[Adults with XXXX in Senegal. Clinical study of 40 homozygote subjects].

@entity1 @entity3004 @entity3458 @entity39
While the well-documented life expectancy of @entity1 with homozygous @entity3458 (SS) is 40 years in industrialized countries, this question remains @entity3004 . The purpose of this prospective study was to establish the clinical phenotype for Senegal. A severity score based on 12 clinical, laboratory, radiological, and prognostic findings was calculated and correlated with age and hemoglobin F level. A total of 40 SS homozygotes over 15 years of age (mean age: 25 years) were hospitalized between January 1996 and January 1998 at the Principal Hospital in Dakar. The most common events requiring hospitalization were vasoocclusive phenomena (n = 26) but the incidence of these complications declined significantly with age (p < 0.05). The mean hemoglobin level was 4.4 mmol/l and the mean hemoglobin F level was 6.2 p. 100. The incidence of visceral involvement was low (lithiasic vesicules in 17 cases, @entity39 of the head of the femur in 7, and abnormal cardiac ultrasound findings in 10). Only one @entity1 died during the study. No correlation was found between severity score and either age or hemoglobin F level. These findings confirm that the phenotype is less severe in Senegal. However they also show that organ damage is common by the time that @entity1 reach adulthood and thus underline the need for prevention and education to improve survival of SS homozygotes in Senegal.
