TY - JOUR AU - Russo, Giovanna AU - De Franceschi, Lucia AU - Colombatti, Raffaella AU - Rigano, Paolo AU - Perrotta, Silverio AU - Voi, Vincenzo AU - Palazzi, Giovanni AU - Fidone, Carmelo AU - Quota, Alessandra AU - Graziadei, Giovanna AU - Pietrangelo, Antonello AU - Pinto, Valeria AU - Ruffo, Giovan Battista AU - Sorrentino, Francesco AU - Venturelli, Donatella AU - Casale, Maddalena AU - Ferrara, Francesca AU - Sainati, Laura AU - Cappellini, Maria Domenica AU - Piga, Antonio AU - Maggio, Aurelio AU - Forni, Gian Luca PY - 2019 DA - 2019/05/30 TI - Current challenges in the management of patients with sickle cell disease – A report of the Italian experience JO - Orphanet Journal of Rare Diseases SP - 120 VL - 14 IS - 1 AB - 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. SN - 1750-1172 UR - https://doi.org/10.1186/s13023-019-1099-0 DO - 10.1186/s13023-019-1099-0 ID - Russo2019 ER -