG) facilitates immune tolerance after bone marrow transplantation (BMT) across major histocompatibility complex (MHC) disparities and may be a useful strategy for non-malignant disorders. We previously reported that donor effector T cell function and GVHD are regulated via recipient invariant natural killer T cell (iNKT cell) IL-4-driven expansion of donor Foxp3(+) naturally occurring Treg...
March 28, 2017: Blood
https://www.readbyqxmd.com/read/28347295/factors-associated-with-anemia-among-sri-lankan-primary-school-children-in-rural-north-central-province
#20
Gayani Shashikala Amarasinghe, Naotunna Palliya Guruge Chamidri Randika Naottunna, Thilini Chanchala Agampodi, Suneth Buddhika Agampodi
BACKGROUND: Despite interventions, childhood anemia is still a major public health problem in low and middle income countries. Purpose of the present study is to determine factors associated with anemia among rural primary school children in Sri Lanka, a country undergoing rapid socioeconomic changes. METHODS: Multi stage cluster sampling was used to select 100 rural schools in NCP and a maximum of 50 children aged 60-131 months were enrolled from each school. Self-administered questionnaires were sent to parents...
March 27, 2017: BMC Pediatrics
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