Lung function in a group of Czech infants with cystic fibrosis

Česká verzia

Authors: V. Koucky; V. Skalická; J. Bartošová; T. Doušová; V. Vávrová; P. Pohunek
Authors place of work: Pediatrická klinika 2. LF UK a FN Motol, Praha
Published in the journal: Čes-slov Pediat 2019; 74 (7): 392-400.
Category:

Summary

Objective: To assess lung function in a group of Czech infants with cystic fibrosis (CF) and to evaluate safety of the infant pulmonary function testing (iPFT) in these patients.

Methods: Fifty-five infants (age range 5–118 weeks) with classic form of CF clinically indicated for iPFT in our lab were sedated with chloralhydrate and underwent multiple breath inert gas washout test, bodypletysmography, tidal breath analysis, single occlusion compliance-resistance measurement and tidal volume rapid thoracoabdominal compression. During iPFT and until full recovery from sedation, haemoglobin saturation, pulse rate, minute ventilation and clinical correlates of lung aspiration were monitored. Lung function was compared to the available international normative values and between clinically defined subgroups (based on patient’s age, microbial colonisation, occurrence of severe pulmonary exacerbations (PEx), history of meconium ileus and exocrine pancreatic function).

Results: Lung function impairment was detected in 63.9% of infants with the most frequent impairment being ventilation inhomogeneity (51.2% of all), followed by hyperinflation (48.6% of all) and peripheral airway obstruction (50.0% of all). Lung function was significantly diminished in older patients (mean age 16.6 vs 2.0 months), patients with at least one PEx, intermittent Pseudomonas aeruginosa and chronic Staphylococcus aureus colonization. Only 1 adverse event occurred – brief desaturation down to 85%.

Conclusion: Lung function impairment may be detected by iPFT as early as in 2 months-aged CF infants, its frequency increases with age. Lung function differs between clinically defined subgroups of patients. iPFT is safe.

Keywords:

Cystic fibrosis – Infants – toddler – multiple breath inert gas washout test – bodypletysmography – tidal breath analysis – single occlusion compliance-resistance measurement – tidal volume rapid thoracoabdominal compression – Safety

Zdroje

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