Mortality and causes of death in children with sickle cell disease in the Netherlands, before the introduction of neonatal screening

Summary This study analyzed the mortality and causes of death in sickle cell disease patients in the Netherlands, to provide a baseline for monitoring the effect of the recently introduced neonatal screening programme and to indicate areas of improvement in the care for these patients. All children (<18 years) diagnosed with sickle cell disease in a tertiary hospital from 1985 to 2007 were included. Vital status was determined up to March 2008. A total of 298 children were included: 189 (63%) patients had HbSS, 17 (6%) HbSβ 0 thalassaemia, 72 (24%) HbSC and 20 (7%) HbSβ + thalassaemia. Twel... Mehr ...