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Clinical features and management of children with primary ciliary dyskinesia in England

Bruna Rubbo1,2, Sunayna Best3, Robert Anthony Hirst4

  • 1School of Clinical and Experimental Medicine, Faculty of Medicine, University of Southampton, Southampton, UK.

Archives of Disease in Childhood
|March 12, 2020
PubMed

Insights

Children with primary ciliary dyskinesia (PCD) in England show poorer lung function compared to cystic fibrosis (CF) patients. Lower BMI in PCD children correlates with reduced lung function, highlighting the importance of nutritional status in management.

Area of Science:

  • Pediatric Pulmonology
  • Rare Diseases
  • Genetics

Background:

  • Primary ciliary dyskinesia (PCD) is a rare genetic disorder affecting cilia function.
  • The National Health Service in England established a specialized service for children with PCD.
  • Understanding the health status and lung function of children with PCD is crucial for effective management.

Purpose of the Study:

  • To describe the health status of children managed by the English National Management PCD Service.
  • To compare the lung function of children with PCD to those with cystic fibrosis (CF).
  • To identify factors influencing lung function in children with PCD.

Main Methods:

  • Multi-center service evaluation of the National Management PCD Service in England.
  • Inclusion of 333 children with PCD reviewed in 2015.
  • Comparison of lung function data with 2970 children with CF from the CF Registry.

Main Results:

  • Children with PCD had significantly lower mean %predicted forced expiratory volume in one second (FEV1) (76.8%) compared to CF (85.0%).
  • Lower FEV1 was observed in PCD patients up to age 15 and correlated with lower body mass index (BMI).
  • Hearing impairment was common (approx. 50%), and 33% had positive respiratory cultures, frequently *Haemophilus influenzae*.

Conclusions:

  • Children with PCD in England exhibit worse lung function than those with CF.
  • Nutritional status, indicated by BMI, is a significant factor affecting lung function in PCD.
  • Further research, including randomized controlled trials, is needed to optimize PCD management strategies.
Abstract

Keywords:
cystic Fibrosislung functionmanagementprimary ciliary dyskinesia

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