Background Sickle cell disease (SCD) is a prevalent genetic disorder in which sickle hemoglobin leads to tissue hypoxia and adverse effects on bone. Several studies have shown an overall reduction in bone mineral density (BMD), attributed to marrow hyperplasia, in patients with SCD. Compared with normal subjects from the general population, found that the patients with SCD had lower BMD values in all scan regions. In particular, vertebral osteoporosis is common in patients with sickle cell disease. Minimal data exist on the prevalence of low BMD and its treatment in Albanian adults with SCD.
Objectives The aim of our study was to describe the prevalence of BMD in adults with SCD in Albania population and to evaluate the effectiveness of the bisphosphonates in the treatment of low BMD.
Methods In this cross-sectioonal study, we collected data from 100 consecutive adult patients (55 male and 45 female, mean age 22.5±1.5 years) with SCD and low BMD who were treated at the University Service of Rheumatology, University Hospital Center Mother Theresa, Tirana, Albania, during 2010. BMD measurement was done using dual energy X-ray absorbtiometry (DEXA) at lumbar spine. Osteopenia and osteoporosis were diagnosed according to WHO criteria. All patients were treated with 10mg alendronate per day and 600UI of vitamin D3 and 1g of calcium carbonate. We evaluated their BMD after six and twelve months of treatment.
Results Median lowest T-score was -2.2 (range: -3.4 to -1.1). Seventy-eight per cent of the patients (n=78) had osteoporosis and 22% (n=22) had osteopenia. After treatment, bone mineral density decreased by 13.8% (P<.001) and 7.8% (P=0.003) in osteoporotic patients and by 6.5% (P=0.002) and 3.6% (P=0.141) in osteopenic patients at 6 and 12 months, respectively. Upon adjustment for age and sex, T-score was increased significantly at 6 and 12 months of treatment compared with pretreatment (-1.9±0.4 and -0.9±0.3 vs. -2.1±0.3, p<0.001)
Conclusions The prevalence of osteoporosis in young adults with SCD is very high. Our results suggest that antiresorptive treatment with biphosphonates can improve the bone mineral density. Additional studies are needed to determine the long-term effects of this treatment.
Disclosure of Interest None Declared
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