[ outcome of 105 Tunisian pediatric patients with sickle cell disease]

ABSTRACT

Sickle cell disease is widespread in the world. It is a public health problem in Tunisia. In this paper, we will study the outcome of 105 Tunisians infants affected by this disease. It is a retrospective study over 8 years, enrolling 105 Tunisian infants with sickle cell disease. Their main age is 10 years 7 months [19 months-18 years]. They are distributed into 58 boys and 47 girls. Sixty two of them ate homozygotes S/S, and 43 are double heterozygotes [S/, thalassemics 41 cases, S/O arab=1 case, S/C=1 case]. The main acute complications were vaso occlusive crises in 85 patients. They essentially reach the limbs. One hundred and twenty one events acute anemia" were observed. They are distributed into 107 events splenic sequestration [884%] and 14 events erythroblastopenia [11.6%]. Eleven of our patients [10.4%] have presented 16 events ischemic cerebral vascular [strokes]. Twenty events acute chest syndrome were observed in 15 patients [14.3%]. Infectious complications were noted in 83 patients [79%]. The priapism was observed in only 4 boys out of 58. The chronic degenerative complications were as follows Femur head necrosis in 9 cases, degenerative retinopathy in one case and ulcer leg in one case. Hydroxyurea was reserved to 30 patients with a severe form of the disease. All of them improve the clinical outcome as attested by the number of days of hospitalisation/patient/year, which drops from 30 days to 3 days only [p<0,001]. We not deplore any death. By its complications, sickle cell disease is a potentielly serious disease. Hydroxyurea is an efficient treatment for the most severe forms