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Beta Thalassemia

Mohammad Yaser Kiarudi, Kaveh Fadakar, Ebrahim Mousavi
PURPOSE: To describe a case of bilateral choroidal neovascularization in a patient with beta-thalassemia major and an interventional therapeutic attempt. METHOD: Retrospective case report. RESULT: A known case of beta-thalassemia major presented with gradual vision loss. Fundoscopy revealed bilateral macular edema accompanied by intraretinal hemorrhages. Optical coherence tomography and fluorescein angiography were compatible with the diagnosis of choroidal neovascularization...
October 20, 2016: Retinal Cases & Brief Reports
Mahmood Rasool, Arif Malik, Uzma Jabbar, Irshad Begum, Mahmood H Qazi, Muhammad Asif, Muhammad I Naseer, Shakeel A Ansari, Jummanah Jarullah, Absarul Haque, Mohammad S Jamal
To check the amount of cellular damage caused by serial transfusions of blood in thalassemia patients.  Methods: A cross-sectional study was conducted in the University of Lahore, Lahore, Pakistan between August 2012 and December 2012. A total of 43 thalassemia patients underwent at least 10 blood transfusions. Comprehensive biochemical analysis of blood was performed to record the levels of creatinine, urea, uric acid, albumin, liver function tests, malondialdehyde (MDA), and ferritin.  Results: Serum creatinine (0...
November 2016: Saudi Medical Journal
Eirini Lioudaki, Martin Whyte
Patients with higher liver iron stores are likely to have a worse cardiac outcome following noncompliance with chelation. Cardiovascular magnetic resonance identifies myocardial siderosis allowing optimization of iron chelation regimes. Diabetes puts thalassemic patients at increased risk of myocardial fibrosis. Dual chelation therapy with deferoxamine and deferiprone offers improved cardiac outcomes.
October 2016: Clinical Case Reports
Shaik Jani Basha, N R Shetty, Harshad Devarbhavi
We report the case of a 42 year male with history of chronic anaemia who was found to have pernicious anaemia with beta thalassemia trait and had on esophago-gastric-duodenoscopy, gastric carcinoids with gastric atrophy. Pernicious anaemia and gastric carcinoids occurring simultaneously in a single individual is rare. Our case emphasises the need for esophago-gastric-duodenoscopy in cases of pernicious anaemia.
July 2016: Journal of the Association of Physicians of India
Kanay Yararbaş, Yasemin Ardıçoğlu, Nejat Akar
No abstract text is available yet for this article.
October 18, 2016: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
Max B van Gent, Wendeline J van der Made, Perla J Marang-van de Mheen, Koen E van der Bogt
OBJECTIVE: To analyze the rate of infections and complications after surgeon-performed, largely ultrasound-guided, central venous catheter (CVC) placement in a pediatric population and to identify patients at high risk of complications. METHODS: All children aged between 4 months and 19 years with a percutaneous CVC inserted between January 1, 2000, and July 31, 2013, were included. Patient records were reviewed retrospectively for the occurrence of infection and other complications until CVC removal or the last outpatient clinic visit and compared between patient groups and with the recent literature...
October 14, 2016: Surgical Infections
Huma Mansoori, Sidra Asad, Anila Rashid, Farheen Karim
No abstract text is available yet for this article.
September 2016: Blood Research
Vincenzo Russo, Andrea Antonio Papa, Anna Rago, Gerardo Nigro
No abstract text is available yet for this article.
September 28, 2016: International Journal of Cardiology
Laleh Shariati, Hossein Khanahmad, Mansoor Salehi, Zahra Hejazi, Ilnaz Rahimmaesh, Mohammad Amin Tabatabaiefar, Mohammad Hossein Modarressi
BACKGROUND: β-thalassemia is a major group of human genetic disorders which involves decrease or cease in the normal synthesis of the β-globin chains of hemoglobin. KLF1 is a key regulatory molecule involved in the γ to β-globin gene switching process directly inducing the expression of beta globin gene and indirectly repressing gamma globin. This study was performed to investigate the ability of an engineered CRISPR/Cas9 system to disrupt the KLF1 gene to inhibit the γ to β hemoglobin switching process in K562 cells...
September 26, 2016: Journal of Gene Medicine
Sharifeh Khosravi, Mansour Salehi, Mahboobeh Ramezanzadeh, Hamed Mirzaei, Rasoul Salehi
BACKGROUND AND AIMS: Thalassemia is curable by bone marrow transplantation; however, finding suitable donors with defined HLA combination remains a major challenge. Cord blood stem cells with preselected HLA system through preimplantation genetic diagnosis (PGD) proved very useful for resolving scarce HLA-matched bone marrow donors. METHODS: A thalassemia trait couple with an affected child was included in this study. We used informative STR markers at the HLA and beta globin loci to develop a single cell multiplex fluorescent PCR protocol...
May 2016: Archives of Medical Research
Jean-Benoît Arlet, Flavia Guillem, Mathilde Lamarque, Michael Dussiot, Thiago Maciel, Ivan Moura, Olivier Hermine, Geneviève Courtois
Major advances have been recently made in understanding the molecular determinants of dyserythropoiesis, particularly due to recent works in β-thalassemia. The purpose of this review is devoted to underline the role of some proteins recently evidenced in the field, that may be new alternative therapeutic targets in the near future to alleviate different types of anemia. Areas covered: This review covers the contemporary aspects of some proteins involved in various types of dyserythropoiesis, including the transcriptional factor GATA-1 and its protective chaperone HSP70, but also cytokines of the transforming growth factor beta (TFG-β) family, TGF-β1 and GDF-11, and hormones as erythroferrone...
October 6, 2016: Expert Review of Proteomics
Shardul Soman, G D Tharadara, Naitik Chhatrala, Shubham Jain
INTRODUCTION: Extramedullary hematopoeisis (EMH) is defined as formation of blood cells outside the bone marrow. It occurs most commonly in the liver and spleen in patients having disorders that lead to chronic anaemia. EMH in spinal canal is a very rare site and cauda equina syndrome due to EMH has very few cases presented in literature. CASE PRESENTATION: A 28 year old male patient presented with complain of incontinenance of bladder and bowel along with saddle anaesthesia from 10 days...
2016: International Journal of Spine Surgery
Hamid Amoozgar, Samaneh Zeighami, Sezaneh Haghpanah, Mehran Karimi
BACKGROUND: The goal of this study was to compare heart function and arrhythmia in clinically asymptomatic patients with beta thalassemia intermedia and beta thalassemia major. METHOD: In this cross-sectional study, 60 patients with beta thalassemia major and 60 patients with beta thalassemia intermedia who had clinically no symptoms of arrhythmia and clinically normal heart function were evaluated using 24-hour ambulatory electrocardiogram monitoring and echocardiography...
September 20, 2016: Hematology (Amsterdam, Netherlands)
Katja Walpurgis, Andreas Thomas, Matthias Vogel, Christian Reichel, Hans Geyer, Wilhelm Schänzer, Mario Thevis
Sotatercept (formerly ACE-011) is a glycosylated, dimeric fusion protein composed of the extracellular domain of the human activin receptor type IIA (ActRIIA) and the Fc region of human IgG1. The protein-based drug candidate acts as a ligand trap which competitively binds to activin A and other members of the transforming growth factor beta superfamily, thus blocking signalling through ActRIIA. Since the inhibition of activin A was found to significantly increase bone formation and quality, Sotatercept was originally developed for the treatment of diseases involving bone loss...
September 20, 2016: Drug Testing and Analysis
Zari Tahannejad Asad, Majid Ghazanfari, Seyyed Nima Naleini, Azam Sabagh, Wesam Kooti
INTRODUCTION: Regarding the functioning of the endocrine system, and especially in the thyroid of patients with thalassemia, multiple studies in different parts of the world have reported conflicting results. The aim of this study was to assess the levels of thyroid hormones and TSH in beta-thalassemic patients in the city of Ahwaz. METHODS: In this matched case-control study, 105 patients in the case group and 105 subjects as controls were randomly selected from clients referred to the Abuzar hospital in 2015-2016...
July 2016: Electronic Physician
Helen Bornaun, Reyhan Dedeoglu, Kazim Oztarhan, Savas Dedeoglu, Erkan Erfidan, Muge Gundogdu, Gonul Aydogan, Dicle Cengiz
BACKGROUND: Myocardial iron overload is the most common cause of mortality in patients with thalassemia major (TM), also known as beta-thalassemia. T2* cardiovascular magnetic resonance imaging (MRI) is the best way of monitoring cardiac iron, and new echocardiographic techniques can be used to assess cardiac function. OBJECTIVES: The aim of this study was to assess the systolic and diastolic right ventricular (RV) function of patients with TM using tissue Doppler imaging (TDI) and to determine whether this echocardiographic technique is an adequate diagnostic tool for the screening and detection of subclinical cardiac dysfunction...
June 2016: Iranian Journal of Pediatrics
Mehrnoush Kowsaryan, Shiva Rahimi, Mandana Zafari, Mehran Fazli, Masoomeh Mousavi
No abstract text is available yet for this article.
August 26, 2016: Annals of Hematology
Maria Ester Bernardo, Alessandro Aiuti
Gene therapy (GT) approaches based on autologous hematopoietic stem cell (HSC) corrected ex vivo have shown therapeutic benefit in a number of inherited disorders. GT bares the advantage of allowing each patient to be her/his own donor while reducing the risks of immune-mediated complications as compared with allogeneic hematopoietic stem cells transplantation (HSCT). In order to achieve stable engraftment of HSC, patients undergoing transplantation of allogeneic or autologous HSC receive a chemotherapy- and/or radiotherapy-based preparation...
August 16, 2016: Human Gene Therapy
Elizabeth A Traxler, Yu Yao, Yong-Dong Wang, Kaitly J Woodard, Ryo Kurita, Yukio Nakamura, Jim R Hughes, Ross C Hardison, Gerd A Blobel, Chunliang Li, Mitchell J Weiss
Disorders resulting from mutations in the hemoglobin subunit beta gene (HBB; which encodes β-globin), mainly sickle cell disease (SCD) and β-thalassemia, become symptomatic postnatally as fetal γ-globin expression from two paralogous genes, hemoglobin subunit gamma 1 (HBG1) and HBG2, decreases and adult β-globin expression increases, thereby shifting red blood cell (RBC) hemoglobin from the fetal (referred to as HbF or α2γ2) to adult (referred to as HbA or α2β2) form. These disorders are alleviated when postnatal expression of fetal γ-globin is maintained...
September 2016: Nature Medicine
Rasoul Baharlou, Mohammad Hassan Davami, Abbas Ahmadi Vasmehjani, Morteza Ebrahimi
BACKGROUND/AIM: Recent studies have shown that IL-17-producing CD4+ T helper (Th17) cells play an important role in proinflammatory processes. In this report we analyzed IL-17, IL-21, and TGF-β serum levels in the peripheral blood of Iranian beta-thalassemia major patients that clinically exhibited splenectomy and iron overload. MATERIALS AND METHODS: Blood samples were collected from 43 beta-thalassemia patients and 43 healthy individuals with no history of malignancies or autoimmune disorders...
2016: Turkish Journal of Medical Sciences
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