keyword
MENU ▼
Read by QxMD icon Read
search

thalassemia major

keyword
https://www.readbyqxmd.com/read/28538510/thalidomide-induced-stroke-in-a-child-with-thalassemia-major
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
https://www.readbyqxmd.com/read/28502814/breathless-beta-thalassemia-major
#9
Vasiliki Katsi, Georgios Georgiopoulos, Georgia Vogiatzi, Dimitris Tousoulis
No abstract text is available yet for this article.
May 11, 2017: American Journal of Medicine
https://www.readbyqxmd.com/read/28485683/cardiac-complications-in-beta-thalassemia-from-mice-to-men
#10
Sirinart Kumfu, Suthat Fucharoen, Siriporn C Chattipakorn, Nipon Chattipakorn
Beta-thalassemia is an inherited hemoglobin disorder caused by reduced or absent synthesis of the beta globin chains of hemoglobin. This results in variable outcomes ranging from clinically asymptomatic to severe anemia, which then typically requires regular blood transfusion. These regular blood transfusions can result in an iron overload condition. The iron overload condition can lead to iron accumulation in various organs, especially in the heart, leading to iron overload cardiomyopathy, which is the major cause of mortality in patients with thalassemia...
January 1, 2017: Experimental Biology and Medicine
https://www.readbyqxmd.com/read/28458927/papillary-fibroelastoma-as-a-cause-of-cardiogenic-embolic-stroke-in-a-%C3%AE-thalassemia-patient-case-report-and-literature-review
#11
Re-I Chin, John J Monda, Maulik Sheth, William Ogle, Gloria Merenda, Debapriya De
We describe a case of a young male without stroke risk factors who presented with a sudden onset of left-sided weakness, left hand numbness, and left eye blurriness. CT scan of the head without contrast and diffusion-weighted MRI of the brain with contrast revealed an ischemic stroke in the right middle cerebral artery distribution. Transesophageal echocardiography (TEE) revealed a mobile pedunculated mass on the posterior surface of the mitral valve. This mass was resected and pathology showed a cardiac papillary fibroelastoma (CPFE), which was determined to be the cause of the patient's cardioembolic stroke...
2017: Case Reports in Cardiology
https://www.readbyqxmd.com/read/28458697/myocardial-iron-load-measured-by-cardiac-magnetic-resonance-imaging-to-evaluate-cardiac-systolic-function-in-thalassemia
#12
Masoumeh Kahnooji, Hamid Reza Rashidinejad, Mohammad Shahram Yazdanpanah, Nahid Azdaki, Ahmad Naghibzadeh-Tahami
BACKGROUND: The assessment of cardiac iron overload in thalassemia major has been considered as an important predictive factor of heart injury. The magnetic resonance imaging (MRI)-derived relaxation time parameter (T2*) varies inversely with iron level, and elevated myocardial iron levels by T2* are associated with depressed left ventricular (LV) ejection fraction (EF). We compared echocardiographic (ECHO) indices of systolic function to myocardial T2* in these patients. METHODS: A cross-sectional database review identified 200 consecutive patients with thalassemia who underwent both ECHO and MRI T2* assessment...
September 2016: ARYA Atherosclerosis
https://www.readbyqxmd.com/read/28456904/myocardial-deformation-in-iron-overload-cardiomyopathy-speckle-tracking-imaging-in-a-beta-thalassemia-major-population
#13
Luca Antonio Felice Di Odoardo, Marianna Giuditta, Elena Cassinerio, Alberto Roghi, Patrizia Pedrotti, Marco Vicenzi, Veronica Maria Sciumbata, Maria Domenica Cappellini, Alberto Pierini
Traditional echocardiography is unable to detect neither the early stages of iron overload cardiomyopathy nor myocardial iron deposition. The aim of the study is to determine myocardial systolic strain indices in thalassemia major (TM), and assess their relationship with T2*, a cardiac magnetic resonance index of the severity of cardiac iron overload. 55 TM cases with recent cardiac magnetic resonance (CMR-T2*) underwent speckle tracking analysis to assess regional myocardial strains and rotation. The results were compared with a normal control group (n = 20), and were subsequently analyzed on the basis of the CMR-T2* values...
April 29, 2017: Internal and Emergency Medicine
https://www.readbyqxmd.com/read/28446301/-analysis-of-gene-mutation-types-of-thalassemia-in-longyan-area-of-fujian-province-in-china
#14
Qing-Fu Dai, Xiao-Lu Li, Yu-Xia Wang, Chun-Fang Cao
OBJECTIVE: To explore the type and distribution of thalassemia gene mutation in Longyan area of Fujian province in China, so as to provide a evidence for prenatal diagnosis and to reduce birth defects. METHODS: The mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH) and hemoglobin electrophoresis were used for screened the mutation types of thalassemia. Genotyping of the screened positive sample was performed by gap single polymerase chain reaction (gap-PCR) and reverse dot blot hybridization (RDB)...
April 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28440111/quality-of-life-outcomes-in-a-pediatric-thalassemia-population-in-egypt
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
keyword
keyword
44472
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"