SUMMARY

Introduction. Sickle-cell anaemia is a systemic disease caused by a genetic haemoglobin disorder that is transmitted by autosomal recessive inheritance. It appears most frequently in geographical areas in which malaria is endemic, though this geographical pattern has varied with recent migratory flows. The estimated incidence of sickle-cell anaemia is low in Spain. Although the clinical manifestations depend on genotype and age, in its most severe forms clinical disease onset may occur in the first months of life and lead to elevated mortality, due principally to infections caused by encapsulated bacteria. Neonatal sickle-cell anaemia screening aims at initiating early prophylactic antibiotic treatment, so as to reduce the incidence and mortality of pneumococcal infection in children with sickle-cell anaemia.

Objective. To assess the efficacy/effectiveness and safety of neonatal sickle-cell disease screening.

Methods. Systematic review of the scientific literature (Updating a health technology assessment report conducted by Galician Agency for Health Technology Assessment in 2004). Electronic search was conducted in databases specialised in systematic reviews, general databases and databases of ongoing research projects. A supplementary search on preventing pneumococcal infection in children (antibiotic prophylaxis and vaccination) was performed. Critical appraisal of scientific literature was conducted by a single reviewer.

Results, conclusions and recomendations see pdf below