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An update on the recent literature on sickle cell bone disease

Osunkwo, Ifeyinwa

Current Opinion in Endocrinology & Diabetes and Obesity: December 2013 - Volume 20 - Issue 6 - p 539–546
doi: 10.1097/

Purpose of review To summarize the findings of the recent publications on sickle cell bone disease (SBD).

Recent findings Individuals with sickle cell disease (SCD) are living longer and develop progressive organ damage including SBD with age. Recent studies suggest alternative radiological diagnostics such as ultrasound and scintigraphy can detect and differentiate between different forms of SBD. MRI with or without diffusion-weighted sequences remains the gold standard. Case reports of cranio-orofacial SBD highlight the rarity of this presentation. Vitamin D deficiency is highly prevalent at all ages, but may not be an independent risk factor for avascular necrosis (AVN). Gene polymorphisms of the Annexin A gene may predict AVN in SCD. A recent study demonstrated reduced days with pain and improved physical activity quality of life following high-dose vitamin D therapy. The high rates of osteopenia and osteoporosis in SCD support the need for research addressing this rising public health problem. Lastly, results of total hip arthroplasty for AVN in SCD has improved significantly over time with the use of cementless prosthetic material and improved supportive care.

Summary SBD remains poorly studied. Prospective randomized studies targeting predictors, diagnostics, prevention, and treatment options for SBD are sorely needed.

Children's Healthcare of Atlanta and The Department of Pediatrics Emory University, Atlanta, Georgia, USA

Correspondence to Ifeyinwa Osunkwo, MD, MPH, 2361 Academy Court NE, Atlanta, GA 30345, USA. Tel: +1 973 214 4726; e-mail:,

© 2013 Wolters Kluwer Health | Lippincott Williams & Wilkins