Longitudinal development of initial, chronic and mucoid Pseudomonas aeruginosa infection in young children with cystic fibrosis

S. L. Heltshe, U. Khan, V. Beckett, A. Baines, J. Emerson, D. B. Sanders, R. L. Gibson, Wayne J Morgan, M. Rosenfeld

Research output: Contribution to journalArticle

11 Citations (Scopus)

Abstract

Background: While the emergence of chronic and mucoid Pseudomonas aeruginosa (Pa) infection are both associated with poorer outcomes among CF patients, their relationship is poorly understood. We examined the longitudinal relationship of incident, chronic and mucoid Pa in a contemporary, young CF cohort in the current era of Pa eradication therapy. Methods: This retrospective cohort was comprised of patients in the U.S. CF Foundation Patient Registry born 2006-2015, diagnosed before age 2, and with at least 3 respiratory cultures annually. Incidence and age-specific prevalence of Pa infection stages (initial and chronic [≥ 3. Pa +. cultures in prior year]) and of mucoid Pa were summarized. Transition times and the interaction between Pa stage and acquisition of mucoid Pa were examined via Cox models. Results: Among the 5592 CF patients in the cohort followed to a mean age of 5.5. years, 64% (n = 3580) acquired Pa. Of those, 13% (n = 455) developed chronic Pa and 17% (n = 594) cultured mucoid Pa. Among those with mucoid Pa, 36% (211/594) had it on their first recorded Pa +. culture, while mucoid Pa emerged at or after entering the chronic stage in 12% (73/594). Mucoidy was associated with significantly increased risk of transition to chronic Pa infection (HR = 2.59, 95% CI 2.11, 3.19). Conclusions: Two-thirds of early-diagnosed young children with CF acquired Pa during a median 5.6. years of follow up, among whom 13% developed chronic Pa and 17% acquired mucoid Pa. Contrary to our hypothesis, 87% of young children who developed mucoid Pa did so before becoming chronically infected.

Original languageEnglish (US)
JournalJournal of Cystic Fibrosis
DOIs
StateAccepted/In press - 2017

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Pseudomonas Infections
Cystic Fibrosis
Pseudomonas aeruginosa

Keywords

  • Cystic fibrosis
  • Epidemiology
  • Mucoidy
  • Pediatric
  • Pseudomonas aeruginosa

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pulmonary and Respiratory Medicine

Cite this

Longitudinal development of initial, chronic and mucoid Pseudomonas aeruginosa infection in young children with cystic fibrosis. / Heltshe, S. L.; Khan, U.; Beckett, V.; Baines, A.; Emerson, J.; Sanders, D. B.; Gibson, R. L.; Morgan, Wayne J; Rosenfeld, M.

In: Journal of Cystic Fibrosis, 2017.

Research output: Contribution to journalArticle

Heltshe, S. L. ; Khan, U. ; Beckett, V. ; Baines, A. ; Emerson, J. ; Sanders, D. B. ; Gibson, R. L. ; Morgan, Wayne J ; Rosenfeld, M. / Longitudinal development of initial, chronic and mucoid Pseudomonas aeruginosa infection in young children with cystic fibrosis. In: Journal of Cystic Fibrosis. 2017.
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title = "Longitudinal development of initial, chronic and mucoid Pseudomonas aeruginosa infection in young children with cystic fibrosis",
abstract = "Background: While the emergence of chronic and mucoid Pseudomonas aeruginosa (Pa) infection are both associated with poorer outcomes among CF patients, their relationship is poorly understood. We examined the longitudinal relationship of incident, chronic and mucoid Pa in a contemporary, young CF cohort in the current era of Pa eradication therapy. Methods: This retrospective cohort was comprised of patients in the U.S. CF Foundation Patient Registry born 2006-2015, diagnosed before age 2, and with at least 3 respiratory cultures annually. Incidence and age-specific prevalence of Pa infection stages (initial and chronic [≥ 3. Pa +. cultures in prior year]) and of mucoid Pa were summarized. Transition times and the interaction between Pa stage and acquisition of mucoid Pa were examined via Cox models. Results: Among the 5592 CF patients in the cohort followed to a mean age of 5.5. years, 64{\%} (n = 3580) acquired Pa. Of those, 13{\%} (n = 455) developed chronic Pa and 17{\%} (n = 594) cultured mucoid Pa. Among those with mucoid Pa, 36{\%} (211/594) had it on their first recorded Pa +. culture, while mucoid Pa emerged at or after entering the chronic stage in 12{\%} (73/594). Mucoidy was associated with significantly increased risk of transition to chronic Pa infection (HR = 2.59, 95{\%} CI 2.11, 3.19). Conclusions: Two-thirds of early-diagnosed young children with CF acquired Pa during a median 5.6. years of follow up, among whom 13{\%} developed chronic Pa and 17{\%} acquired mucoid Pa. Contrary to our hypothesis, 87{\%} of young children who developed mucoid Pa did so before becoming chronically infected.",
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T1 - Longitudinal development of initial, chronic and mucoid Pseudomonas aeruginosa infection in young children with cystic fibrosis

AU - Heltshe, S. L.

AU - Khan, U.

AU - Beckett, V.

AU - Baines, A.

AU - Emerson, J.

AU - Sanders, D. B.

AU - Gibson, R. L.

AU - Morgan, Wayne J

AU - Rosenfeld, M.

PY - 2017

Y1 - 2017

N2 - Background: While the emergence of chronic and mucoid Pseudomonas aeruginosa (Pa) infection are both associated with poorer outcomes among CF patients, their relationship is poorly understood. We examined the longitudinal relationship of incident, chronic and mucoid Pa in a contemporary, young CF cohort in the current era of Pa eradication therapy. Methods: This retrospective cohort was comprised of patients in the U.S. CF Foundation Patient Registry born 2006-2015, diagnosed before age 2, and with at least 3 respiratory cultures annually. Incidence and age-specific prevalence of Pa infection stages (initial and chronic [≥ 3. Pa +. cultures in prior year]) and of mucoid Pa were summarized. Transition times and the interaction between Pa stage and acquisition of mucoid Pa were examined via Cox models. Results: Among the 5592 CF patients in the cohort followed to a mean age of 5.5. years, 64% (n = 3580) acquired Pa. Of those, 13% (n = 455) developed chronic Pa and 17% (n = 594) cultured mucoid Pa. Among those with mucoid Pa, 36% (211/594) had it on their first recorded Pa +. culture, while mucoid Pa emerged at or after entering the chronic stage in 12% (73/594). Mucoidy was associated with significantly increased risk of transition to chronic Pa infection (HR = 2.59, 95% CI 2.11, 3.19). Conclusions: Two-thirds of early-diagnosed young children with CF acquired Pa during a median 5.6. years of follow up, among whom 13% developed chronic Pa and 17% acquired mucoid Pa. Contrary to our hypothesis, 87% of young children who developed mucoid Pa did so before becoming chronically infected.

AB - Background: While the emergence of chronic and mucoid Pseudomonas aeruginosa (Pa) infection are both associated with poorer outcomes among CF patients, their relationship is poorly understood. We examined the longitudinal relationship of incident, chronic and mucoid Pa in a contemporary, young CF cohort in the current era of Pa eradication therapy. Methods: This retrospective cohort was comprised of patients in the U.S. CF Foundation Patient Registry born 2006-2015, diagnosed before age 2, and with at least 3 respiratory cultures annually. Incidence and age-specific prevalence of Pa infection stages (initial and chronic [≥ 3. Pa +. cultures in prior year]) and of mucoid Pa were summarized. Transition times and the interaction between Pa stage and acquisition of mucoid Pa were examined via Cox models. Results: Among the 5592 CF patients in the cohort followed to a mean age of 5.5. years, 64% (n = 3580) acquired Pa. Of those, 13% (n = 455) developed chronic Pa and 17% (n = 594) cultured mucoid Pa. Among those with mucoid Pa, 36% (211/594) had it on their first recorded Pa +. culture, while mucoid Pa emerged at or after entering the chronic stage in 12% (73/594). Mucoidy was associated with significantly increased risk of transition to chronic Pa infection (HR = 2.59, 95% CI 2.11, 3.19). Conclusions: Two-thirds of early-diagnosed young children with CF acquired Pa during a median 5.6. years of follow up, among whom 13% developed chronic Pa and 17% acquired mucoid Pa. Contrary to our hypothesis, 87% of young children who developed mucoid Pa did so before becoming chronically infected.

KW - Cystic fibrosis

KW - Epidemiology

KW - Mucoidy

KW - Pediatric

KW - Pseudomonas aeruginosa

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