Clinical features of homozygous SS disease in Jamaican children

ABSTRACT

The clinical features in the first 12 years of life of 100 patients with homozygous SS anaemia are described. The majority of patients presented before age 2 years. The earliest diagnosis was made at 3 months of age. The hand foot syndrome, painful crisis and megaloblastic change were early manifestations of disease, whereas leg ulceration did not occur until beyond the age of 6 years. No examples of haemolytic crises were seen. Growth did not appear to be retarded by Jamaican standards. Death occurred in 2 cases (AU)