A Clinico-Epidemiological Study of Sickle Cell Anaemia in Saudi Arabia

Abstract

Ninety-nine Saudi Arabian children aged between 3 months and 15 years attending the sickle cell anaemia (SCA) clinic were studied using a review of case notes and an interview at clinic visits. All the patients come from the southwest and west area of Saudi Arabia. Despite the lack of infant screening for SCA, 73 per cent were diagnosed before the age of 3 years, reflecting severe early symptoms of the disease. The serious complications of SCA were two times higher, the number of admissions were three times higher and the attack rate for pneumococcal meningitis was nine times higher in our study population than previously reported from the Eastern Province of the country. Hand-foot syndrome was the commonest complication affecting 58 per cent of the children. It has been stated previously that there is no need to give penicillin prophylaxis to SCA children in Saudi Arabia, based on the observations in the Eastern Province only. Our study shows that SCA in western and southwestern population of Saudi Arabia is as severe as is described in American blacks. Therefore, we recommend penicillin prophylaxis to be given to the SCA children in the southwest and west regions of the country.

Clinical and haematological profile of children with sickle cell anaemia admitted to a rural medical college of Chhattisgarh, India

“…This is similar to the findings reported by Al Saqladi AW et al in Yemeni children and other parts of Saudi Arabia. 4,5 Retrospective data analysis with prospective cohort study done by Jain D et al in Maharashtra also reported the mean age of presentation at 3.85 years. 6 However in American and Jamaican cohorts the age of diagnosis is earlier and almost all children were diagnosed during infancy.…”

Morbidity and pregnancy outcomes associated with sickle cell anemia among Saudi women

“…This difference might reflect a possible improvement in the management of SCD in Saudi Arabia; however, this point requires further evaluation [13–15]. The increased rates of stillbirth and IUGR reported among pregnant women with SCD could be owing to compromised placental blood flow secondary to maternal vaso‐occlusion of the placenta that leads to placental infarction and insufficient function [16]. The effect of chronic maternal anemia and pregnancy‐induced hypertension among patients with SCD further complicates this scenario.…”

“…Previous studies revealed 2 different forms of SCD that are prevalent in Saudi Arabia [16,20]; the Asian β globin haplotype, which is more common in the Eastern region, and the Benin haplotype (African origin), which is more prevalent in the Western region. Clinically, the SCD encountered among patients from the Eastern region of the country has features consistent with elevated hemoglobin F levels and, more frequently, α‐thalassemia compared with patients from the Western region, where SCD appears to be more severe [16,20].…”

Clinical Expression of Homozygous Sickle Cell Gene in Yemeni Children = الأعراض السريرية التي يعاني منها الأطفال اليمنيون المصابون بفقر الدم المنجلي

“…Splenomegaly was common presentation in this study (45.7%) and this, in some patients, may progress to rapid blood sequestration in spleen causing acute splenic sequestration. Splenic sequestration crisis occurred in (6.6%) of our patients as compared to (7%) in Omanis (13) , (1%) in Eastern Saudi Arabia and (2.2%) in South Western Saudi Arabia (14,18) . All these rates are much lower than the reported rate of (25%) from Jamaica (15) as seen in table -4 .The incidence of aplastic crisis was (5.7%) in our cases which is more than that reported in Omanies (2%) (13) , and Eastern Saudi Arabia (2 %) (14) but markedly lower than the incidence reported from Jamaica (9%) (15) .…”