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thalassemia major

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https://www.readbyqxmd.com/read/29350657/an-icet-a-survey-on-hypoparathyroidism-in-patients-with-thalassaemia-major-and-intermedia-a-preliminary-report
#1
Vincenzo De Sanctis, Ashraf T Soliman, Duran Canatan, Heba Elsedfy, Mehran Karimi, Shahina Daar, Hala Rimawi, Soteroula Christou, Nicos Skordis, Ploutarchos Tzoulis, Praveen Sobti, Shruti Kakkar, Yurdanur Kilinc, Doaa Khater, Saif A Alyaarubi, Valeriya Kaleva, Su Han Lum, Mohamed A Yassin, Forough Saki, Maha Obiedat, Salvatore Anastasi, Maria Concetta Galati, Giuseppe Raiola, Saveria Campisi, Nada Soliman, Mohamed Elshinawy, Soad Al Jaouni, Salvatore Di Maio, Yasser Wali, Ihab Zaki Elhakim, Christos Kattamis
Hypoparathyroidism (HPT) is a rare disease with leading symptoms of hypocalcemia, associated with high serum phosphorus levels and absent or inappropriately low levels of parathyroid hormone (PTH). In patients with thalassemias it is mainly attributed to transfusional iron overload, and suboptimal iron chelation therapy. The main objectives of this survey were to provide data on the prevalence, demographic and clinical features of HPT in thalassemia major (TM) and intermedia (TI) patients living in different countries, and to assess its impact in clinical medical practice...
January 16, 2018: Acta Bio-medica: Atenei Parmensis
https://www.readbyqxmd.com/read/29344533/the-effect-of-family-centered-empowerment-program-on-self-efficacy-of-adolescents-with-thalassemia-major-a-randomized-controlled-clinical-trial
#2
Leili Borimnejad, Soroor Parvizy, Hamid Haghaani, Belgheis Sheibani
Background: Chronic nature of thalassemia causes changes in different aspects of life in patients, including their self-efficacy. The aim of this study was to determine the effect of family-centered empowerment program on the self-efficacy of adolescents with Thalassemia major. Methods: A quasi-experimental study was performed on adolescents with thalassemia major in 2013 in Bandar Abbas, Iran. The participants were divided into intervention and control groups, respectively...
January 2018: International Journal of Community Based Nursing and Midwifery
https://www.readbyqxmd.com/read/29340122/a-cross-sectional-study-of-glycemic-status-and-zinc-level-in-patients-with-beta-thalassemia-major
#3
Mohammad Ali Mashhadi, Zahra Sepehri, Zahra Heidari, Mahmoud Ali Kaykhaei, Aliyeh Sargazi, Farhad Kohan, Hanieh Heidari
Background: Endocrinopathies and diabetes mellitus are prevalent in patients with beta-thalassemia major Recently some studies demonstrate a link between low levels of serum zinc level and higher prevalence of diabetes. The aim of this study was to evaluate the glucose tolerance in patients suffered from beta-thalassemia major and determine the association of Homeostasis Model Assessment (HOMA) parameters with zinc status among these patients. Materials andMethods: In this cross sectional study, clinical data of patients who were suffered from thalassemia major, aged≥10 years were collected...
October 1, 2017: International Journal of Hematology-oncology and Stem Cell Research
https://www.readbyqxmd.com/read/29337597/hydroxyurea-for-lifelong-transfusion-dependent-%C3%AE-thalassemia-a-meta-analysis
#4
Ali H Algiraigri, Nicola A M Wright, Elizabeth Oddone Paolucci, Aliya Kassam
OBJECTIVE: Chronic blood transfusion remains the most feasible therapeutic option for lifelong transfusion-dependent β-thalassemia (lifelong TDβT). However, it is associated with serious risks and complications. Hydroxyurea (HU), an oral chemotherapeutic drug, is expected to increase hemoglobin levels, thereby minimizing the burden of blood transfusion and its complications. Growing literature over the last twenty years suggests promising results of the use HU in lifelong TDβT; however, its role and safety remain unanswered questions...
January 16, 2018: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29326805/detection-of-%C3%AE-thalassemia-carriers-by-red-cell-parameters-obtained-from-automatic-counters-using-mathematical-formulas
#5
Idit Lachover Roth, Boaz Lachover, Guy Koren, Carina Levin, Luci Zalman, Ariel Koren
Background: β-thalassemia major is a severe disease with high morbidity. The world prevalence of carriers is around 1.5-7%. The present study aimed to find a reliable formula for detecting β-thalassemia carriers using an extensive database of more than 22,000 samples obtained from a homogeneous population of childbearing age women with 3161 (13.6%) of β-thalassemia carriers and to check previously published formulas. Methods: We applied a mathematical method based on the support vector machine (SVM) algorithm in the search for a reliable formula that can differentiate between thalassemia carriers and non-carriers, including normal counts or counts suspected to belong to iron-deficient women...
2018: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/29318368/increased-levels-of-advanced-glycation-end-products-positively-correlate-with-iron-overload-and-oxidative-stress-markers-in-patients-with-%C3%AE-thalassemia-major
#6
Maryam Sadat Mirlohi, Hamid Yaghooti, Saeed Shirali, Ali Aminasnafi, Samaneh Olapour
The impaired biosynthesis of the β-globin chain in β-thalassemia leads to the accumulation of unpaired alpha globin chains, failure in hemoglobin formation, and iron overload due to frequent blood transfusion. Iron excess causes oxidative stress and massive tissue injuries. Advanced glycation end products (AGEs) are harmful agents, and their production accelerates in oxidative conditions. This study was conducted on 45 patients with major β-thalassemia who received frequent blood transfusions and chelation therapy and were compared to 40 healthy subjects...
January 9, 2018: Annals of Hematology
https://www.readbyqxmd.com/read/29311011/-screening-for-nephropathy-in-major-sickle-cell-syndromes-in-patients-monitored-at-the-national-reference-center-for-sickle-cell-disease-in-niamey-niger
#7
Moumouni Garba, Zeinabou Maiga Moussa Tondi, Hassan Diongoule, Samaila Aboubacar, Abdou Ide, Nadège Ruddy Biyao-Nelson, Illiassou Soumaila, Soumana Alido
BACKGROUND: Sickle cell anemia is the most common hereditary hemopathy in the world. It is a disease that attacks all the systems of the organism. The kidneys are among the most sensitive organs of this disease. The main objective of this study is to detect sickle cell nephropathy in patients followed at the National Reference Center for Sickle Cell Disease in Niamey. METHODS: It is a prospective study carried out over a period of one year (January to December 2016)...
January 5, 2018: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/29296892/allogeneic-bone-marrow-transplant-in-the-absence-of-cytoreductive-conditioning-rescues-mice-with-%C3%AE-thalassemia-major
#8
Yongliang Huo, Jonathan R Lockhart, Shanrun Liu, Suean Fontenard, Mike Berlett, Thomas M Ryan
β-thalassemia is a group of inherited blood disorders that result in defects in β-globin chain production. Cooley anemia (CA), or β-thalassemia major, is the most severe form of the disease and occurs when an individual has mutations in both copies of the adult β-globin gene. Patients with CA fail to make adult hemoglobin, exhibit ineffective erythropoiesis, experience severe anemia, and are transfusion dependent for life. Currently, allogeneic bone marrow transplantation (BMT) is the only cure; however, few patients have suitable donors for this procedure, which has significant morbidity and mortality...
November 28, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296711/klf1-directly-activates-expression-of-the-novel-fetal-globin-repressor-zbtb7a-lrf-in-erythroid-cells
#9
Laura J Norton, Alister P W Funnell, Jon Burdach, Beeke Wienert, Ryo Kurita, Yukio Nakamura, Sjaak Philipsen, Richard C M Pearson, Kate G R Quinlan, Merlin Crossley
Genes encoding the human β-like hemoglobin proteins undergo a developmental switch from fetal γ-globin to adult β-globin expression around the time of birth. β-hemoglobinopathies, such as sickle-cell disease and β-thalassemia, result from mutations affecting the adult β-globin gene. The only treatment options currently available carry significant adverse effects. Analyses of heritable variations in fetal hemoglobin (HbF) levels have provided evidence that reactivation of the silenced fetal γ-globin genes in adult erythroid cells is a promising therapy...
April 25, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296707/cyclophosphamide-improves-engraftment-in-patients-with-scd-and-severe-organ-damage-who-undergo-haploidentical-pbsct
#10
Courtney D Fitzhugh, Matthew M Hsieh, Tiffani Taylor, Wynona Coles, Katherine Roskom, Delon Wilson, Elizabeth Wright, Neal Jeffries, Christopher J Gamper, Jonathan Powell, Leo Luznik, John F Tisdale
Peripheral blood stem cell transplantation (PBSCT) offers a curative option for sickle cell disease (SCD). Although HLA-matched sibling transplantation is promising, the vast majority of patients lack such a donor. We sought to develop a novel nonmyeloablative HLA-haploidentical PBSCT approach that could safely be used for patients with severe organ damage. Based on findings in our preclinical model, we developed a phase 1/2 trial using alemtuzumab, 400 cGy total body irradiation, and escalating doses of posttransplant cyclophosphamide (PT-Cy): 0 mg/kg in cohort 1, 50 mg/kg in cohort 2, and 100 mg/kg in cohort 3...
April 25, 2017: Blood Advances
https://www.readbyqxmd.com/read/29291192/trends-in-5-10-20-and-30-year-survival-rates-of-beta-thalassemia-patients-in-southern-iran-1995-2016-a-retrospective-cohort-study
#11
Jafar Hassanzadeh, Alireza Mirahmadizadeh, Mehran Karimi, Yousef Veisani, Shahab Rezaeian
Background: There is currently lack of knowledge about survival trend analysis of thalassemia patients. Therefore, the aim of the present study was to assess 5-, 10-, 20-, and 30-year survival of thalassemia patients over a 20-year time period. Methods: In this retrospective cohort study, we analysed the data of 982 beta-thalassemia patients in Iran. Birth cohort and traditional cohort analyses were used to obtain the 5-, 10-, 20-, and 30-year survival rates in various time intervals between 1995 and 2016. Results: Five and 10-year survival rates remained unchanged since 1995-2016...
December 13, 2017: Journal of Public Health Research
https://www.readbyqxmd.com/read/29278272/oral-manifestations-and-dentofacial-anomalies-in-%C3%AE-thalassemia-major-children-in-dubai-uae
#12
Sheikha Al-Raeesi, Mawlood Kowash, Amar Hassan, Manal Al-Halabi
AIMS: The purpose of this study was to identify special oral and dentofacial manifestations of β-thalassemia major (ΒTM) in Emarati children in Dubai (UAE). MATERIALS AND METHODS: A total of 38 (ΒTM) and 76 healthy Emirati children were recruited. An oral examination was conducted to determine dmft/DMFT indices, Simplified Oral Hygiene Index, occlusal anomalies, dentofacial, and soft-tissue abnormalities. RESULTS: ΒTM children had significantly higher DMFT compared to the healthy controls (p-value, 0...
December 26, 2017: Special Care in Dentistry
https://www.readbyqxmd.com/read/29273732/decisional-conflict-and-regret-shared-decision-making-about-pregnancy-affected-by-%C3%AE-thalassemia-major-in-southeast-of-iran
#13
Zahra Moudi, Zenab Phanodi, Hossein Ansari, Mostafa Montazer Zohour
To study the effect of shared decision-making (SDM) on decisional conflict (DC) scores immediately after consultation and to assess the decisional regret (DR) scores in the first 3 months following women's decision regarding termination of pregnancy. This quasi-experimental study was conducted during August 3rd-February 20th, 2016. We included 80 women whose fetuses were diagnosed with β-thalassemia major (β-TM) through chorionic villi sampling and were referred to the only prenatal diagnosis center at Ali-Asghar Hospital, Zahedan, Iran...
December 22, 2017: Journal of Human Genetics
https://www.readbyqxmd.com/read/29261151/iron-overload-and-chelation-therapy-in-non-transfusion-dependent-thalassemia
#14
REVIEW
Rayan Bou-Fakhredin, Abdul-Hamid Bazarbachi, Bachar Chaya, Joseph Sleiman, Maria Domenica Cappellini, Ali T Taher
Iron overload (IOL) due to increased intestinal iron absorption constitutes a major clinical problem in patients with non-transfusion-dependent thalassemia (NTDT), which is a cumulative process with advancing age. Current models for iron metabolism in patients with NTDT suggest that suppression of serum hepcidin leads to an increase in iron absorption and subsequent release of iron from the reticuloendothelial system, leading to depletion of macrophage iron, relatively low levels of serum ferritin, and liver iron loading...
December 20, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29247522/pharmacokinetics-and-pharmacodynamics-of-treosulfan-in-patients-with-thalassemia-major-undergoing-allogeneic-hematopoietic-stem-cell-transplantation
#15
Ezhilpavai Mohanan, John C Panetta, Kavitha M Lakshmi, Eunice S Edison, Anu Korula, Fouzia Na, Aby Abraham, Auro Viswabandya, Biju George, Vikram Mathews, Alok Srivastava, Poonkuzhali Balasubramanian
Treosulfan (Treo) based conditioning regimen prior to hematopoietic stem cell transplantation (HSCT) has been successfully used in treating hematological malignant and non-malignant diseases. We report Treo pharmacokinetics in patients with thalassemia major undergoing HSCT (n=87), receiving Treo at a dose of 14g/m2 /day. Median Treo AUC and clearance (CL) was 1326mg*h/L and 10.8L/h/m2 respectively. There was wide inter-individual variability in Treo AUC and CL (64 and 68%) which was not explained by any of the variables tested...
December 16, 2017: Clinical Pharmacology and Therapeutics
https://www.readbyqxmd.com/read/29240037/fetal-anemia-causing-hydrops-fetalis-from-an-alpha-globin-variant-homozygous-hemoglobin-constant-spring
#16
Patcharee Komvilaisak, Ratana Komvilaisak, Arunee Jetsrisuparb, Surapon Wiangnon, Junya Jirapradittha, Pakaphan Kiatchoosakun, Goonapa Fucharoen
BACKGROUND: Fetal anemia is often assumed to be due to red cell alloimmunization and Parvovirus infection, and can lead to hydrops fetalis and death in utero. Other causes, such as mutations of hemoglobin alpha, are less commonly considered. METHODS: We report 7 cases with fetal anemia causing hydrops fetalis. Serial Doppler ultrasound for measurement peak systolic velocity (PSV) of middle cerebral artery (MCA) was used for evaluation of fetal anemia. Fetal anemia is suggested if the MCA/PSV ratio is >1...
December 12, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29240028/genotype-phenotype-correlation-of-%C3%AE-thalassemia-in-croatian-patients-a-specific-hbb-gene-mutations
#17
Jerko Vucak, Daniel Turudic, Danko Milosevic, Marko Bilic, Zrinko Salek, Martina Rincic, Ernest Bilic
An analysis of genotype-phenotype correlation was performed for 14 patients with beta-thalassemia who had been registered in Referral Centre for hematology and oncology of the University Hospital Centre, Zagreb, Croatia. HBB gene mutations were determined using a gene-specific Q5 High-Fidelity PCR analysis with direct DNA sequencing of amplified transcripts. Mahidol score index used for classification of thalassemia severity was found to be low for all the patients enrolled in the study, indicating a mild β-thalassemia phenotype with no signs of disease progression...
December 12, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29238504/iron-overload-assessment-in-%C3%AE-thalassemia-major-is-t2-magnetic-resonance-imaging-the-answer
#18
Natasha Ali
No abstract text is available yet for this article.
October 2017: Electronic Physician
https://www.readbyqxmd.com/read/29235740/thalassemia-major-and-intermedia-in-patients-older-than-35-years-a-single-center-experience
#19
Liat Shargian-Alon, Oren Pasvolsky, Pia Raanani
BACKGROUND: During the past decades, beta thalassemia major (TM) and beta thalassemia intermedia (TI) have transformed from a universally fatal disease at a young age into a chronic disease. This advancement is attributed to improved chelation therapy as well as enhanced management strategies, with focused attention on disease and treatment-related complications. OBJECTIVES: To describe characteristics of adults with thalassemia as well as treatment modalities, disease and treatment-related complications, and socioeconomic information of the patients...
December 2017: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/29235162/iron-chelating-effect-of-silymarin-in-patients-with-%C3%AE-thalassemia-major-a-crossover-randomised-control-trial
#20
Hadi Darvishi-Khezri, Ebrahim Salehifar, Mehrnoush Kosaryan, Hossein Karami, Mohammadreza Mahdavi, Abbas Alipour, Aily Aliasgharian
This study aimed to determine the potential iron-chelating effects of silymarin in patients with β-thalassemia major receiving standard iron-chelation therapy. We evaluated whether addition of silymarin to standard iron-chelation therapy could improve iron burden markers and liver and cardiac function in these patients, via a placebo-controlled, crossover clinical study. Silymarin (140 mg) or placebo were administered thrice daily to all patients (n = 82) for 12 weeks, and after a 2-week washout period, patients were crossed over to the other groups...
December 13, 2017: Phytotherapy Research: PTR
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