دورية أكاديمية

Current challenges in the management of patients with sickle cell disease – A report of the Italian experience

التفاصيل البيبلوغرافية
العنوان: Current challenges in the management of patients with sickle cell disease – A report of the Italian experience
المؤلفون: Giovanna Russo, Lucia De Franceschi, Raffaella Colombatti, Paolo Rigano, Silverio Perrotta, Vincenzo Voi, Giovanni Palazzi, Carmelo Fidone, Alessandra Quota, Giovanna Graziadei, Antonello Pietrangelo, Valeria Pinto, Giovan Battista Ruffo, Francesco Sorrentino, Donatella Venturelli, Maddalena Casale, Francesca Ferrara, Laura Sainati, Maria Domenica Cappellini, Antonio Piga, Aurelio Maggio, Gian Luca Forni
المصدر: Orphanet Journal of Rare Diseases, Vol 14, Iss 1, Pp 1-9 (2019)
بيانات النشر: BMC, 2019.
سنة النشر: 2019
المجموعة: LCC:Medicine
مصطلحات موضوعية: Hemoglobin disorder, Hemoglobinopathy, Hydroxyurea, Migrants, Sickle cell screening, Sickle cell disease, Medicine
الوصف: Abstract Sickle cell disease (SCD) is an inherited red blood cell disorder caused by a structural abnormality of hemoglobin called sickle hemoglobin (HbS). Clinical manifestations of SCD are mainly characterized by chronic hemolysis and acute vaso-occlusive crisis, which are responsible for severe acute and chronic organ damage. SCD is widespread in sub-Saharan Africa, in the Middle East, Indian subcontinent, and some Mediterranean regions. With voluntary population migrations, people harboring the HbS gene have spread globally. In 2006, the World Health Organization recognized hemoglobinopathies, including SCD, as a global public health problem and urged national health systems worldwide to design and establish programs for the prevention and management of SCD. Herein we describe the historical experience of the network of hemoglobinopathy centers and their approach to SCD in Italy, a country where hemoglobinopathies have a high prevalence and where SCD, associated with different genotypes including ß-thalassemia, is present in the native population.
نوع الوثيقة: article
وصف الملف: electronic resource
اللغة: English
تدمد: 1750-1172
Relation: http://link.springer.com/article/10.1186/s13023-019-1099-0; https://doaj.org/toc/1750-1172
DOI: 10.1186/s13023-019-1099-0
URL الوصول: https://doaj.org/article/4eb86eed929a495c968f5398eba2981d
رقم الأكسشن: edsdoj.4eb86eed929a495c968f5398eba2981d
قاعدة البيانات: Directory of Open Access Journals
الوصف
تدمد:17501172
DOI:10.1186/s13023-019-1099-0