Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis after newborn screening

Stephen Stick; Siobhan Brennan; C. Murray; T. Douglas; Britta Von Ungern-Sternberg; L.W. Garratt; Catherine Gangell; Nicholas De Klerk; B. Linnane; S. Ranganathan; P. Robinson; C. Robertson; Peter Sly

doi:10.1016/j.jpeds.2009.05.005

Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis after newborn screening

Stephen Stick, Siobhan Brennan, C. Murray, T. Douglas, Britta Von Ungern-Sternberg, L.W. Garratt, Catherine Gangell, Nicholas De Klerk, B. Linnane, S. Ranganathan, P. Robinson, C. Robertson, Peter Sly

UWA Centre for Child Health Research (affiliated with the Telethon Kids Institute)

Research output: Contribution to journal › Article › peer-review

291 Citations (Scopus)

Abstract

ObjectivesTo determine the prevalence of bronchiectasis in young children with cystic fibrosis (CF) diagnosed after newborn screening (NBS) and the relationship of bronchiectasis to pulmonary inflammation and infection.Study designChildren were diagnosed with CF after NBS. Computed tomography and bronchoalveolar lavage were performed with anesthesia (n = 96). Scans were analyzed for the presence and extent of abnormalities.ResultsThe prevalence of bronchiectasis was 22% and increased with age (P = .001). Factors associated with bronchiectasis included absolute neutrophil count (P = .03), neutrophil elastase concentration (P = .001), and Pseudomonas aeruginosa infection (P = .03).ConclusionsPulmonary abnormalities are common in infants and young children with CF and relate to neutrophilic inflammation and infection with P. aeruginosa. Current models of care for infants with CF fail to prevent respiratory sequelae. Bronchiectasis is a clinically relevant endpoint that could be used for intervention trials that commence soon after CF is diagnosed after NBS.

Original language	English
Pages (from-to)	623-628 e1
Journal	The Journal of Pediatrics
Volume	155
Issue number	5
DOIs	https://doi.org/10.1016/j.jpeds.2009.05.005
Publication status	Published - 2009

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Stick, S., Brennan, S., Murray, C., Douglas, T., Von Ungern-Sternberg, B., Garratt, L. W., Gangell, C., De Klerk, N., Linnane, B., Ranganathan, S., Robinson, P., Robertson, C., & Sly, P. (2009). Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis after newborn screening. The Journal of Pediatrics, 155(5), 623-628 e1. https://doi.org/10.1016/j.jpeds.2009.05.005

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title = "Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis after newborn screening",

abstract = "ObjectivesTo determine the prevalence of bronchiectasis in young children with cystic fibrosis (CF) diagnosed after newborn screening (NBS) and the relationship of bronchiectasis to pulmonary inflammation and infection.Study designChildren were diagnosed with CF after NBS. Computed tomography and bronchoalveolar lavage were performed with anesthesia (n = 96). Scans were analyzed for the presence and extent of abnormalities.ResultsThe prevalence of bronchiectasis was 22% and increased with age (P = .001). Factors associated with bronchiectasis included absolute neutrophil count (P = .03), neutrophil elastase concentration (P = .001), and Pseudomonas aeruginosa infection (P = .03).ConclusionsPulmonary abnormalities are common in infants and young children with CF and relate to neutrophilic inflammation and infection with P. aeruginosa. Current models of care for infants with CF fail to prevent respiratory sequelae. Bronchiectasis is a clinically relevant endpoint that could be used for intervention trials that commence soon after CF is diagnosed after NBS.",

author = "Stephen Stick and Siobhan Brennan and C. Murray and T. Douglas and {Von Ungern-Sternberg}, Britta and L.W. Garratt and Catherine Gangell and {De Klerk}, Nicholas and B. Linnane and S. Ranganathan and P. Robinson and C. Robertson and Peter Sly",

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doi = "10.1016/j.jpeds.2009.05.005",

language = "English",

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Stick, S, Brennan, S, Murray, C, Douglas, T, Von Ungern-Sternberg, B, Garratt, LW, Gangell, C, De Klerk, N, Linnane, B, Ranganathan, S, Robinson, P, Robertson, C & Sly, P 2009, 'Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis after newborn screening', The Journal of Pediatrics, vol. 155, no. 5, pp. 623-628 e1. https://doi.org/10.1016/j.jpeds.2009.05.005

TY - JOUR

T1 - Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis after newborn screening

AU - Stick, Stephen

AU - Brennan, Siobhan

AU - Murray, C.

AU - Douglas, T.

AU - Von Ungern-Sternberg, Britta

AU - Garratt, L.W.

AU - Gangell, Catherine

AU - De Klerk, Nicholas

AU - Linnane, B.

AU - Ranganathan, S.

AU - Robinson, P.

AU - Robertson, C.

AU - Sly, Peter

PY - 2009

Y1 - 2009

N2 - ObjectivesTo determine the prevalence of bronchiectasis in young children with cystic fibrosis (CF) diagnosed after newborn screening (NBS) and the relationship of bronchiectasis to pulmonary inflammation and infection.Study designChildren were diagnosed with CF after NBS. Computed tomography and bronchoalveolar lavage were performed with anesthesia (n = 96). Scans were analyzed for the presence and extent of abnormalities.ResultsThe prevalence of bronchiectasis was 22% and increased with age (P = .001). Factors associated with bronchiectasis included absolute neutrophil count (P = .03), neutrophil elastase concentration (P = .001), and Pseudomonas aeruginosa infection (P = .03).ConclusionsPulmonary abnormalities are common in infants and young children with CF and relate to neutrophilic inflammation and infection with P. aeruginosa. Current models of care for infants with CF fail to prevent respiratory sequelae. Bronchiectasis is a clinically relevant endpoint that could be used for intervention trials that commence soon after CF is diagnosed after NBS.

AB - ObjectivesTo determine the prevalence of bronchiectasis in young children with cystic fibrosis (CF) diagnosed after newborn screening (NBS) and the relationship of bronchiectasis to pulmonary inflammation and infection.Study designChildren were diagnosed with CF after NBS. Computed tomography and bronchoalveolar lavage were performed with anesthesia (n = 96). Scans were analyzed for the presence and extent of abnormalities.ResultsThe prevalence of bronchiectasis was 22% and increased with age (P = .001). Factors associated with bronchiectasis included absolute neutrophil count (P = .03), neutrophil elastase concentration (P = .001), and Pseudomonas aeruginosa infection (P = .03).ConclusionsPulmonary abnormalities are common in infants and young children with CF and relate to neutrophilic inflammation and infection with P. aeruginosa. Current models of care for infants with CF fail to prevent respiratory sequelae. Bronchiectasis is a clinically relevant endpoint that could be used for intervention trials that commence soon after CF is diagnosed after NBS.

U2 - 10.1016/j.jpeds.2009.05.005

DO - 10.1016/j.jpeds.2009.05.005

M3 - Article

SN - 0022-3476

VL - 155

SP - 623-628 e1

JO - The Journal of Pediatrics

JF - The Journal of Pediatrics

IS - 5

ER -

Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis after newborn screening

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