Abstract

INTRODUCTION: Sickle cell disease (SCD) is an inherited autosomal recessive disorder with bone mineral density (BMD) as a common clinical manifestation of SCD. With a prevalence of 2.6%, Saudi Arabia is among the highest incidence of SCD worldwide. The purpose of this research was to examine how SCD evolves and how it affects bone density in Saudi patients from an Eastern Province tertiary hospital. METHODS: This was an observational prospective study conducted in the tertiary care hospital among 119 SCD patients. Patients were divided into two groups: Group A – severe SCD patients requiring hospital care ≥3/year; and Group B included patients with a smooth course of SCD who did not require frequent hospitalization (<3 hospitalizations per year), with a milder course of the disease. Analysis was based on the frequency of hospitalizations with pain crises and measuring BMD. RESULTS: Of 119 patients, 73.1% had low bone density. Compared to the femur (47.9%), the spine (62.2%) had a higher prevalence of low bone density. The prevalence of low BMD did not significantly differ between the two groups (64.8 vs. 79.9%, P = 0.081). Patients with more frequent hospital visits had significantly higher Mg concentrations (2.30 vs. 0.84, P = 0.001), higher gamma-glutamyl transferase (59.44 vs. 39.49, P = 0.030), and significantly lower 25-hydroxy Vitamin D (34.82 vs. 49.48, P = 0.004). CONCLUSIONS: Patients with SCD had a generally higher prevalence of low BMD. Further research is needed to answer the proposed debate about the accuracy of DXA scanning in patients with SCD.

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