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Do pulmonary and extrapulmonary features differ among cystic fibrosis, primary ciliary dyskinesia, and healthy

Hilal Denizoglu Kulli1, Hulya Nilgun Gurses1, Melih Zeren2

  • 1Department of Cardiopulmonary Physiotherapy and Rehabilitation, Division of Physiotherapy and Rehabilitation, Faculty of Health Sciences, Bezmialem Vakif University, Istanbul, Turkey.

Pediatric Pulmonology
|September 3, 2020
PubMed

Insights

Children with primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) show similar pulmonary function impairments. However, PCD patients exhibit worse respiratory muscle strength, highlighting the need for targeted physiotherapy.

Area of Science:

  • Pediatric Pulmonology
  • Respiratory Medicine
  • Clinical Physiology

Background:

  • Primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) share similarities in impaired mucociliary clearance and symptoms.
  • Limited research exists on respiratory muscle strength in children with PCD.
  • This study investigates pulmonary and extrapulmonary characteristics in children with CF and PCD compared to healthy controls.

Purpose of the Study:

  • To compare pulmonary and extrapulmonary characteristics of children with CF and PCD.
  • To assess respiratory muscle strength in children with PCD and compare it to CF and healthy controls.
  • To identify unique clinical features for targeted physiotherapy.

Main Methods:

  • Evaluated pulmonary and extrapulmonary characteristics using spirometry, 6-minute walk test (6MWT), and maximal respiratory pressure measurements.
  • Assessed peripheral muscle strength via knee extensor strength test.
  • Included children with CF, PCD, and age-matched healthy controls.

Main Results:

  • Children with PCD and CF showed similar pulmonary function test (PFT) results, except for FEF25-75, which was lower in PCD.
  • Maximum expiratory pressure (MEP) was significantly lower in children with PCD compared to both CF and healthy controls.
  • 6MWT distance was reduced in children with CF compared to healthy controls; knee extensor strength varied among groups but lacked statistical significance.

Conclusions:

  • Children with CF and PCD exhibit impairments in pulmonary function, respiratory muscle strength, and functional capacity compared to healthy peers.
  • Distinct characteristics of PCD and CF necessitate individualized physiotherapy assessments and treatments.
  • Clinicians should prioritize evaluation of respiratory and peripheral muscle strength in children with PCD.
Abstract

Keywords:
cystic fibrosisfunctional capacitylung functionmuscle strengthprimary ciliary dyskinesiarespiratory muscle strength

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