Colombatti Raffaella, Dalla Pozza Laura Visonà, Mazzucato Monica, Sainati Laura, Pierobon Marta, Facchin Paola
Haematologica. 2008 Mar;93(3):463-4. doi: 10.3324/haematol.11766.
Sickle cell disease (SCD) has become a paradigm of immigration hematology in Europe. Accurate up-to date information is needed to determine SCD prevalence, define real burden of disease and develop appropriate clinical networks of care, especially in regions lacking screening programs. We used two independent sources of data (Regional Register of Rare Disorders and Regional Register of Hospital Discharge Records) to determine extent of SCD and pattern of hospitalization of pediatric patients in the Veneto Region of NorthEast Italy. A steady increase of case notifications and hospitalizations has been observed in the past five years. Ninety-five percent of patients are immigrants with HbS/HbS SCD. Specialized regional registers can be used to define disease extent and guide targeted interventions in regions still lacking comprehensive care screening programs.
镰状细胞病（SCD）已成为欧洲移民血液学的一个范例。需要准确的最新信息来确定SCD的患病率，明确疾病的实际负担，并建立适当的临床护理网络，尤其是在缺乏筛查项目的地区。我们使用了两个独立的数据来源（罕见疾病区域登记册和医院出院记录区域登记册）来确定意大利东北部威尼托地区儿科患者的SCD范围和住院模式。在过去五年中，病例报告和住院人数稳步增加。95%的患者是携带HbS/HbS SCD的移民。专门的区域登记册可用于确定疾病范围，并指导在仍缺乏全面护理筛查项目的地区进行有针对性的干预。
