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Respiratory rate in infants with cystic fibrosis throughout the first year of life and association with lung clearance index measured shortly after birth
Authors:Insa Korten  Elisabeth Kieninger  Sophie Yammine  Giulia Cangiano  Sylvia Nyilas  Pinelopi Anagnostopoulou  Florian Singer  Claudia E. Kuehni  Nicolas Regamey  Urs Frey  Carmen Casaulta  Ben D. Spycher  Philipp Latzin
Affiliation:1. Paediatric Respiratory Medicine, Inselspital, University Children''s Hospital of Bern, University of Bern, Switzerland;2. Graduate School for Cellular and Biomedical Sciences, University of Bern, Switzerland;3. University Children''s Hospital (UKBB), Basel, Switzerland;4. Paediatric Department, Sapienza University of Rome, Italy;5. Institute for Social and Preventive Medicine, University of Bern, Switzerland;6. Division of Respiratory Medicine, Children''s Hospital Lucerne, Switzerland
Abstract:

Background

Lung impairment in cystic fibrosis (CF) starts in infancy. However, tools to monitor early lung disease are limited. Respiratory rate (RR) as a key vital sign is easy to assess during sleep and is elevated during acute respiratory disease. Thus, elevated RR could indicate early lung impairment and potentially serve as a diagnostic tool in disease monitoring.

Methods

In a prospective cohort of infants with CF diagnosed by newborn screening and healthy controls RR was measured and respiratory symptoms reported weekly throughout infancy. Infants performed a lung function measurement within the first weeks of life.

Results

The analyses included 5656 measurements from 153 infants (43 with CF). RR declined from 43.2 (40.5)/min at 6?weeks of age to 28.3 (24.6)/min at 50?weeks in infants with CF (healthy controls). Infants with CF had consistently higher RR than controls (mean difference: 4.15/min; (95% CI 2.86–5.44); p?

Conclusions

Infants with CF have a higher RR compared to healthy controls during the first year of life. The association with early LCI measurements, the current gold standard to assess physiology of peripheral airways persisted throughout the study period. This may indicate tracking of lung function by RR. It might thus be an early subtle sign of functional respiratory deficit. Further studies will show if RR can be used as a sensitive and promising marker to monitor early CF lung disease.
Keywords:BILD  Basel-Bern-Infant-Lung-Development cohort  CF  cystic fibrosis  Coeff  Coefficient  CV  coefficient of variation  CFTR  Cystic Fibrosis Transmembrane Regulator  FRC  functional residual capacity  LCI  lung clearance index  LRTI  lower respiratory-tract infections  MBW  multiple breath washout  NBS  newborn screening  RR  respiratory rate  SCILD  Swiss-Cystic-Fibrosis-Infant-Lung-Development cohort  SD  standard deviation  Respiratory rate  Infant lung function  Lung clearance index  Cystic fibrosis  Infancy
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