مشخصات مقاله | |
ترجمه عنوان مقاله | تالاسمی بتا: نظارت و رویکردهای درمانی جدید |
عنوان انگلیسی مقاله | Beta Thalassemia: Monitoring and New Treatment Approaches |
انتشار | مقاله سال 2019 |
تعداد صفحات مقاله انگلیسی | 15 صفحه |
هزینه | دانلود مقاله انگلیسی رایگان میباشد. |
پایگاه داده | نشریه الزویر |
نوع نگارش مقاله |
مقاله مروری (Review Article) |
مقاله بیس | این مقاله بیس نمیباشد |
نمایه (index) | MedLine – Scopus – Master Journals List – JCR |
نوع مقاله | ISI |
فرمت مقاله انگلیسی | |
ایمپکت فاکتور(IF) |
3.014 در سال 2018 |
شاخص H_index | 74 در سال 2019 |
شاخص SJR | 1.683 در سال 2018 |
شناسه ISSN | 0889-8588 |
شاخص Quartile (چارک) | Q1 در سال 2018 |
مدل مفهومی | ندارد |
پرسشنامه | ندارد |
متغیر | ندارد |
رفرنس | دارد |
رشته های مرتبط | پزشکی |
گرایش های مرتبط | ژنتیک پزشکی، خون و آنکولوژی |
نوع ارائه مقاله |
ژورنال |
مجله | کلینیک های آنکولوژی/هماتولوژی آمریکای شمالی – Hematology/oncology Clinics Of North America |
دانشگاه | Division of Hematology, Children’s Hospital of Philadelphia, 3401 Civic Center Boulevard, Colket Translational Research Building, Room 11024, Philadelphia, PA 19104, USA |
کلمات کلیدی | تالاسمی بتا، بار اضافی آهن، چنگاله و ژن درمانی |
کلمات کلیدی انگلیسی | Beta thalassemia، Iron overload، Chelation، Gene therapy |
شناسه دیجیتال – doi |
https://doi.org/10.1016/j.hoc.2019.01.003 |
کد محصول | E12976 |
وضعیت ترجمه مقاله | ترجمه آماده این مقاله موجود نمیباشد. میتوانید از طریق دکمه پایین سفارش دهید. |
دانلود رایگان مقاله | دانلود رایگان مقاله انگلیسی |
سفارش ترجمه این مقاله | سفارش ترجمه این مقاله |
فهرست مطالب مقاله: |
INTRODUCTION
BETA THALASSEMIA PATHOPHYSIOLOGY BETA THALASSEMIA THERAPY IRON OVERLOAD AND CHELATION ALLOGENEIC STEM CELL TRANSPLANT MONITORING NEW THERAPEUTIC APPROACHES SUMMARY REFERENCES |
بخشی از متن مقاله: |
INTRODUCTION Beta thalassemias represent a class of disorders with a high global prevalence and significant health and economic impact.1 Since the elucidation of the molecular mechanism in the 1960s, there has been significant progress in treatment of disease complications. With increasing use of transfusion therapy, iron overload has become a pressing problem, and chelation therapy is a key component of treatment. The first part of this review focuses on monitoring of disease complications, transfusion therapy, iron overload, and chelator toxicity. In the second part, the authors review new developments in therapy for beta thalassemia. BETA THALASSEMIA PATHOPHYSIOLOGY Thalassemias are a class of disorders caused by imbalance of the alpha (a) and beta (b) globin chains that make up the principal adult oxygen transporter hemoglobin A (a2b2). Beta thalassemias result from a relative excess of a chains due to reduced production of b globin chains and, in some instances, increased dosage of a globin genes.2 In addition to reduced functional hemoglobin production, red blood cells (RBCs) and their precursors are damaged by a globin, which is unstable in the absence of a binding partner. Free a globin forms precipitates, leads to formation of reactive oxygen species, and damages RBC membranes leading to hemolysis and abnormal erythroid maturation. The beta thalassemia phenotype is determined by the degree of the imbalance and ranges from minimal effects in beta thalassemia trait to severe transfusion-dependent anemia. Symptoms in beta thalassemia are due to a combination of anemia and ineffective erythropoiesis. Increased erythropoietin levels due to anemia drive erythroblast proliferation through JAK2-STAT5 signaling; additional RBC extrinsic and intrinsic factors have been implicated in this process and are reviewed elsewhere.3 Complications of beta thalassemia are numerous and include growth failure, bone disease, cardiac abnormalities (pulmonary hypertension, heart failure, arrhythmias), predisposition to thrombosis, extramedullary hematopoiesis (splenomegaly, masses with compression), and a broad range of endocrinopathies. |