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The musculoskeletal complications of cystic fibrosis

R J Massie1, S J Towns, E Bernard

  • 1Department of Respiratory Medicine, New Children's Hospital, Westmead, New South Wales, Australia.

Journal of Paediatrics and Child Health
|October 10, 1998
PubMed

Insights

Musculoskeletal complications like hypertrophic pulmonary osteoarthropathy (HPOA) affect children with cystic fibrosis (CF). These issues, along with kyphosis, are linked to poorer health outcomes and lung function in CF patients.

Area of Science:

  • Paediatric Rheumatology
  • Respiratory Medicine
  • Medical Imaging

Background:

  • Cystic Fibrosis (CF) is a genetic disorder affecting multiple organ systems.
  • Musculoskeletal complications are recognized but not fully characterized in paediatric CF populations.
  • Early identification and understanding of these complications are crucial for patient management.

Purpose of the Study:

  • To investigate the range and prevalence of musculoskeletal issues in Australian children with CF.
  • To identify factors associated with the development of these complications.
  • To assess the prognostic significance of specific musculoskeletal findings.

Main Methods:

  • A cohort of 125 paediatric patients with CF attending a specialized clinic were assessed.
  • Clinical examinations were supplemented with serological tests and bone scans.
  • Data on pulmonary function and clinical scores were collected.

Main Results:

  • 21 out of 125 patients exhibited musculoskeletal complications, with 17 linked to CF.
  • Common complications included hypertrophic pulmonary osteoarthropathy (HPOA) (n=6), kyphosis (n=4), and thoracic deformity (n=2).
  • HPOA and kyphosis correlated with older age, reduced pulmonary function, and lower Shwachman scores. Three HPOA patients died within a year.

Conclusions:

  • Musculoskeletal involvement in paediatric CF increases with age and declining clinical/pulmonary status.
  • Symptomatic HPOA in CF patients indicates a poor prognosis.
  • These findings highlight the importance of monitoring skeletal health in children with CF.
Abstract

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