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(Beta Thalassemia) OR (Thalassemia Major)

Phumin Chaweephisal, Arunee Phusua, Kanda Fanhchaksai, Supatra Sirichotiyakul, Pimlak Charoenkwan
INTRODUCTION: Identification of beta-thalassemia carrier in prenatal screening relies on the elevated Hb A2 level. Borderline Hb A2 levels pose a diagnostic challenge. We determined the HBB genotypes in subjects with borderline Hb A2 in northern Thailand and studied the effects of coinherited alpha0 -thalassemia on Hb A2 levels. METHODS: Blood samples with Hb A2 3.1-10.0% from 2193 samples submitted for prenatal thalassemia screening were selected. Information on HBB genotypes and coinherited alpha0 -thalassemia were collected...
October 4, 2018: Blood Cells, Molecules & Diseases
Suraj Haridas Upadya, M S Rukmini, Sowmya Sundararajan, B Shantharam Baliga, Nutan Kamath
Background: Thalassemia is the most common genetic disorder worldwide. Use of iron chelators has improved survival but endocrine complications have become more frequent. The frequency of hypothyroidism in Beta Thalassemia Major (BTM) children ranges from 6 to 30 %. Thyroid dysfunction mainly occurs by gland infiltration, chronic tissue hypoxia, free radical injury, and organ siderosis. Objectives: (a) To evaluate the thyroid function status in chronically transfused children with BTM, in the first and second decade of life and (b) to study the influence of factors like duration and amount of blood transfusions, serum ferritin level, and iron chelation therapy on thyroid function...
2018: International Journal of Pediatrics
Manu Goyal, M Dinaker, K Gayathri
CD4 counts along with viral loads are important parameters in the monitoring of human immunodeficiency virus (HIV) infection. Human T-cell lymphotropic virus type I (HTLV-I) is known to be an etiological agent for adult T-cell leukemia/lymphoma (ATLL). Coinfection of HTLV-I and HIV is well known in regions with high seroprevalence, and there is no published data in the Indian scenario. We present an interesting case of occurrence of CD4+ T-cell proliferation in a known beta thalassemia major with acquired HIV seropositivity accompanied by simultaneously increasing CD4+ counts and viral loads...
October 2018: Indian Journal of Pathology & Microbiology
Vikrant Sood, Juhi Chawla, Bikrant B Lal, Rajeev Khanna, Chhagan Bihari, Seema Alam
A 10-year-old female child, a known case of Beta-Thalassemia major with superimposed chronic Hepatitis C Viral (HCV) infection, underwent bone marrow transplantation and presented, 7 months later, with worsening hepatic functions and skin rash. Considering the wide variety of possible etiologies, she was evaluated and later confirmed as a case of hepatic dysfunction secondary to HCV related hepatic injury with concomitant chronic graft versus host disease (dermatological involvement only).
September 2018: Journal of Clinical and Experimental Hepatology
Ali Sabbagh, Bijan Keikhaei, Morteza Joorabian, Masumeh Maleki Behzad, Mohammad Momeni
OBJECTIVE: Portal vein thrombosis (PVT) has been described as a rare complication after splenectomy. PVT associated risk factors after splenectomy in hematological disorders are poorly recognized. The aim of this study was to assess the prevalence and risk factors of PVT incidence in splenectomized patients. METHODS: One hundred twelve splenectomized patients with various hematologic diseases between 2008 and 2018 were enrolled in this study. Diagnosis was confirmed by Doppler ultrasonography (DUSG) and risk factors for PVT were sought based on the comparison of clinical and laboratory features between patients without and with PVT...
September 20, 2018: Blood Cells, Molecules & Diseases
(no author information available yet)
No abstract text is available yet for this article.
October 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
F Q Wen, S X Liu
No abstract text is available yet for this article.
October 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Ziya Salman, Tamer Yılmaz, Güldal Mehmetçik
No abstract text is available yet for this article.
October 1, 2018: Archives of Biochemistry and Biophysics
Christian Mühlfeld, Joana Neves, Christina Brandenberger, Jan Hegermann, Christoph Wrede, Sandro Altamura, Martina U Muckenthaler
Iron accumulates in the lungs of patients with common respiratory diseases or transfusion-dependent beta-thalassemia. Based on our previous work, we hypothesized that systemic iron overload affects the alveolar region of the lung and in particular the surfactant producing alveolar epithelial type II (AE2) cells. Mice with a point mutation in the iron exporter ferroportin, a model for human hemochromatosis type 4 were compared to wildtype mice (n = 5 each). Lungs were fixed and prepared for light and electron microscopy (EM) according to state-of-the-art protocols to detect subcellular iron localization by scanning EM/EDX and to perform design-based stereology...
October 3, 2018: Histochemistry and Cell Biology
Wenguang Jia, Ping Chen, Wenqiang Chen, Yongqing Li
This study aimed to study the differences in Raman spectra of red blood cells (RBCs) among patients with β-thalassemia and controls using laser tweezers Raman spectroscopy (LTRS) system.A total of 33 patients with β-thalassemia major, 49 with β-thalassemia minor, and 65 controls were studied. Raman spectra of RBCs for each sample were recorded. Principal component analysis (PCA), one-way analysis of variance (ANOVA), and independent-sample t test were performed.The intensities of Raman spectra of β-thalassemia (major and minor) RBCs were lower than those of controls, especially at bands 1546, 1603, and 1619 cm...
September 2018: Medicine (Baltimore)
Umberto Barbero, Federico Fornari, Silvia Guarguagli, Carmen Maria Gaglioti, Filomena Longo, Baldassarre Doronzo, Matteo Anselmino, Antonio Piga
The prevalence of atrial fibrillation (AFib) in β-thalassemia major (β-TM) patients has increased in the last few years, reaching up to 33.0%. Several factors may drive this value to even more in the next few years. We summarized the main challenges in the management and therapy of AFib in this very specific group of patients.
October 1, 2018: Hemoglobin
Taylor L Turnbull, Peter M Houston, Nicholas I Batalis
No abstract text is available yet for this article.
September 1, 2018: American Surgeon
Taylor L Turnbull, Peter M Houston, Nicholas I Batalis
No abstract text is available yet for this article.
September 1, 2018: American Surgeon
Ardeshir Ghavamzadeh, Amir Kasaeian, Tahereh Rostami, Azadeh Kiumarsi
BACKGROUND: Allogeneic hematopoietic stem cell transplantation (HSCT) currently is the only available curative option for transfusion-dependent thalassemia. Peripheral blood is a more convenient source for HSCT in comparison with bone marrow. Information about the relative success of transplantation with these two graft sources would help physicians and patients choose between them. The aim of this study was to evaluate the pros and cons of using peripheral blood instead of bone marrow as the graft source in thalassemia transplantation...
September 25, 2018: Biology of Blood and Marrow Transplantation
Lieshu Tong, Josef Kauer, Xi Chen, Kaiqin Chu, Hu Dou, Zachary J Smith
Anemia affects more than ¼ of the world's population, mostly concentrated in low-resource areas, and carries serious health risks. Yet current screening methods are inadequate due to their inability to separate iron deficiency anemia (IDA) from genetic anemias such as thalassemia trait (TT), thus preventing targeted supplementation of oral iron. Here we present an accurate approach to diagnose anemia and anemia type using measures of pediatric red cell morphology determined through machine learning applied to optical light scattering measurements...
September 28, 2018: Lab on a Chip
Ashutosh Lal, Trisha E Wong, Jennifer Andrews, Vinod V Balasa, Jong H Chung, Craig M Forester, Alan K Ikeda, Siobán B Keel, Monica B Pagano, Geetha Puthenveetil, Sanjay J Shah, Jennifer C Yu, Elliott P Vichinsky
BACKGROUND: The severe forms of thalassemia are the most common inherited anemias managed with regular blood transfusion therapy. Transfusion policies and complications are critical to quality of life and survival, but there is a lack of standardized care. STUDY DESIGN AND METHODS: A survey of 58 items was completed in 2016 by 11 centers in California, Washington, Oregon, Nevada, and Arizona providing long-term care for thalassemia. The questionnaire addressed demographic information, transfusion practices and complications, and educational needs...
September 27, 2018: Transfusion
Kamal Dumaidi, Amer Al-Jawabreh, Fekri Samarah, Maha Rabayaa
Background: HCV and HBV present a great challenge in the management of β -thalassemia patients. Objective: The present study aimed to determine the prevalence of both HBV and HCV in multitransfused-dependent β -thalassemia patients in northern West Bank, Palestine, using sero-molecular markers. Methods: Serum sample from 139 multitransfused β -thalassemia patients were tested for HBV and HCV markers including HBsAg, anti-HBc, anti-HBs, HBV-DNA, and anti-HCV and HCV-RNA...
2018: Canadian Journal of Infectious Diseases & Medical Microbiology
Maddalena Casale, Stefania Picariello, Felice Corvino, Giuseppe Cerasari, Saverio Scianguetta, Francesca Rossi, Marcello Persico, Silverio Perrotta
A 20-year-old male affected by transfusion-dependent β-thalassemia (β-thal), was prescribed intensive chelation therapy with deferoxamine (DFO) and deferiprone (DFP) because of severe hepatic and cardiac iron overload and β-blocker and warfarin to manage a previous event of atrial fibrillation (AFib) and heart failure. After a few months, he developed critical liver failure, renal tubulopathy and severe electrolyte imbalance. Laboratory and instrumental evaluations were performed to carry out differential diagnosis of acute liver failure and an exclusion diagnosis of drug induced liver injury (DILI) was made...
September 25, 2018: Hemoglobin
V P Choudhry
No abstract text is available yet for this article.
September 21, 2018: Indian Journal of Pediatrics
Rita Panyang, Anju Barhai Teli, Sidhartha Protim Saikia
Background: Anemia is a condition in which the number of red blood cells becomes insufficient to meet the body's physiologic needs. Anemia is one of the major public health problems in India. The aim of this study was to find the prevalence and determinants of anemia among the adult females of tea garden community of Assam. Materials and Methods: A community-based cross-sectional study was conducted among 770 numbers of adult females belonging to the tea garden community...
July 2018: Journal of Family Medicine and Primary Care
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