[Incidence and prevalence of hemoglobinopathies in children in The Netherlands]

To evaluate the prevalence and incidence of thalassaemia major and sickle-cell disease in children.

Descriptive nationwide epidemiological study.

Clinical Genetic Centre Utrecht.

Prevalence data were collected by a written survey among all 333 Dutch paediatricians (1992; response rate 99.1%). Incidence data are collected monthly by the Dutch Paediatric Surveillance Unit (1992/'93; response rate: 86%).

In September 1992, 128 children were being treated by a paediatrician for sickle-cell disease. Two children had parents born in the Netherlands, but all children were of other ethnic origins, mainly from Surinam, the Dutch Antilles, Turkey and Africa; 50 children were born in the Netherlands. 31 children were under treatment for thalassaemia major, none of them of original Dutch descent; the most frequent ethnic backgrounds were Turkey and Morocco; 20 children were born in the Netherlands. From October 1992 till December 1993 (15 months) 18 children were newly diagnosed with sickle-cell disease, of whom 7 were born in the Netherlands, and 8 children were newly diagnosed with thalassaemia major, of whom 2 were born in the Netherlands.

Sickle-cell disease and thalassaemia major are (still) rare diseases in the Netherlands. With the present migration and the increase of consanguineous marriage, they are expected to become a more important health issue.