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Abstract
Sickle cell disease (SCD) is one of the most common severe monogenic disorders in the world. Hemoglobin polymerization, leading to erythrocyte rigidity and vaso-occlusion, is central to the pathophysiology of this disease. Physical growth is known to be impaired in SCD and affected children are usually lighter and shorter than their healthy counterparts. The prevalence and severity of growth impairment vary with geographical location and are most marked in low-resource settings. Causes of growth retardation in SCD are complex and multiple factors are likely to contribute. The aim of our work was to study growth pattern in sickle cell patients, and to detect differences in growth parameters between sickle SS and sickle Ý. also to detect correlation between blood transfusion, vaso occlusive crises, serum ferrittin and some parameters of growth in the two groups (SS and SÝ). This study was conducted on 80 patients with sickle cell disease (50 sickle SS and 30 SÝ), who were following up at the pediatric hematology clinic, Cairo university and another 50 age-and sex- matched healthy children were enrolled as controls. Growth assessment was done using WHO reference values z-score and pubertal assessment using tanner stage. The study revealed that growth and puberty were affected in all patients with SCD, while there were no significant differences in growth and pubertal parameters between sickle SS and sickle Ý except for body mass index. There for regular and close up of the patients growth and pubertal signs should be performed for detection of any delay and it`s subsequent management