Natural history of sickle hemoglobinopathies in Omani children

Purpose. Sickle hemoglobinopathies as described in neighbouring countries exhibits a wide spectrum of clinical behaviour. In Oman, however, its clinical course has never been characterized and therefore a study of the natural history of sickle cell disease was undertaken. Patients and Methods. Using retrospective review of case notes, a total of 375 Omani children, aged between 3 months and 16 years were studied. The severity index for each patient was calculated from a total score of 12 clinical and laboratory variables. All the cases were diagnosed using a high performance liquid chromatography technique and level of hemoglobin A2 was determined using elution method. Seventy six cases of homozygous SCA were haplotyped. Fifty of them were mapped for presence or absence of Alpha Thalassemia trait. Results and Conclusions. A total of 157 cases (41.9%) were diagnosed before the age of one year. The severity index correlated well with the age of diagnosis (P