Sickle cell disease in childhood in Madina

ABSTRACT

Sickle cell disease [SCD] is a common disease in Saudi Arabia, with a high prevalence in the Eastern and Southern regions. This study reports on 53 cases of SCD encountered in the Madina area. In a retrospective study of 6000 pediatric patients, 53 children [0.88%] with sickle cell disease were admitted in the Maternity and Children's Hospital at Madina, between November 1990 and October 1991. Of these, 39 patients [73.58%] were Saudis and 14 [26.41%] were non-Saudis. Thirty-six patients were homozygous SS and 17 were sickle thalassemic. The main causes of admission were vaso-occlusive crisis [77.35%], infection [67.92%], acute chest syndrome [22.64%], anemia [12.6%], and cerebrovascular accident [9.43%]. The lowest and highest age groups recorded in this study were six months and 12 years, respectively. About 70% of the patients are still being followed up, and none of the patients has died. This disease is one of the major causes of morbidity in this region of Saudi Arabia. Measures required include neonatal screening programs for the early detection of the disease, as well as research into new drugs to counter the disease