EPIDEMIOLOGIC AND CLINICAL-STUDY OF SICKLE-CELL DISEASE IN FRANCE, FRENCH-GUIANA AND ALGERIA

The main clinical and haematological features of sickle cell patients were compared in 618 French, 50 Guianese and 87 Algerian patients. In homozygous sickle cell patients, the proportion of icteric subjects ri ses with age in all centres; the prevalence of splenomegaly reaches a peak in children from 1 to 5 years and then decreases; jaundice and sp lenomegaly are more often noted in Algerian and Guianese than French p atients. The prevalence of painful crisis is comparable in the 3 centr es. In 465 French SS children, having a mean age of 7.3 +/- 5.9 years, the prevalence of a past history of meningitis is 7.3 %, of septicaem ia 4.1 % of osteomyelitis 8.8 These percentages do not differ signific antly between countries. Prevalence of a past history of cerebrovascul ar accident is 3.2 % in French SS patients; 1.2 % in SC, 3.8 % in Sbet a thalassaemia. A past history of acute splenic sequestration was note d significantly more often in SS (11.75 %) and Sbeta thalassaemia (14. 3 %) than SC (3.6 %) in French children (p < 0.05). Proportions of sub jects transfused at least once do not differ between countries; SS chi ldren are more transfused (64 %) than SC (15.6 %) and Sbeta thalassaem ic (66 %) (p < 10-4). Haemoglobin and reticulocyte counts do not diffe r significantly between countries. In conclusion, no major differences were detected between French, Guianese and Algerian homozygous sickle cell patients: this may be due to the fact that France is in itself a mosaic of ethnic origins.