SICKLE-CELL DISEASE IN SOUTH ZAIRE - STUD Y OF 2 SERIES OF 251 AND 340 PATIENTS DURING THE PERIOD 1988-1992

Background.- This work concerns 591 sickle cell disease patients follo wed by the same paediatrics team in south Zaire. Population.- Two seri es of homozygous sickle cell patients were studied: 1) 251 in-patients hospitalized in Kolwezi during the period 1988-1989, and 2) 340 out-p atients examined in Lubumbashi during the period 1990-1992. Results.- In the first series, a high proportion of children aged 3-5 years (30. 7%) and 6-12 years (54%) was observed in comparison with a control gro up of patients hospitalized for anemia at the same time. The symptoms of the disease occurred during the first year of life in 80% of the ch ildren: hand-foot syndrome and/or anemia. Tooeth decay was observed as soon as the age of 3 years with a high frequency (62%) as compared wi th the control group. Epistaxis, sometimes very important, was observe d in 39.5% and 52% of the cases in children respectively aged 6-12 yea rs and up to 13. A splenomegaly was noted during a longer period than in the control group, suggesting associated causing factors different from malaria, perhaps alpha thalassemia. The high frequency of viral c ontaminations due to transfusion in illustrated by the seropositivity prevalences of HIV (11.5%), HBV (10%) and in the children in Lubumbash i. Conclusion.- The main interest of these series of children is to po int out the clinical specificities of a cohort genetically homogenous, offering the opportunity of defining basis of inter-individual variab ility of sickle cell disease.