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thalassemia major

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https://www.readbyqxmd.com/read/29779447/development-of-postmeiotic-cells-in-vitro-from-spermatogonial-cells-of-prepubertal-cancer-patients
#1
Maram Abofoul-Azab, Ali AbuMadigem, Eitan Lunenfeld, Joseph Kapelushnik, Qinghua Shi, Haim Pinkas, Mahmoud Huleihel
Aggressive chemotherapy in childhood often results in testicular damage and consequently jeopardizes future fertility. The presence of spermatogonial cells (SPGCs) in the testes of prepubertal cancer patient boys (PCPBs) can be used to develop future strategies for male fertility preservation. In the present study, we examined the presence of SPGCs in testes of chemotherapy-treated PCPBs and their ability to develop spermatogenesis in vitro using a three-dimensional (3D) culture system. Seven testicular biopsies were obtained from chemotherapy-treated PCPBs and one from a patient with β-thalassemia major...
May 19, 2018: Stem Cells and Development
https://www.readbyqxmd.com/read/29762364/seroprevalence-of-toxoplasma-gondii-infection-among-%C3%AE-thalassemia-major-pediatric-population-implications-for-transfusion-transmissible-toxoplasmosis
#2
Nora El-Tantawy, Ahmad Darwish, Eman Eissa
BACKGROUND: Children with β-thalassemia major who regularly receive blood transfusion are at risk of developing transfusion-transmitted infection. Toxoplasmosis is a common and a serious parasitic disease with high prevalence and could be transmitted through blood transfusion from healthy asymptomatic donors. However, screening Toxoplasma gondii before blood donation has not been considered. OBJECTIVE: To determine the prevalence of T. gondii antibodies among thalassemia children undergoing blood transfusion...
May 14, 2018: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/29761371/tolerability-and-efficacy-of-deferasirox-in-patients-with-transfusional-iron-overload-results-from-a-german-2-year-non-interventional-study
#3
Florian Nolte, Holger Nückel, Burkhard Schmidt, Thomas Geer, Oleg Rubanov, Holger Hebart, Andrea Jarisch, Stefan Albrecht, Christiane Johr, Christiane Schumann, Wolf-Karsten Hofmann
BACKGROUND: Iron overload (IOL) due to repetitive transfusions of packed red blood cells (pRBC) has a major impact on morbidity and mortality in patients with inherited bone marrow failure syndromes and hemoglobinopathies such as thalassemia and sickle cell disease. However, whether IOL influences the outcome of elderly patients with myeloid malignancies is not yet clear. Moreover, clinical trials have reported high drop-out rates during treatment with the oral iron chelator deferasirox (DFX)...
May 14, 2018: Journal of Cancer Research and Clinical Oncology
https://www.readbyqxmd.com/read/29755708/growth-and-endocrine-function-in-tunisian-thalassemia-major-patients
#4
Naouel Guirat Dhouib, Monia Ben Khaled, Monia Ouederni, Habib Besbes, Ridha Kouki, Fethi Mellouli, Mohamed Bejaoui
β-thalassemia major (β-TM) is among the most common hereditary disorders imposing high expenses on health-care system worldwide. The patient's survival is dependent on lifetime blood transfusion which leads to iron overload and its toxicity in various organs including endocrine glands. This article provides an overview of endocrine disorders in beta-TM patients. This single center investigation enrolled 28 β-TM patients (16 males, 12 females) regularly transfused with packed red cell since early years of life...
2018: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/29754517/diagnosis-and-treatment-of-cardiac-iron-overload-in-transfusion-dependent-thalassemia-patients
#5
Natthaphat Siri-Angkul, Siriporn C Chattipakorn, Nipon Chattipakorn
Thalassemia is among the most common genetic diseases. Patients with severe forms of the disease are transfusion-dependent, leading to iron overload. A condition which can eventually develop in the iron-loaded heart is iron overload cardiomyopathy, a debilitating disease that accounts for the majority of deaths in thalassemia patients. Areas covered: This review article provides a comprehensive summary of the diagnosis and treatment of cardiac iron overload in transfusion-dependent thalassemia patients, with discussion covering current weak points and potential improvements of the relevant diagnostic and therapeutic strategies...
May 12, 2018: Expert Review of Hematology
https://www.readbyqxmd.com/read/29743790/safety-and-efficacy-of-sofosbuvir-and-daclatasvir-for-hepatitis-c-virus-infection-in-patients-with-%C3%AE-thalassemia-major
#6
Rajiv Mehta, Mayank Kabrawala, Subhash Nandwani, Pankaj Desai, Vishwa Bhayani, Sanjay Patel, Viral Parekh
Background and Aims: β-thalassemia major patients are susceptible to Hepatitis C Virus (HCV) infection owing to life-long dependency for blood-transfusion. Moreover, this patient population is at risk of progression of liver fibrosis or development of cirrhosis as a consequence of both iron overload and HCV infection. Hence, this study was carried out to evaluate efficacy and safety of the combination regimen of sofosbuvir and daclatasvir for HCV infection in β-thalassemia major patients...
March 2018: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/29742871/the-effect-of-abo-blood-groups-hemoglobinopathy-and-heme-oxygenase-1-polymorphisms-on-malaria-susceptibility-and-severity
#7
Jiraporn Kuesap, Kesara Na-Bangchang
Malaria is one of the most important public health problems in tropical areas on the globe. Several factors are associated with susceptibility to malaria and disease severity, including innate immunity such as blood group, hemoglobinopathy, and heme oxygenase-1 (HO-1) polymorphisms. This study was carried out to investigate association among ABO blood group, thalassemia types and HO-1 polymorphisms in malaria. The malarial blood samples were collected from patients along the Thai-Myanmar border. Determination of ABO blood group, thalassemia variants, and HO-1 polymorphisms were performed using agglutination test, low pressure liquid chromatography and polymerase chain reaction, respectively...
April 2018: Korean Journal of Parasitology
https://www.readbyqxmd.com/read/29737797/thalassemia-and-other-hemoglobinopathies-among-anemic-individuals-in-metro-manila-philippines-and-their-intake-of-iron-supplements
#8
Mario V Capanzana, Ma Angelina L Mirasol, Geoffry Smith, Imelda Angeles-Agdeppa, Leah Perlas, Francisco de Los Reyes, Maria Sofia Amarra
BACKGROUND AND OBJECTIVES: Iron deficiency is the most common cause of anemia worldwide. In Southeast Asia, studies showed that genetic hemoglobin disorders also contribute significantly to the burden of anemia. The study aimed to estimate the proportion of thalassemia and other hemoglobinopathies versus iron deficiency and other causes in a sample of anemic individuals; describe the characteristics of thalassemic subjects in terms of severity of anemia, adequacy of iron stores, and hematological profile; examine the intake of iron supplements among individuals with varying causes of anemia...
2018: Asia Pacific Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/29729700/fc%C3%AE-receptor-iii-expression-and-morphological-maturity-on-neutrophil-are-associated-with-higher-iron-level-of-major-beta-thalassemia
#9
Mohammad Ghozali, Tiwi Harjanti Cakranita, Adi Imam Tjahjadi, Lelani Reniarti, Reni Ghrahani, Mraa Syamsunarno, Budi Setiabudiawan, Ramdan Panigoro
Lifetime blood transfusion experienced by major β-thalassemia patients complicated with iron overload, therefore, may lead to their tissue injury. Ultimately, free toxic iron may alter immune response via dysregulation of immune cell activity producing prolonged effector reaction. Neutrophil as one of the vital innate immune cell despite serves as the first line of defense resulting acute inflammation has a pivotal role in chronic inflammation while releasing the toxic substance that interferes biological processes...
April 30, 2018: Cellular and Molecular Biology
https://www.readbyqxmd.com/read/29726078/unrelated-and-related-donor-transplantation-for-beta-thalassemia-major-a-single-center-experience-from-india
#10
M Joseph John, Amrith Mathew, Chepsy C Philip, Sohan Singh, Tanuja Tanuja, Naveen Kakkar
Hematopoietic stem cell transplantation (HSCT) remains the only curative treatment in patients with β-thalassemia major. A matched sibling or a related donor is usually found in only 25%-30% of the patients. There are limited data on matched unrelated donor (MUD) transplants from India. We reviewed HSCT outcome in 56 children with TM who underwent 57 transplants at our center. Related donor (RD) (n=43) and MUD (n=14) transplants were performed with TreoFluT-based conditioning regimen in majority (95%) of patients...
May 3, 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29716764/significantly-higher-frequencies-of-hemoglobin-iron-vitamin-b12-and-folic-acid-deficiencies-and-of-hyperhomocysteinemia-in-patients-with-behcet-s-disease
#11
Ying-Shiung Kuo, Julia Yu-Fong Chang, Yi-Ping Wang, Yang-Che Wu, Yu-Hsueh Wu, Andy Sun
BACKGROUND/PURPOSE: Behcet's disease (BD) patients may have one or more hematinic deficiencies. This study evaluated whether there were significantly higher frequencies of hemoglobin, iron, vitamin B12, and folic acid deficiencies and of hyperhomocysteinemia in BD patients. METHODS: The blood hemoglobin, iron, vitamin B12, folic acid, and homocysteine concentrations in 63 BD patients were measured and compared with the corresponding levels in 126 age- and sex-matched healthy control subjects...
April 28, 2018: Journal of the Formosan Medical Association, Taiwan Yi Zhi
https://www.readbyqxmd.com/read/29695942/genotype-phenotype-correlation-among-beta-thalassemia-and-beta-thalassemia-hbe-disease-in-thai-children-predictable-clinical-spectrum-using-genotypic-analysis
#12
Chanchai Traivaree, Chalinee Monsereenusorn, Piya Rujkijyanont, Warakorn Prasertsin, Boonchai Boonyawat
Introduction: Beta-thalassemia is a group of inherited hemolytic anemias and one of the most common genetic disorders in Thailand. The clinical spectrum of beta-thalassemia disease ranges from mild to severe clinical symptoms including mild beta-thalassemia intermedia (TI) and severe beta-thalassemia major (TM). Objective: This study aimed to determine the correlation between beta-globin gene ( HBB ) mutations and their phenotypic manifestations by evaluating patients' clinical characteristics, transfusion requirements, growth and hematologic parameters, and hemoglobin typing among pediatric patients treated at Phramongkutklao Hospital...
2018: Journal of Blood Medicine
https://www.readbyqxmd.com/read/29682246/distribution-of-hepatitis-c-virus-genotypes-in-patients-with-major-%C3%AE-thalassemia-in-mashhad-northeast-iran
#13
Sanaz Ahmadi-Ghezeldasht, Zahra Badiei, Hamid Reza Sima, Mohammad Reza Hedayati-Moghaddam, Meysam Habibi, Mohsen Khamooshi, Ahmad Azimi
BACKGROUND Hepatitis C virus (HCV) is considered to be the major cause of post-transfusion hepatitis in patients with thalassemia. We aimed to determine the HCV prevalence, genotypes, and viral load among patients with major β-thalassemia in Mashhad, Iran. METHODS Medical records of all 550 patients with major β-thalassemia who referred to ThalassemiaHemophilia Center of Mashhad (Sarvar Clinic) were reviewed from October to November 2011. Plasma samples of the patients were tested for the presence of anti-HCV antibodies by enzyme linked immunosorbent assay...
January 2018: Middle East Journal of Digestive Diseases
https://www.readbyqxmd.com/read/29675595/involvement-of-cytosolic-and-mitochondrial-iron-in-iron-overload-cardiomyopathy-an-update
#14
REVIEW
Richard Gordan, Suwakon Wongjaikam, Judith K Gwathmey, Nipon Chattipakorn, Siriporn C Chattipakorn, Lai-Hua Xie
Iron overload cardiomyopathy (IOC) is a major cause of death in patients with diseases associated with chronic anemia such as thalassemia or sickle cell disease after chronic blood transfusions. Associated with iron overload conditions, there is excess free iron that enters cardiomyocytes through both L- and T-type calcium channels thereby resulting in increased reactive oxygen species being generated via Haber-Weiss and Fenton reactions. It is thought that an increase in reactive oxygen species contributes to high morbidity and mortality rates...
April 19, 2018: Heart Failure Reviews
https://www.readbyqxmd.com/read/29673692/cost-effectiveness-of-hematopoietic-stem-cell-transplantation-compared-to-transfusion-chelation-for-treatment-of-thalassemia-major
#15
M Joseph John, Gaurav Jyani, Aikaj Jindal, Ranjeet Singh Mashon, Amrith Mathew, Shruti Kakkar, Pankaj Bahuguna, Shankar Prinja
Hematopoietic Stem Cell Transplant (HSCT) is the only cure for thalassaemia major (TM) which inflicts a significant one-time cost. Hence, it is important to explore the cost-effectiveness of HSCT versus lifelong regular transfusion chelation (TC) therapy. This study was undertaken to estimate incremental cost per quality adjusted life year (QALY) gained with the intervention group HSCT, and the comparator group TC, in TM patients. A combination of decision tree and Markov model was used for analysis. Hospital database, supplemented with review of published literature were used to derive input parameters for the model...
April 16, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29668553/glucose-homeostasis-markers-in-beta-thalassemia
#16
Sunil Gomber, Anjali Bagaria, Sri V Madhu, Pooja Dewan
BACKGROUND: Glycosylated hemoglobin (HbA1c) has been a well-recognized marker of glucose homeostasis among thalassemics. Recently some studies have proposed the role of fructosamine as a better marker as compared with HbA1c. Hence, the study was carried out to find out which marker holds promise among Indian beta-thalassemic children. METHODS: In this case-control study, 60 diagnosed cases of beta-thalassemia major and equal number of normal controls who were ≥8 years of age were enrolled...
April 17, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29664655/short-term-assessment-of-hsct-effects-on-the-hypothalamus-pituitary-axis-in-pediatric-thalassemic-patients
#17
Amir Ali Hamidieh, Fariba Mohseni, Maryam Behfar, Zohreh Hamidi, Kamran Alimoghaddam, Mohamad Pajouhi, Bagher Larijani, Mohammad-Reza Mohajeri-Tehrani, Ardeshir Ghavamzadeh
BACKGROUND: Beta thalassemia major (BTM) and its treatment by hematopoietic stem cell transplantation (HSCT) may have deleterious effects on the endocrine systems. We assessed endocrine complications of HSCT in pediatric patients for 3 months. METHODS: In 20 (6 female) pediatric major thalassemic patients (mean age of 10.8 ± 3.9 years old), prolactin, luteinizing hormone (LH), follicle-stimulating hormone (FSH), T4, T3, thyroid-stimulating hormone (TSH), IGF-1, testosterone (in males) or estradiol (in females) were measured as a batch at the Endocrinology and Metabolism Research Center (EMRC) of Tehran University of Medical Sciences (TUMS) laboratories before HSCT and 1 and 3 months afterwards...
February 1, 2018: Archives of Iranian Medicine
https://www.readbyqxmd.com/read/29663858/assessment-of-liver-and-cardiac-iron-overload-using-mri-in-patients-with-chronic-anemias-in-latin-american-countries-results-from-asimila-study
#18
Rodolfo Cancado, Nora P Watman, Clarisse Lobo, Zulay Chona, Fernando Manzur, Fabiola Traina, Miriam Park, Guillermo Drelichman, Juan Pablo Zarate, Luis Marfil
OBJECTIVES: A multicenter, noninterventional, observational study was conducted in the Latin American countries including Argentina, Brazil, Colombia, Mexico, and Venezuela to assess the prevalence of liver and cardiac iron overload using magnetic resonance imaging (MRI) in patients with chronic anemias except thalassemia. METHODS: Patients aged >10 years with transfusion-dependent anemias, except thalassemia, either with <20 units of red blood cell (RBC) transfusions with serum ferritin (SF) levels >2000 ng/mL or with ≥20 units of RBC transfusions regardless of SF level in their lifetime, were enrolled...
April 17, 2018: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/29663590/use-of-deferasirox-exjade-for-iron-overload-in-peritoneal-dialysis-patients
#19
Erwin Yii, James Cg Doery, Zane Kaplan, Peter G Kerr
BACKGROUND: A 54 year old male with β-Thalassemia major developed ESRD and was managed with continuous ambulatory peritoneal dialysis. Although not able to be transfused due to high titre red cell antibodies he did require management of iron overload. Deferasirox (Exjade) was administered orally. There was concern that excretion of iron via the peritoneal dialysate may raise the risk of iron-dependent infections (Yersinia and Rhizopus). METHODS: Whilst receiving Exjade 1000mg /day, a total collection of 12...
April 16, 2018: Nephrology
https://www.readbyqxmd.com/read/29663188/magnetic-resonance-imaging-during-management-of-patients-with-transfusion-dependent-thalassemia-a-single-center-experience
#20
Zeynep Karakas, Yasin Yilmaz, Zuhal Bayramoglu, Serap Karaman, Selime Aydogdu, Ayse Ozkan Karagenc, Deniz Tugcu, Memduh Dursun
BACKGROUND: Cardiac and hepatic magnetic resonance imaging evaluation during treatment can tailor physicians' chelation therapy titrations. AIM: The aim of the study was to assess the relationship of cardiac and hepatic T2* values with chelation therapy in patients with transfusion-dependent thalassemia (TDT). METHODS: A total of 106 patients with TDT who were followed up in Istanbul Medical Faculty Thalassemia Center were evaluated for the study...
April 16, 2018: La Radiologia Medica
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