COMPARATIVE-STUDY OF FANCONI-ANEMIA IN CHILDREN OF DIFFERENT ETHNIC-ORIGIN IN SOUTH-AFRICA

A comparative study of clinical, hematologic, and cytogenetic findings was made in 40 black and 35 white children with Fanconi anemia. The b lack children were Bantu-speaking Negroid stock of diverse tribal orig in. The white children were predominantly Afrikaans stock of Dutch/Ger man/French Huguenot origin. All of the patients had IFAR scores of 2 t o 4+ and over 80% in each group had increased spontaneous and/or mutag en-induced chromosomal breakage (CB-positive). There were no significa nt clinical differences between black and white patients or between CB -pos and CB-neg patients, with the exception of white children in whom significantly more CB-pos patients had thumb and radial anomalies tha n the CB-neg patients. The age-at onset of hematologic manifestations was the same for all groups, but more black than white CB-pos patients were severely anemic at the time of diagnosis. Response to androgen a nd steroid therapy occurred in only 33% of black children compared wit h 86-90% of white children; 81% of black patients died during the 18 y ear study period compared with 30% of white children, but the age at d eath was similar. More sophisticated studies are required to determine whether these differences are genetically determined or related to cu ltural, educational, and socio-economic differences between the two et hnic groups. (C) 1994 Wiley-Liss, Inc.