[Sickle cell disease in the People's Republic of Angola. 1. Epidemiology and clinical aspects].

: In a period of 6 years 819 children affected with drepanocytosis (S. E.) were treated at the children's clinic in Luanda. At the time of diagnosis the age of the 457 boys amounted to 3.4 +/- 2.7 years and that of girls to 4.3 +/- 3.4 years. The age distribution shows that 40.2% of the patients were in their first 2 years of life. Conditions of crisis which affected 70% of the diseased children are typical of this disease. The family anemnesis demonstrated that 13.6% of all brothers and sisters covered were HbSS positive. 34.1% of them died at an age of 3.5 +/- 3.5 years (boys) or 2.4 +/- 2.3 years (girls) respectively. The physical development measured in the body mass shows that it is significantly diminished in comparison with healthy children of the same age. The characteristic symptom of this disease, splenomegaly, existed in 27.8% of the children with a mean age of 2.8 +/- 2.3 years. In one age group of 4.4 +/- 2.6 years it could even be identified in 11.3% of the cases.