In France, as in the USA and Great Britain, neonatal screening for sicle cell disease has become necessary because this disease is frequent as a monogenic hereditary disease. It ranks second to cystic fibrosis on average and first in the Paris area region. Detection is useful for an early diagnosis so that adequate follow up and treatment can be arranged. The choosen procedure is the analysis by isoelectricfocusing Agarose gel (Isolab), confirmation by citrate agar electrophoresis and quantification by HPLC Variant (BIO-RAD), of a dried blood specimen on filter paper taken selectively in relation to the ethnic origin of the parents. During the first three years, since 1990 by systematic pilot screening 104 sicklers were detected in 172,350 infants tested. Marked differences were noted according to the region. The highest prevalence was registered in the Paris area (1/1200) and the lowest in the North France (1/1600). Since 1993, a selective screening was established and extended to more maternity units leading to a gain of about 50 percent gain in the prevalence in the population tested. In 1995, for the five laboratories performing the screening tests we had found 90 SCD on 59,729 newborns tested (prevalence for Paris area [1/400] and for North France[1/900] and South France [1/5000]). This screening has been accepted as a national program and this service must be offered to all maternity units before the end of 1996.

Evaluating studies refering to the efficacy of selectivity and patient follow up have begun.