[Sickle cell anemia in the south of Zaire. Study of two series of 251 and 340 patients followed-up 1988-1992].
BACKGROUND
This work concerns 591 sickle cell disease patients followed by the same paediatrics team in south Zaire.


METHODS
Two series of homozygous sickle cell patients were studied: 1) 251 in-patients hospitalized in Kolwezi during the period 1988-1989, and 2) 340 out-patients 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 group 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 children: hand-foot syndrome and/or anemia. Tooth decay was observed as soon as the age of 3 years with a high frequency (62%) as compared with the control group. Epistaxis, sometimes very important, was observed in 39.5% and 52% of the cases in children respectively aged 6-12 years 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 contaminations due to transfusion is illustrated by the seropositivity prevalences of HIV (11.5%), HBV (10%) and in the children in Lubumbashi.


CONCLUSIONS
The main interest of these series of children is to point out the clinical specificities of a cohort genetically homogenous, offering the opportunity of defining basis of inter-individual variability of sickle cell disease.
Tshilolo
L
L
Service de pédiatrie, hôpital Gécamines, Kolwesi, Zaïre.
Mukendi
R
R
Girot
R
R
D000293:Adolescent; D000367:Age Factors; D000755:Anemia, Sickle Cell / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology* / Q000208:ethnology; D002648:Child; D002675:Child, Preschool; D015023:Democratic Republic of the Congo / Q000453:epidemiology; D003710:Demography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D014777:Virus Diseases / Q000635:transmission
La drépanocytose dans le sud du Zaïre. Etude de deux séries de 251 et 340 malades suivis entre 1988 et 1992.