THE FEATURES OF SICKLE-CELL DISEASE IN SAUDI CHILDREN

Using a prospective and retrospective approach, the features of sickle cell disease (SCD) were investigated in 137 SCD children from the south-western region of Saudi Arabia.The patients were followed for a period of 2-5 years, during which period the severity of the disease was assessed and a 'severity index', was calculated for individual patients.The patients with SCD were classified into five groups based on the absence of thalassaemia (sickle cell anaemia, SCA), presence of β°-thalassaemia (HbS/β°-thalassaemia), SCA with α-thalassaemia.2 [heterozygotes (-α/αα) or homozygotes (-α/α)] and S/β°-thaalassaemia with α-thalassaemia. The results showed a high prevalence of associated α-thalassaemia and variable levels of HbF in these patients. SCA patients with associated α-thalassaemia (-α/-α) and S/β°-thalissaemia patients with one α-gene deletion had the highest values for haematological parameters and lowest values of red cell indices. No specific difference could be identified in the clinical manifestations hi the different groups with the exception that long bone crisis and hand-foot syndrome were not encountered hi patients with associated α-thalassaemia. The frequency of hepatomegaly and splenomegaly was also lower in this group. © 1990 Oxford University Press.