부산대학교 도서관

BACKGROUND: Ireland has been relatively free of sickle-cell disease (SCD) and a care policy for the disease has not been established. AIM: To determine the prevalence of childhood SCD in Ireland and to predict requirements for a comprehensive care and assessment programme. METHODS: We retrospectively analysed the data of children with SCD presenting with sickle-cell crisis to one institution from January 1999 to April 2001. We also determined the nature and severity of the presenting haemoglobinopathy phenotype. RESULTS: Ninety-two patients with haemoglobinopathy have been registered with the Paediatric Haematology Service. The majority are from Nigeria with a smaller number from Angola and the Congo. Sixty have sickle-cell trait, 23 SCD, four haemoglobin SC disease and two haemoglobin E (HbE). There have been 32 sickle-cell crises. The majority were haemolytic or splenic sequestration events with a smaller number of aplastic and vaso-occlusive events and one osteomyelitis. CONCLUSION: The increasing number of children presenting with SCD as a result of the increasing refugee numbers requires a comprehensive care approach similar to that required for paediatric haemophilia to ensure optimum care.