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thalassemia major

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https://www.readbyqxmd.com/read/28627425/a-multicenter-retrospective-analysis-stressing-importance-of-long-term-follow-up-after-hematopoietic-cell-transplantation-for-%C3%AE-thalassemia
#1
S Chaudhury, M Ayas, C Rosen, M Ma, M Viqaruddin, S Parikh, S Kharbanda, K Y Chiang, A Haight, M Bhatia, Gmt Guilcher, A Thompson, S Shenoy
Allogeneic hematopoietic cell transplantation (HCT) is curative in patients with β-thalassemia major. However, the majority of reports on HCT outcomes lack long-term follow-up data with the exception of single center reports. An international multicenter retrospective data collection and analysis was conducted in 176 β-Thalassemia patients who were 1 year or beyond after first HCT to evaluate follow up methods and outcomes at 7 centers- Median age at HCT was 5.5 years (range, 0.6- 18.5) and median follow-up was 7 years (range, 1-20)...
June 13, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28624809/genome-wide-analysis-of-aberrantly-expressed-lncrnas-and-mirnas-with-associated-co-expression-and-cerna-networks-in-%C3%AE-thalassemia-and-hereditary-persistence-of-fetal-hemoglobin
#2
Ketong Lai, Siyuan Jia, Shanjuan Yu, Jianming Luo, Yunyan He
The implications of lncRNAs regarding fetal hemoglobin (HbF) induction in hemoglobin disorders remain poorly understood. In this study, microarray analysis was performed to profile lncRNAs, miRNAs and mRNAs in individuals with hereditary persistence of fetal hemoglobin (HPFH), β-thalassemia carriers with high HbF levels and healthy controls. The results show aberrant expression of 862 lncRNAs, 568 mRNAs and 63 miRNAs in the high-HbF group compared with the control group. Altered NR_001589, NR_120526, T315543, miR-486-3p, miR-19b-1-5p and miR-20a-3p expression was confirmed by quantitative reverse transcription-polymerase chain reaction, and Spearman correlation coefficients revealed significant positive correlations with HbF...
May 29, 2017: Oncotarget
https://www.readbyqxmd.com/read/28621205/neurocognitive-dysfunction-in-children-with-%C3%AE-thalassemia-major-psychometric-neurophysiologic-and-radiologic-evaluation
#3
M S Elalfy, R H Aly, H Azzam, K Aboelftouh, R H Shatla, M Tarif, M Abdatty, R M Elsayed
OBJECTIVE: To evaluate the impact of iron chelating drugs and serum ferritin on the neurocognitive functions of patients with β thalassemia major (β-TM), using psychometric, neurophysiologic and radiologic tests. METHODS: Eighty children with β-TM were enrolled into the study and were compared to 40 healthy controls. All participants were evaluated by measuring serum ferritin, neurocognitive assessment by Benton Visual Retention Test, Wechsler Intelligence Scale for Children, Wisconsin Card Sort Test, P300 and magnetic resonance spectroscopy (MRS)...
June 16, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28617058/the-assessment-of-skin-color-and-iron-levels-in-pediatric-patients-with-%C3%AE-thalassemia-major-using-a-visual-skin-color-chart
#4
Ibrahim H Bucak, Habip Almis, Samet Benli, Mehmet Turgut
Patients with β-thalassemia major (β-TM), a disease that emerges due to disorder of hemoglobin (Hb) synthesis, require life-long erythrocyte transfusion. The purpose of this study was to evaluate skin color and iron levels of patients with β-TM using a visual skin color chart. Each patient's skin color was matched on a skin color chart under a fluorescent lamp by the same physician on each occasion. Iron, iron binding capacity, ferritin and complete blood count (CBC) were studied for each patient enrolled...
June 15, 2017: Hemoglobin
https://www.readbyqxmd.com/read/28605653/left-ventricle-remodeling-in-patients-with-%C3%AE-thalassemia-major-an-emerging-differential-diagnosis-with-left-ventricle-noncompaction-disease
#5
Elisabetta Chiodi, Marianna Nardozza, Maria Rita Gamberini, Alessia Pepe, Massimo Lombardi, Giorgio Benea, Donato Mele
To differentiate left ventricle non-compaction (LVNC) from hypertrabeculated myocardium due to LV remodeling in β-thalassemia major (β-TM) patients, cardiac magnetic resonance (CMR) images of 38 β-TM patients and 10 LVNC patients were compared using 3 diagnostic criteria: ratio of diastolic segmental non-compacted to compacted myocardium (NC/C ratio) >2.5, percentage of non-compacted LV mass (NC-LVM%) >20% and >25% of global LV mass. Specificity of NC/C ratio of >2.5 was the lowest (58%) and of NC-LVM% of >25% the highest (93%)...
May 17, 2017: Clinical Imaging
https://www.readbyqxmd.com/read/28600663/serum-hepcidin-as-a-diagnostic-marker-of-severe-iron-overload-in-beta-thalassemia-major
#6
Ahmed Maher Kaddah, Amina Abdel-Salam, Marwa Salah Farhan, Reham Ragab
OBJECTIVES: To investigate potential usefulness of serum hepcidin in the diagnosis of iron overload in children with β-thalassemia. METHODS: A study was conducted on 30 thalassemia major (TM), 30 thalassemia intermedia (TI) and 60 healthy children as controls. Serum hepcidin was measured by Human Hepcidin, ELISA Kit. RESULTS: β-thalassemia patients had a higher serum hepcidin compared to the controls (p < 0.001). TM group had higher hepcidin and ferritin compared to the TI group (p = 0...
June 10, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28596656/assessment-of-heart-and-liver-iron-overload-in-thalassemia-major-patients-using-t2-magnetic-resonance-imaging
#7
Hamid Farhangi, Zahra Badiei, Hasan Mottaghi Moghaddam, Mohammad Reza Keramati
Accumulation of excess iron in heart can lead to cardiac dysfunction, which is the most common cause of death in thalassemia major patients. Biopsy is an invasive procedure and therefore not an ideal option to assess iron load. However, standard/usual non-invasive methods, such as ferritin measurement, have some limitations and the results show poor correlations with iron load. Magnetic Response Imaging (MRI-T2*), as a non-invasive and reliable method for iron load assessment in organs such as liver and heart, can be suggested as a favorable alternative...
June 2017: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/28592008/-analysis-of-the-influence-of-iron-overload-in-glucose-metabolism-in-thalassemia-major-patients
#8
L Y Liang, W Q Lao, Z Meng, L N Zhang, L L Hou, H Ou, Z L Liu, Z W He, X Y Luo, J P Fang
Objective: This study aimed at determining the characteristics of the glucose homeostasis and its relationship with iron overload of the patients with β-thalassemia major (β-TM). Method: From Sun Yat-sen Memorial Hospital between January 2014 and December 2015, a total of 57 transfusion-dependent β-TM patients with 5-18 years old were enrolled in this study and fasting blood glucose(FBG) and insulin level, serum ferritin (SF), serum iron, transferrin, total iron binding capacity, unsaturated iron binding capacity were determined...
June 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28589785/revisiting-beta-thalassemia-intermedia-past-present-and-future-prospects
#9
Naouel Ben Salah, Rayan Bou-Fakhredin, Fethi Mellouli, Ali T Taher
BACKGROUND: The spectrum of thalassemias is wide ranging from thalassemia minor, which consists of mild hypochromic microcytic anemia without obvious clinical manifestations, to thalassemia major (TM), which is characterized by severe anemia since the first years of life and is transfusion dependent. Thalassemia intermedia (TI) describes those patients with mild or moderate anemia. OBJECTIVE: To describe the genetic features and major clinical complications of TI, and the therapeutic approaches available in the management of this disease...
June 7, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28585595/treatment-of-chronic-hepatitis-c-in-thalassemia-major-patients
#10
Shaimuna Fareeha Sajjad, Waquaruddin Ahmad, Syed Sarafaraz Hussain Jaffery, Mohammad Asif, Syed Ejaz Alam
Treatment of chronic hepatitis C (HCV) is difficult in thalassemics due to the haemolytic side effects of therapy. This study evaluated the treatment response to conventional interferon and ribavirin in HCV patients with thalassemia major. It was conducted at PMRC Research Centre, Jinnah Postgraduate Medical Centre (JPMC) Karachi. At baseline complete blood count, liver function tests, serum protein/ albumin, random blood glucose, serum ferritin, TSH, HCV RNA (quantitative) and genotyping were done. Conventional interferon 3 MIU thrice weekly and ribavirin 400 mg daily was given for 24 or 48 weeks...
June 2017: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/28566824/use-of-strain-strain-rate-tissue-velocity-imaging-and-endothelial-function-for-early-detection-of-cardiovascular-involvement-in-patients-with-beta-thalassemia
#11
Abhinav Gupta, Aditya Kapoor, Shubha Phadke, Archana Sinha, Shridhar Kashyap, Roopali Khanna, Sudeep Kumar, Naveen Garg, Satyendra Tewari, Pravin Goel
BACKGROUND: Global ventricular function often remains normal in patients with beta-thalassemia major (β-TM) until late. Tissue Doppler and strain imaging may be useful to assess regional myocardial function abnormalities in these patients. METHODS: Systolic (Sm), early diastolic (Em), and late diastolic (Am) (Em/Am) myocardial velocities at basal lateral and septal left ventricular (LV) segments, strain (S), and strain rate (SR) in basal and mid LV, right ventricular (RV) and septum were measured in 30 patients (β-TM, 12...
May 2017: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/28546763/mutation-analysis-of-%C3%AE-thalassemia-in-east-western-indian-population-a-recent-molecular-approach
#12
Parth S Shah, Nidhi D Shah, Hari Shankar P Ray, Nikunj B Khatri, Ketan K Vaghasia, Rutvik J Raval, Sandip C Shah, Mandava V Rao
BACKGROUND: β-Thalassemia is the most prevalent genetic disorder in India. Its traits and coinheritance vary from mild to severe conditions, resulting in thalassemia minor, intermediate, and major, depending upon many factors. PURPOSE: The objective of this study was to identify the incidence of β-thalassemia traits, their coinheritance, and mutations, as well as to support the patients already diagnosed with β-thalassemia in East-Western Indian population for better management...
2017: Application of Clinical Genetics
https://www.readbyqxmd.com/read/28538510/thalidomide-induced-stroke-in-a-child-with-thalassemia-major
#13
Sushil Gunaseelan, Anand Prakash
β-thalassemia major is a hereditary anemia resulting from defects in β-globin production. It is also characterized by a hypercoagulable state with an increased risk of thrombosis. Thalidomide, a drug known for its immunomodulating and antiangiogenic properties, has recently been demonstrated to induce γ-globin gene expression and to increase the proliferation of erythroid cells. An increasing incidence of thromboembolic events in thalidomide-treated patients has been reported. This is often in the context of thalidomide combinations with other drugs, including steroids and particularly anthracycline-based chemotherapy, and with very low incidence of thrombosis with single-agent thalidomide treatment...
May 22, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28538505/thalassemia-major-and-priapism-a-case-report-of-an-adolescent
#14
Sibel Öz, Serhan Küpeli, Gülay Sezgin, İbrahim Bayram
Priapism is defined as a prolonged pathologic penile erection without sexual stimulation. In children, priapism secondary to sickle cell disease or hematological malignancy is a frequent condition. Appropriate treatment of priapism varies; the treatment is primarily etiological, conservative management. In the present report, we aimed to present a case of asplenic thalassemia major who developed priapism, improved with hydration and ibuprofen treatment. Clinicians should take into account that priapism can be encountered in patients with thalassemia major...
May 22, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28523047/alpha-thalassemia-deletions-found-in-suspected-cases-of-beta-thalassemia-major-in-pakistani-population
#15
Saba Shahid, Muhammad Nadeem, Danish Zahid, Jawad Hassan, Saqib Ansari, Tahir Shamsi
BACKGROUND & OBJECTIVE: Alpha (α) thalassemia is a hereditary disorder and is caused by deletions or mutations in globin genes. It is present in two clinically significant forms: hemoglobin Bart hydrops fetalis (Hb Bart) syndrome and hemoglobin H (HbH) disease. It is highly prevalent in South-East Asia or Mediterranean countries. The most common deletion reported in alpha thalassemia in Pakistani population was -α(3.7) with a frequency of 8.3%, and the rare forms were -α(4.2) (0.2%) and ααα(anti3...
March 2017: Pakistan Journal of Medical Sciences Quarterly
https://www.readbyqxmd.com/read/28521805/thalassemias-in-south-asia-clinical-lessons-learnt-from-bangladesh
#16
REVIEW
Mohammad Sorowar Hossain, Enayetur Raheem, Tanvira Afroze Sultana, Shameema Ferdous, Nusrat Nahar, Sazia Islam, Mohammad Arifuzzaman, Mohammad Abdur Razzaque, Rabiul Alam, Sonia Aziz, Hazera Khatun, Abdur Rahim, Manzur Morshed
Thalassemias are emerging as a global public health concern. Due to remarkable success in the reduction of childhood mortality by controlling infectious diseases in developing countries, thalassemias are likely to be a major public health concern in the coming decades in South Asia. Despite the fact that Bangladesh lies in the world's thalassemia belt, the information on different aspects (epidemiology, clinical course, mortality, complications and treatment outcomes) of thalassemias is lacking. In this comprehensive review, the aim is to to depict the epidemiological aspects of thalassemias, mutation profile and current treatment and management practices in the country by sharing the experience of dealing with 1178 cases over 2009-2014 time periods in a specialized thalassemia treatment centre...
May 18, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28510704/skin-disorders-in-egyptian-children-with-%C3%AE-thalassemia-major
#17
Sameh S Fahmey, Gamal Taha, Abdelaziz El-Refaey, Sherry Adly
Background: Thalassemia major is one of the common genetic disorders in Egypt. Skin disorders are usually neglected and frequently underdiagnosed among these patients. Objective: This work aimed to study the frequency and pattern of skin manifestations in Egyptian children with β-thalassemia. Methods: Fifty-four β-thalassemia major patients being followed at the Hematology Clinic of Beni-Suef University Hospital were selected to participate in this study...
May 16, 2017: Journal of Tropical Pediatrics
https://www.readbyqxmd.com/read/28506685/simultaneous-genotyping-of-%C3%AE-thalassemia-deletional-and-nondeletional-mutations-by-real-time-pcr-based-multicolor-melting-curve-analysis
#18
Qiuying Huang, Xudong Wang, Ning Tang, Tizhen Yan, Ping Chen, Qingge Li
α-Thalassemia, which is caused by defective synthesis of the hemoglobin α-globin chains, is the most commonly inherited recessive hemoglobin abnormality. Genetic detection of a defective α-globin gene is challenging because of a variety of large deletions of the α-globin gene cluster and nondeletional mutations. Separate detections of them are often required using complex and error-prone open-tube methods. We report a novel real-time PCR-based assay that can simultaneously genotype four major deletional and three common nondeletional mutations in two parallel reactions by using multicolor melting curve analysis...
May 12, 2017: Journal of Molecular Diagnostics: JMD
https://www.readbyqxmd.com/read/28503278/conjugate-and-23-valent-pneumococcal-polysaccharide-booster-vaccination-in-asplenic-patients-with-thalassemia-major-a-randomized-clinical-trial-study
#19
Mohammad Sadegh Rezai, Javad Ghaffari, Mohammadreza Mahdavi, Amir Bahari, Shahram Ala
BACKGROUND: Pneumococcal vaccine provides protection against invasive pneumococcal disease in population at risk. This study was conducted to compare the antibody response to 13-valent pneumococcal conjugate vaccine and 23-valent pneumococcal polysaccharide vaccine in patients with thalassemia major. METHODS: A randomized cross-over clinical trial was performed on 50 asplenic patients with thalassemia major who referred to thalassemia center at Bouali Sina Hospital, Sari, Iran from 2013 to 2014...
2017: Caspian Journal of Internal Medicine
https://www.readbyqxmd.com/read/28503277/prevalence-of-diabetes-impaired-fasting-glucose-and-impaired-glucose-tolerance-in-patients-with-thalassemia-major-in-iran-a-meta-analysis-study
#20
REVIEW
Milad Azami, Ali Sharifi, Siros Norozi, Akram Mansouri, Kourosh Sayehmiri
BACKGROUND: This study aimed to investigate the prevalence of diabetes, impaired fasting glucose (IFG) and impaired glucose tolerance (IGT) in Iranian patients with thalassemia major. METHODS: The current study has been conducted based on PRISMA guideline. To obtain the documents, Persian and English scientific databases such as Magiran, Iranmedex, SID, Medlib, IranDoc, Scopus, PubMed, ScienceDirect, Cochrane, Web of Science, Springer, Wiley Online Library as well as Google Scholar were searched until December 2015...
2017: Caspian Journal of Internal Medicine
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