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Osteonecrosis in Youth with Sickle Cell Disease-A Narrative Review
1Azrieli Research Centre of CHU Sainte Justine, Department of Pediatrics, University of Montreal, Montreal, QC H3T 1J4, Canada.
Abstract:
Osteonecrosis can cause significant and chronic musculoskeletal morbidity in children and adolescents with sickle cell disease (SCD). Although the femoral head is most frequently affected, lesions can also occur in the humeral head, vertebral bodies, knees, and other joints. Progressive joint damage can result in chronic pain, impaired mobility, loss of function, and the need for surgical intervention at a young age. As survival continues to improve in SCD, the long-term burden of osteonecrosis is becoming increasingly important. This narrative review summarizes the current understanding of osteonecrosis in pediatric and adolescent SCD. We review its epidemiology, pathophysiology, clinical presentation, imaging findings, and management. Disease burden increases with age and is closely linked to markers of severe SCD, including frequent vaso-occlusive crises and acute chest syndrome.
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