Original Articles
Clinical outcome after early Pseudomonas aeruginosa infection in cystic fibrosis,☆☆

https://doi.org/10.1067/mpd.2001.112897Get rights and content

Abstract

Objective: To determine the clinical consequences of acquiring Pseudomonas aeruginosa infection during early childhood in children with cystic fibrosis (CF). Design: Prospective, observational cohort study of 56 children with CF identified by newborn screening during 1990-92. Each child underwent an annual bronchial lavage during the first 2 to 3 years of life. Clinical outcome was determined at 7 years of age. Results: P aeruginosa infection was diagnosed in 24 (43%) cohort subjects. Four children died before 7 years of age, all of whom had been infected with a multi-resistant, mucoid strain of P aeruginosa (P =.04). In survivors, P aeruginosa infection was associated with significantly increased morbidity as measured by lower National Institutes of Health scores, greater variability in lung function, increased time in the hospital, and higher rates of recombinant human deoxyribonuclease therapy (P <.01). In this young CF cohort, best forced expiratory volume in 1 second was an insensitive measure of increased morbidity. Conclusions: Acquisition of P aeruginosa was common by 7 years of age in this CF birth cohort and was associated with increased morbidity and mortality. An improved disease severity score would improve the evaluation and study of early CF lung disease. (J Pediatr 2001;138:699-704)

Section snippets

Subjects

The state of Victoria, Australia, has had a newborn screening program for CF since 1989. Diagnosis is confirmed by gene mutation analysis and a sweat chloride level ≥60 mmol/L. By excluding those with meconium ileus or a sibling with CF, this program identifies 93.8% of affected infants.10 Eligible infants were identified between January 1990 and December 1992 and prospectively enrolled from February to December 1992, either within the first 6 months of life (newborn cohort) or at 12 or 24

Subjects

Between 1990 and 1992, 56 of 66 (86%) children given a diagnosis of CF during the neonatal period were enrolled. The 10 non-participants were no different from study subjects in sex, genotype, or pancreatic status at diagnosis; and outcome measures were not significantly different for this group (data not shown).

Subject characteristics at the time of recruitment are shown in Table I.

. Subject characteristics at recruitment by infection group

Empty CellRecruited infants (n = 56)*Final study subjects (n = 53)

Discussion

This prospective cohort study of unselected infants with CF, identified by newborn screening, showed that 45% had acquired P aeruginosa before 7 years of age. Early acquisition was associated with increased mortality, and survivors had lower NIH scores, lower mean FEV1, and higher hospitalization rates.

Compared with older patients with CF, little is known about P aeruginosa in young children. In another study in infants identified by newborn screening more respiratory symptoms and

Acknowledgements

We thank the Department of Anaesthesia, Royal Children’s Hospital, Melbourne, for their assistance with BL.

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    Supported by grants from the Murdoch Children’s Research Institute and the JB Were Charitable Fund, Melbourne. G. Nixon received the Grand Lodge of New Zealand Freemasons Child Health Fellowship.

    ☆☆

    Reprint requests: Gillian Nixon, Department of Respiratory Medicine, Royal Children’s Hospital, Flemington Rd, Parkville, Victoria 3052, Australia.

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