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https://read.qxmd.com/read/30768496/an-investigation-into-variability-in-the-therapeutic-response-to-deferiprone-in-patients-with-thalassemia-major-retraction
#1
(no author information available yet)
No abstract text is available yet for this article.
February 12, 2019: Therapeutic Drug Monitoring
https://read.qxmd.com/read/30765134/defective-cytokine-production-from-monocytes-macrophages-of-e-beta-thalassemia-patients-in-response-to-pythium-insidiosum-infection
#2
Somkiat Ud-Naen, Tunsuda Tansit, Duangjit Kanistanon, Angkana Chaiprasert, Wanchai Wanachiwanawin, Yuttana Srinoulprasert
BACKGROUND: Pythium insidiosum has been mainly reported to cause morbidity and mortality in thalassemia patients. P. insidiosum zoospores can germinate to be hyphae within a few hours; therefore, it is difficult to study the initial immune response that P. insidiosum zoospores induce. The present study aims to compare immune responses against P. insidiosum zoospore infection by comparing monocytes/macrophages from thalassemia patients with those from non-thalassemia controls. METHODS: In order to keepP...
February 8, 2019: Immunobiology
https://read.qxmd.com/read/30745767/n-terminal-pro-b-type-natriuretic-peptide-levels-and-cardiac-hemosiderosis-in-adolescent-%C3%AE-thalassemia-major-patients
#3
Ahmad Kautsar, Najib Advani, Murti Andriastuti
Background: Iron-induced cardiomyopathy remains the leading cause of mortality in patients with β-thalassemia major. Iron overload cardiomyopathy, which may be reversible through iron chelation, is characterized by early diastolic dysfunction. Amino-terminal pro-brain natriuretic peptide (NT-proBNP) is a sensitive biomarker of diastolic dysfunction. Aim: The aim of the study is to evaluate the diagnostic value of NT-proBNP as a surrogate marker of iron overload examined with magnetic resonance imaging T2-star (MRI T2*)...
January 2019: Annals of Pediatric Cardiology
https://read.qxmd.com/read/30745477/quality-matters-hematopoietic-stem-cell-transplantation-versus-transfusion-and-chelation-in-thalassemia-major
#4
Shivani Patel, Venkateswaran Vellaichamy Swaminathan, V Sr Mythili, M Sr Venkatadesikalu, Meena Sivasankaran, Dhaarani Jayaraman, R Balasubramaniam, Ramya Uppuluri, Revathi Raj
OBJECTIVE: To compare quality of life of children with thalassemia major who have undergone stem cell transplantation with those on regular transfusion. METHODS: The study included 40 children who underwent transplantation and 40 children and 20 adults on regular transfusion and iron chelation therapy. The quality of life assessment was done using the Pediatric Quality of Life Inventory 4.0 Generic Core Scale. RESULTS: The mean total summary score, psychosocial summary score and physical score was 92, 91 and 92...
December 15, 2018: Indian Pediatrics
https://read.qxmd.com/read/30738465/-prevalence-and-genetic-analysis-of-%C3%AE-thalassemia-in-neonates-in-wuhan-area-of-china
#5
Qian Xiong, Xi-Jiang Hu, Xiang Dai, Bin Zhou, Wen-Qian Cai
OBJECTIVE: To investigate the β-thalassemia genotypes in neonates in Wuhan area of China and their characteristics of molecular epidemiology. METHODS: A total of 2721 neonates in Wuhan who were positive in primary screening for β-thalassemia were included in this study. Genotypes of β-thalassemia gene were determined with PCR-flow cytometry and fluorescence hybridization assay. RESULTS: There were 537 cases of β-thalassemia with over 15 kinds of genotypes, and 19 cases of α-composite β-thalassemia with 8 genotypes...
February 2019: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://read.qxmd.com/read/30721337/hyperuricemia-urine-uric-excretion-and-associated-complications-in-thalassemia-patients
#6
Juthatip Chaloemwong, Adisak Tantiworawit, Thanawat Rattanathammethee, Chatree Chai-Adisaksopha, Ekarat Rattarittamrong, Lalita Norasetthada, Pimlak Charoenkwan, Worawit Louthrenoo
Thalassemia patients have a high cell turnover rate due to chronic hemolysis and ineffective erythropoiesis; therefore, hyperuricemia is anticipated. This study aimed to identify the prevalence of hyperuricemia, gout and nephrolithiasis, conditions associated with serum uric acid (SUA), and urine uric acid excretion (UUA) in thalassemia patients. This was a cross-sectional study in patients aged 15 years or older at Chiang Mai University Hospital. All patients had blood and 24-h urine collection test. We enrolled 112 thalassemia patients in which 67...
February 5, 2019: Annals of Hematology
https://read.qxmd.com/read/30716678/a-sensitive-electrochemical-genosensor-for-highly-specific-detection-of-thalassemia-gene
#7
Mohammad-Bagher Gholivand, Arezoo Akbari
Beta thalassemias (βth) are the result of mutations in the β-globin gene. In this report, an electrochemical genosensor was made to detect the sequences anent with the β-globin gene. This biosensor is based on immobilizing 20-mer single stranded oligonucleotide (probe) on the Au nanoparticles- poly (4-aminothiophenol)/ reduced graphene oxide/glassy carbon electrode (AuNPs-PAT/rGO/ GCE) and hybridizing this oligonucleotide along with its complementary sequence (target). The vastness of the probe and target sequences hybridization was studied through differential pulse voltammetry (DPV) along with electrochemical impedance spectroscopy (EIS) using the [Fe(CN)6]3- /4- (1:1) as a hybridization index...
January 15, 2019: Biosensors & Bioelectronics
https://read.qxmd.com/read/30713307/successful-treatment-of-acute-chest-syndrome-with-manual-exchange-transfusion-in-a-patient-with-sickle-beta-thalassemia
#8
Akihiro Kawahara, Takehiko Morioka, Yuichiro Otani, Keishi Kanno, Taro Edahiro, Noriyasu Fukushima, Sachi Nagasaka, Mika Housai, Masaki Kakimoto, Naoki Tsuji, Shuntaro Asano, Yuka Kikuchi, Tomoki Kobayashi, Daisuke Miyamori, Ryoko Ishida, Kazuki Kimura, Nobusuke Kishikawa, Masafumi Mizooka, Tatsuo Ichinohe, Susumu Tazuma
Acute chest syndrome (ACS), characterized by fever, respiratory symptoms, and new pulmonary infiltration, is a serious complication of sickle cell disease (SCD). Regardless of the etiology, the conventional treatment options for ACS include empirical antibiotic therapy, the administration of analgesics, and red cell transfusion. The indications and methods of red cell transfusion are critical. We herein report the case of a 26-year-old African-American man with SCD who developed ACS and who was successfully treated with manual exchange transfusion...
February 1, 2019: Internal Medicine
https://read.qxmd.com/read/30708965/%C3%AE-lipoic-acid-reduces-iron-induced-toxicity-and-oxidative-stress-in-a-model-of-iron-overload
#9
Giuseppina Camiolo, Daniele Tibullo, Cesarina Giallongo, Alessandra Romano, Nunziatina L Parrinello, Giuseppe Musumeci, Michelino Di Rosa, Nunzio Vicario, Maria V Brundo, Francesco Amenta, Margherita Ferrante, Chiara Copat, Roberto Avola, Giovanni Li Volti, Antonio Salvaggio, Francesco Di Raimondo, Giuseppe A Palumbo
Iron toxicity is associated with organ injury and has been reported in various clinical conditions, such as hemochromatosis, thalassemia major, and myelodysplastic syndromes. Therefore, iron chelation therapy represents a pivotal therapy for these patients during their lifetime. The aim of the present study was to assess the iron chelating properties of α-lipoic acid (ALA) and how such an effect impacts on iron overload mediated toxicity. Human mesenchymal stem cells (HS-5) and animals (zebrafish, n = 10 for each group) were treated for 24 h with ferric ammonium citrate (FAC, 120 µg/mL) in the presence or absence of ALA (20 µg/mL)...
January 31, 2019: International Journal of Molecular Sciences
https://read.qxmd.com/read/30706992/refugee-children-with-beta-thalassemia-in-turkey-overview-of-demographic-socioeconomic-and-medical-characteristics
#10
Arzu Yazal Erdem, Fatma Demir Yenigürbüz, Esra Pekpak, Burcu Akıncı, Elif Aktekin, Cengiz Bayram, Zeynep Yıldız Yıldırmak, Eda Ataseven, Sinan Akbayram, İlgen Şaşmaz, Başak Taburoğlu Yılmaz, Ayşe Özkan, Sibel Akpınar Tekgündüz, Doğan Köse, Tuba Karapınar, Mustafa Büyükavcı, Ertan Sal, Turan Bayhan, Serap Kirkiz, Şule Ünal, Raziye Canan Vergin, Metin Çil, Barış Malbora, Ali Ayçiçek, Hüsniye Neşe Yaralı, Namık Yaşar Özbek
AIM: Since the beginning of the Syrian civil war, more than 3.5 million Syrians have been under temporary protection status in Turkey. Because beta-thalassemia (BT) is a prevalent disorder in the Mediterranean countries, we decided to estimate the prevalence of and make an overview of the demographic, socioeconomic, medical characteristics, and healthcare problems of refugee children with BT. PATIENTS: Eighteen Turkish Pediatric Hematology Oncology Centers (PHOC) with 318 refugee children from 235 families participated in the study...
February 1, 2019: Pediatric Blood & Cancer
https://read.qxmd.com/read/30704988/editing-aberrant-splice-sites-efficiently-restores-%C3%AE-globin-expression-in-%C3%AE-thalassemia
#11
Shuqian Xu, Kevin Luk, Qiuming Yao, Anne H Shen, Jing Zeng, Yuxuan Wu, Hong-Yuan Luo, Christian Brendel, Luca Pinello, David H K Chui, Scot A Wolfe, Daniel E Bauer
The thalassemias are compelling targets for therapeutic genome editing in part because monoallelic correction of a subset of hematopoietic stem cells (HSCs) would be sufficient for enduring disease amelioration. A primary challenge is the development of efficient repair strategies that are effective in HSCs. Here we demonstrate that allelic disruption of aberrant splice sites, one of the major classes of thalassemia mutations, is a robust approach to restore gene function. We target the IVS1-110G>A mutation using Cas9 ribonucleoprotein (RNP) and the IVS2-654C>T mutation by Cas12a/Cpf1 RNP in primary CD34+ hematopoietic stem and progenitor cells (HSPCs) from β-thalassemia patients...
January 31, 2019: Blood
https://read.qxmd.com/read/30704764/pantoprazole-reduces-serum-ferritin-in-patients-with-thalassemia-major-and-intermedia-a-randomized-controlled-study
#12
Aziz Eghbali, Atefeh Khalilpour, Hassan Taherahmadi, Bahador Bagheri
AIM: Complications due to iron overload exert a problematic situation in patients with thalassemia. Proton pump inhibitors (PPIs) like pantoprazole are effective agents to reduce acid gastric acid secretion and perhaps to interrupt iron absorption in conditions with increased iron absorption. Our purpose was to study effects of pantoprazole addition to chelators on iron levels in patients with thalassemia major and intermedia. METHODS: This randomized, controlled, and single center trial was performed on 60 patients with thalassemia major and intermedia in Amir Kabir hospital, Iran...
January 14, 2019: Thérapie
https://read.qxmd.com/read/30701409/gene-therapy-for-beta-hemoglobinopathies-milestones-new-therapies-and-challenges
#13
REVIEW
Valentina Ghiaccio, Maxwell Chappell, Stefano Rivella, Laura Breda
Inherited monogenic disorders such as beta-hemoglobinopathies (BH) are fitting candidates for treatment via gene therapy by gene transfer or gene editing. The reported safety and efficacy of lentiviral vectors in preclinical studies have led to the development of several clinical trials for the addition of a functional beta-globin gene. Across trials, dozens of transfusion-dependent patients with sickle cell disease (SCD) and transfusion-dependent beta-thalassemia (TDT) have been treated via gene therapy and have achieved reduced transfusion requirements...
January 30, 2019: Molecular Diagnosis & Therapy
https://read.qxmd.com/read/30700180/quality-of-life-of-pakistani-children-with-%C3%AE-thalassemia-major
#14
Humaira Yasmeen, Shahida Hasnain
Compromised quality of life (QoL) has been reported in individuals suffering from β-thalassemia major (β-TM) in Pakistan. However, insufficient data of its associated psychosocial, physical and other disease-related determinants is available. In an observational analytical study, 200 subjects aged between 5-25 years, were examined using a transfusion-dependent QoL (TranQoL) questionnaire. Clinical records and other related data were also gathered from transfusion center databases. The TranQoL mean score was 48...
January 30, 2019: Hemoglobin
https://read.qxmd.com/read/30697927/blood-transfusion-therapy-for-%C3%AE-thalassemia-major-and-hemoglobin-e-%C3%AE-thalassemia-adequacy-trends-and-determinants-in-sri-lanka
#15
Sachith Mettananda, Hashan Pathiraja, Ravindu Peiris, Nethmi Wickramarathne, Dayananda Bandara, Udaya de Silva, Chamila Mettananda, Anuja Premawardhena
BACKGROUND: Regular blood transfusion therapy still remains the cornerstone in the management of β-thalassemia. Although recommendations are clear for patients with β-thalassemia major, uniform transfusion guidelines are lacking for patients with hemoglobin E β-thalassemia. In this study, we aim to describe the adequacy, trends, and determinants of blood transfusion therapy in a large cohort of pediatric patients with β-thalassemia major and hemoglobin E β-thalassemia. METHODS/PROCEDURE: This cross-sectional study was performed among all regularly transfused patents with β-thalassemia aged 2 to 18 years attending three large thalassemia centers in Sri Lanka...
January 29, 2019: Pediatric Blood & Cancer
https://read.qxmd.com/read/30697270/the-effect-of-splenectomy-on-complement-regulatory-proteins-in-erythrocytes-in-%C3%AE-thalassemia-major
#16
Ayşegül Uğur Kurtoğllu, Belkıs Koçtekin, Erdal Kurtoğlu, Mustafa Yildiz
Introduction: Hemolysis due to ineffective erythropoiesis is a serious problem β-thalassemia major (β-TM) patients. The role of complement system in the etiopathogenesis of hemolysis observed in β-TM were released. Hemolysis induced by activation of complement system is prevented by complement regulatory proteins. Decay accelerating factor (CD55), membrane inhibitor of reactive lysis (CD59), and complement reception 1 (CR1, CD35) are among these proteins. The absence of these proteins thus accounts for the increased susceptibility of erythrocytes to complement lysis...
January 2019: Archives of Medical Science: AMS
https://read.qxmd.com/read/30692802/manual-red-cell-exchange-transfusion-to-avert-sickle-cell-related-complications
#17
Ruhi A Mehra, Seema A Gupta, D B Borkar
Sickle cell-beta thalassemia is a double heterozygous state. Red cell exchange (RCE) transfusion reduces the concentration of sickle cells without increasing the hematocrit or whole-blood viscosity. It can be performed manually or by erythrocytapheresis. RCE transfusion is an effective tool for both acute and chronic complications of sickle cell disease. In patients unaffording erythrocytapheresis, even manual RCE can give favorable results. A 37-year-old male, a known case of sickle cell-beta+ thalassemia (βs β+), presented with avascular necrosis of right femur and humeral head...
July 2018: Asian Journal of Transfusion Science
https://read.qxmd.com/read/30692794/experience-of-hepatitis-c-virus-seroprevalence-and-its-genomic-diversity-among-transfusion-dependent-thalassemia-patients-in-a-transfusion-center
#18
Kallol Kumar Bhattacharyya, Aritra Biswas, Debanjali Gupta, Provash C Sadhukhan
INTRODUCTION: One of the most common blood-borne transfusion-transmitted diseases is hepatitis C. Patients with a history of multiple blood transfusions are significantly at a greater risk of infection by contaminated blood and blood products. Beta thalassemia major is one such condition where repeated blood transfusions are required for patient management. MATERIALS AND METHODS: The present study was conducted to investigate the serological prevalence of hepatitis C virus (HCV), its viremia, and genotype distribution with clinical parameters among multitransfused thalassemic individuals...
July 2018: Asian Journal of Transfusion Science
https://read.qxmd.com/read/30683996/antibody-persistence-5-years-after-a-13-valent-pneumococcal-conjugate-vaccine-in-asplenic-patients-with-%C3%AE-thalassemia-assessing-the-need-for-booster
#19
Ioanna Papadatou, Theano Lagousi, Antonis Kattamis, Vana Spoulou
Streptococcus pnemoniae is a major cause of morbidity and mortality among splenectomised patients with β-thalassemia major. We have previously shown that a 13-valent pneumococcal conjugate vaccine (PCV13) induces robust early immune responses in such patients, while history of repeated immunisations with the 23-valent polysaccharide pneumococcal vaccine (PPSV23) results in attenuation of the response to PCV13. However, the duration of vaccine-induced protection in splenectomised thalassemic patients and the associated need for booster immunisation remains unclear...
January 25, 2019: Annals of Hematology
https://read.qxmd.com/read/30680524/evaluation-of-osseous-changes-in-dental-panoramic-radiography-of-thalassemia-patients-using-mandibular-indexes-and-fractal-size-analysis
#20
Seval Bayrak, Duygu Göller Bulut, Kaan Orhan, Enver Alper Sinanoğlu, Emine Şebnem Kurşun Çakmak, Melis Mısırlı, Handan Ankaralı
OBJECTIVE: This study aimed to evaluate the radiomorphometric indexes and fractal dimension (FD) on dental panoramic radiography (DPR) of Thalassemia Major (TM) patients. METHODS: In 59 TM patients and in 59 healthy control subjects, mandibular cortical index (MCI), mandibular cortical width (MCW), panoramic mandibular index (PMI), and FD of four different regions (FD1-4) were evaluated and compared using DPRs. RESULTS: The distribution of MCI in TM patients was similar to control subjects (p > 0...
January 24, 2019: Oral Radiology
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