THE MUSCULOSKELETAL COMPLICATIONS OF CYSTIC-FIBROSIS

Objective: To determine the spectrum of musculoskeletal complications of cystic fibrosis (CF) in a paediatric population in Australia. Metho d: Clinical assessment followed by serology and bone scan on patients attending a specialized CF clinic. Results: Of 125 patients studied, 2 1 had musculoskeletal complications, 17 attributable to CF. Eleven had joint involvement (six hypertrophic pulmonary osteoarthropathy (HPOA) ), one CF arthropathy, two ciprofloxacin induced arthralgia, one joint contracture following long-line placement, one chest infection associ ated arthralgia), four kyphosis (two also with HPOA) and two thoracic deformity. HPOA was associated with older age, lower average pulmonary function and lower average Shwachman score. Three patients with HPOA died within 12 months of reporting symptoms. Kyphosis was also associa ted with older age and lower pulmonary function. Conclusion: Increasin g age with deteriorating clinical and pulmonary function were associat ed with a higher incidence of musculoskeletal involvement. The develop ment of symptomatic HPOA is a marker of poor prognosis.