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thalassemia major

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https://www.readbyqxmd.com/read/28933303/cardioprotective-potential-of-iron-chelators-and-prochelators
#1
Hana Jansova, Tomas Simunek
Heart is a particularly sensitive organ to iron overload and cardiomyopathy due to the excessive cardiac iron deposition causes most deaths in disorders such as beta-thalassemia major. Free or loosely bound iron ions readily cycle between ferrous and ferric states and catalyze Haber-Weiss reaction that yields highly reactive and toxic hydroxyl radicals. Treatment with iron chelators (desferrioxamine, deferiprone, and deferasirox) substantially improved cardiovascular morbidity and mortality in iron overloaded patients...
September 20, 2017: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/28932366/comparison-of-iron-chelation-effects-of-deferoxamine-deferasirox-and-combination-of-deferoxamine-and-deferiprone-on-liver-and-cardiac-t2-mri-in-thalassemia-maior
#2
Shahla Ansari, Azita Azarkeivan, Ghasem Miri-Aliabad, Saeed Yousefian, Tahereh Rostami
BACKGROUND: Cardiac complications due to iron overload are the most common cause of death in patients with thalassemia major. The aim of this study was to compare iron chelation effects of deferoxamine, deferasirox, and combination of deferoxamine and deferiprone on cardiac and liver iron load measured by T2* MRI. METHODS: In this study, 108 patients with thalassemia major aged over 10 years who had iron overload in cardiac T2* MRI were studied in terms of iron chelators efficacy on the reduction of myocardial siderosis...
2017: Caspian Journal of Internal Medicine
https://www.readbyqxmd.com/read/28930976/detection-of-residual-donor-erythroid-progenitor-cells-after-hematopoietic-stem-cell-transplantation-for-patients-with-hemoglobinopathies
#3
Roman Crazzolara, Gabriele Kropshofer, Michael Steurer, Sieghart Sopper, Wolfgang Schwinger
The presence of incomplete chimerism is noted in a large proportion of patients following bone marrow transplant for thalassemia major or sickle cell disease. This observation has tremendous implications, as subsequent therapeutic immunomodulation strategies can improve clinical outcome. Conventionally, polymerase chain reaction-based analysis of short tandem repeats is used to identify chimerism in donor-derived blood cells. However, this method is restricted to nucleated cells and cannot distinguish between dissociated single-cell lineages...
September 6, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/28929945/innovative-pcr-without-dna-extraction-for-african-sickle-cell-disease-diagnosis
#4
L Detemmerman, S Olivier, V Bours, F Boemer
OBJECTIVES: Hemoglobin (Hb) disorders consist of thalassemia and Hb structural variants, of which the major forms are associated with severe anemia and/or vascular occlusion. Current diagnostic techniques are highly accurate and mostly based on isoelectric focusing, high-performance liquid chromatography or mass spectrometry, which often require advanced laboratory equipment. In sub-Saharan Africa, the Hb disorders are mainly associated to the pathological variants hemoglobin S (HbS) and HbC...
September 20, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28929515/treatment-of-hepatitis-c-virus-infection-with-direct-acting-antiviral-drugs-is-safe-and-effective-in-patients-with-hemoglobinopathies
#5
Raffaella Origa, Maria Laura Ponti, Aldo Filosa, Alfonso Galeota Lanza, Antonio Piga, Giorgio Maria Saracco, Valeria Pinto, Antonino Picciotto, Paolo Rigano, Salvatore Madonia, Rosamaria Rosso, Domenico D'Ascola, Maria Domenica Cappellini, Roberta D'Ambrosio, Immacolata Tartaglione, Lucia De Franceschi, Barbara Gianesin, Vito Di Marco, Gian Luca Forni
Progression of liver fibrosis in patients with hemoglobinopathies is strongly related to the severity of iron overload and the presence of chronic hepatitis C virus (HCV) infection. Effective iron chelation therapy and HCV infection eradication may prevent liver complications. The European Association for the Study of the Liver guidelines recommend interferon-free regimens for the treatment of HCV infection in patients with hemoglobinopathies. However, data regarding the use of direct-acting antiviral drugs (DAAs) in this patient population are few...
September 20, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28904678/-epidemiological-profile-of-hemoglobinopathies-a-cross-sectional-and-descriptive-index-case-study
#6
Fatima Dahmani, Souad Benkirane, Jaafar Kouzih, Aziz Woumki, Hassan Mamad, Azlarab Masrar
Hemoglobinopathies are congenital disorders resultimg from hemoglobin abnormalities. Major forms are often severe, their management is difficult and associated with a great psychosocial impact on patients and their families. They are classified as rare diseases and are still insufficiently known by health professionals. This lack of knowledge is at the origin of diagnostic errors, delay in their management and therefore high morbidity and mortality rate for these patients. In 2008, the World Health Organization (WHO) has published data on hemoglobinopathies epidemiology: more than 330...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28900315/efficacy-and-safety-of-deferasirox-in-pediatric-patients-of-thalassemia-at-a-tertiary-care-teaching-hospital
#7
Dhaval R Thakor, Chetna K Desai, Jigar D Kapadia, Ram K Dikshit, K M Mehariya
OBJECTIVE: To evaluate efficacy, safety and utilization pattern of deferasirox in paediatric patients of transfusion dependant β Thalassemia Major at a tertiary care teaching hospital in Gujarat. MATERIALS AND METHODS: This observational, prospective-retrospective, single centre, continuous study was conducted in a tertiary care teaching hospital among paediatric patients of transfusion dependent β Thalassemia Major. Patients treated with deferasirox for not more than 12 weeks were enrolled...
April 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/28882060/mri-assessment-of-pituitary-iron-accumulation-by-using-pituitary-r2-in-%C3%AE-thalassemia-patients
#8
Mustafa Bozdağ, Selen Bayraktaroğlu, Yeşim Aydınok, Mehmet Cem Çallı
Background Patients with thalassemia major (TM) require repeated blood transfusions, which leads to accumulation of iron in a wide variety of tissues. Accumulation of iron in the pituitary gland can lead to irreversible hypogonadotropic hypogonadism (HH) in this group of patients. Purpose To investigate the reliability of pituitary-R2 as a marker to estimate the extent of pituitary iron load by comparing the pituitary magnetic resonance imaging (MRI) findings with hepatic iron load and serum ferritin levels...
January 1, 2017: Acta Radiologica
https://www.readbyqxmd.com/read/28881347/benefits-of-curcumin-supplementation-on-antioxidant-status-in-%C3%AE-thalassemia-major-patients-a-double-blind-randomized-controlled-clinical-trial
#9
Esmat Nasseri, Elahe Mohammadi, Ahmad Tamaddoni, Durdi Qujeq, Farid Zayeri, Hamid Zand
BACKGROUND: β-Thalassemia major, the most common inherited anemia in the world, is associated with imbalance in the oxidant-antioxidant system. The objective of this study was to evaluate the efficacy of curcumin supplementation on markers of oxidative stress in patients with β-Thalassemia. METHODS: This double-blind randomized controlled clinical trial was performed on 61 β-thalassemia major patients. Subjects in the curcumin group received two 500 mg curcumin capsules daily and patients in the placebo group took 2 placebo capsules daily for 12 weeks...
September 8, 2017: Annals of Nutrition & Metabolism
https://www.readbyqxmd.com/read/28875336/progression-of-liver-fibrosis-can-be-controlled-by-adequate-chelation-in-transfusion-dependent-thalassemia-tdt
#10
D Maira, E Cassinerio, A Marcon, M Mancarella, M Fraquelli, P Pedrotti, M D Cappellini
A substantial proportion of patients with transfusion-dependent beta-thalassemia major suffer from chronic liver disease. Iron overload resulting from repeated transfusions and HCV infection has been implicated in the development of liver fibrosis. Hepatic siderosis and fibrosis were assessed in 99 transfusion-dependent thalassemia (TDT) patients using transient elastography (TE) and liver iron concentration (LIC) assessed by T2*MRI at baseline and after 4 years. Data were analyzed retrospectively. At baseline, the overall mean liver stiffness measurement (LSM) was 7...
September 5, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28875008/renal-hemosiderosis-among-iranian-transfusion-dependent-%C3%AE-thalassemia-major-patients
#11
Mozhgan Hashemieh, Mitra Radfar, Azita Azarkeivan, Seyed Mohammad Taghi Hosseini Tabatabaei, Sedigheh Nikbakht, Mehdi Yaseri, Kourosh Sheibani
Background: In recent years, the success in management of thalassemic patients, has allowed for some previously unrecognized complications including renal abnormalities to emerge. This prospective study aimed to investigate kidney iron overload by means of MRI T2* and also renal function based on laboratory tests for early markers of glomerular and tubular dysfunction among adult Iranian transfusion-dependent thalassemia major patients. Subjects and Methods: Two-hundred and two patients with transfusion-dependent β-thalassemia major were included in this study in Zafar Adult Thalassemia Center, Tehran, Iran...
April 1, 2017: International Journal of Hematology-oncology and Stem Cell Research
https://www.readbyqxmd.com/read/28859037/health-related-quality-of-life-and-associated-factors-among-thalassemia-major-patients-southeast-of-iran
#12
Ali Bazi, Omolbanin Sargazi-Aval, Amin Safa, Ebrahim Miri-Moghaddam
Low quality of life (QOL) is a feature that has been overlooked in thalassemia major (TM) patients. Our aim was to assess QOL in school-aged TM patients in Zabol city and surrounding rural areas in southeast of Iran. The study was performed in 2014. QOL was evaluated using Pediatric Quality of Life Inventory 4 (PedsQL4) questionnaire addressing physical, emotional, social, and educational, along with psychological health in 80 TM patients. Also, 80 age-matched and sex-matched subjects without any chronic illness served as control group...
August 30, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28845843/spinal-cord-compression-secondary-to-extramedullary-hematopoiesis-a-rareness-in-a-young-adult-with-thalassemia-major
#13
Shehab Fareed, Ashraf T Soliman, Vincenzo De Sanctis, Samah Kohla, Dina Soliman, Diala Khirfan, Adriana Tambuerello, Mohamed Talaat, Abdulqadir Nashwan, Palmira Caparrotti, Mohamed A Yassin
We report a case of a thalassemia major male patient with back pain associated to severe weakness in lower extremities resulting in the ability to ambulate only with assistance. An urgent magnetic resonance imaging (MRI) of  thoracic and lumbosacral spine was requested. A posterior intraspinal extradural mass lesion compressing the spinal cord at the level of thoracic T5-8 was present, suggesting an extramedullary hematopoietic centre, compressing the spinal cord. He was treated successfully with thalassemia major alone...
August 23, 2017: Acta Bio-medica: Atenei Parmensis
https://www.readbyqxmd.com/read/28845406/in-vitro-hb-production-in-b-thalassemia-patients-is-not-a-predictor-of-clinical-responsiveness-to-hydroxyurea
#14
Mohammad Reza Mahdavi, Farzin Pourfarzad, Mehrnoush Kosaryan, Mohammad Taghi Akbari
BACKGROUND: The hematologic response to hydroxyurea (HU) is varied among β-thalassemia (BT) patients. The BCL11A and SOX6 genes are involved in response to HU. This study aimed to investigate the in-vitro responsiveness of HU among BT major patients homozygote for IVSII-1G>A mutation and XmnI single nucleotide polymorphism (SNP) in order to find whether the in-vitro Hb concentration is a predictor of clinical (HU) responsiveness. METHODS: In this case-control study, twenty BT patients homozygote for IVSII-1G>A mutation and XmnI SNP from Thalassemia Research Center, Sari, Iran in 2015 were selected and categorized into two groups of 10 Responder (R) and 10 Non-Responder (NR) according to their clinical HU response...
July 2017: Iranian Journal of Public Health
https://www.readbyqxmd.com/read/28836463/prevalence-incidence-trend-and-complications-of-thalassemia-in-iraq
#15
Kamal A Kadhim, Kadhim H Baldawi, Faris H Lami
Globally, thalassemia is the most common hereditary hemoglobinopathy, and occurs in 4.4/10,000 live births. In the developing world, the majority of patients die before the age of 20 years. In Iraq, there is little data on the epidemiology and burden of thalassemia. The objectives of this study were to determine the prevalence, incidence, trend, and complications of thalassemia patients in Iraq. All thalassemia patients registered in the accessible 16 (of the 19) thalassemia centers in Iraq until December 31 2015, were included...
August 24, 2017: Hemoglobin
https://www.readbyqxmd.com/read/28818403/heavy-metal-levels-in-patients-with-ineffective-erythropoiesis
#16
Turan Bayhan, Şule Ünal, Eyüp Çırak, Onur Erdem, Cemal Akay, Orhan Gürsel, İbrahim Eker, Erdem Karabulut, Fatma Gümrük
OBJECTIVES: Iron is taken into enterocytes at the duodenum via apical divalent metal-ion transporter 1 protein. Besides iron, divalent metal-ion transporter 1 also transports other divalent metals. We aimed to investigate blood heavy metal levels in patients with ineffective erythropoiesis. METHODS: Blood levels of heavy metals including Pb, Al, Cd, Cr, Co, Cu, and Zn were measured in patients with thalassemia major (TM), thalassemia intermedia (TI), congenital dyserythropoietic anemia (CDA), and age- and sex-matched healthy controls...
August 2, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28815805/intracellular-iron-overload-leading-to-dna-damage-of-lymphocytes-and-immune-dysfunction-in-thalassemia-major-patients
#17
Jyoti Shaw, Ayan Chakraborty, Arijit Nag, Arnab Chattopadyay, Anjan Kr Dasgupta, Maitreyee Bhattacharyya
OBJECTIVES: To investigate the cause and effects of intracellular iron overload in lymphocytes of thalassemia major patients. METHODS: 66 thalassemia major patients having iron overload and 10 age matched controls were chosen for the study. Blood sample was collected and serum ferritin, oxidative stress, lymphocyte DNA damage were examined as well as infective episodes were also counted. RESULTS: Case-control analysis revealed significant oxidative stress, iron overload, DNA damage and rate of infections in thalassemia cases as compared to controls...
August 16, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28811695/pai-1-study-in-thalassemia-major-patients-receiving-multiple-blood-transfusion
#18
Ashwini Kumar, H S Batra, Mithu Banerjee, S Bandyopadhyay, T K Saha, Pratibha Misra, Vivek Ambade
Thalassemia is a congenital hemolytic disease which is treated by repeated blood transfusion. Chronic iron overload is currently considered to be the primary cause of mortality in β-thalassemia, mainly due to the induction of left-sided cardiac failure. Iron overload results from a number of mechanisms associated with the disease itself. In addition to chronic iron overload thalassemic patients are more prone for procoagulant status which in turn lead to clinical thrombotic events. The hypercoagulable state in thalassemia is due to multiple elements, a combination of which is often the drive behind a clinical thromboembolic events...
July 2017: Indian Journal of Clinical Biochemistry: IJCB
https://www.readbyqxmd.com/read/28777254/t-type-and-l-type-calcium-channel-blockers-for-the-treatment-of-cardiac-iron-overload-an-update
#19
Sirinart Kumfu, Siriporn C Chattipakorn, Nipon Chattipakorn
In thalassemia patients iron overload cardiomyopathy is a major cause of cardiac dysfunction and mortality. Despite many advances in the development of new iron chelating agents, heart failure still occurs in some patients and can lead to an increase in mortality rate. Recently, potential novel therapeutic strategies in the treatment of these patients have focused on L-type and T-type calcium channel blockers. These two channels have been reported as being the main routes for cardiac iron uptake under conditions of iron overload...
August 4, 2017: Journal of Cardiovascular Pharmacology
https://www.readbyqxmd.com/read/28771834/molecular-prenatal-diagnosis-of-alpha-and-beta-thalassemia-in-pregnant-hakka-women-in-southern-china
#20
Pingsen Zhao, Heming Wu, Zhixiong Zhong, Liubing Lan, Mei Zeng, Hualan Lin, Huaxian Wang, Zhiyuan Zheng, Luxian Su, Wei Guo
BACKGROUND: To date, there has been no systematic study of DNA-based prenatal diagnosis of thalassemia in pregnant Hakka women in southern China. METHODS: A total of 279 pregnant Hakka women with confirmed cases of thalassemia who had been treated at the Meizhou People's Hospital in China's Guangdong Province from January 2014 to December 2016 were here enrolled. Genomic DNA was extracted from peripheral blood of couples and villus, amniotic fluid, or fetal cord blood...
August 3, 2017: Journal of Clinical Laboratory Analysis
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