Literature DB >> 32304511

Outcomes of Children With Cystic Fibrosis Admitted to PICUs.

Michael A Smith1, Meghan E McGarry2, Ngoc P Ly2, Matt S Zinter3.   

Abstract

OBJECTIVES: Data on outcomes of children with cystic fibrosis admitted to PICUs are limited and outdated. Prior studies cite PICU mortality rates ranging from 37.5% to 100%. Given the advances made in cystic fibrosis care, we expect outcomes for these patients to have changed significantly since last studied. We provide an updated report on PICU mortality and the factors associated with death among critically ill children with cystic fibrosis.
DESIGN: Retrospective multicenter cohort analysis utilizing data from the Virtual Pediatric Systems database.
SETTING: Data were collected from 135 PICUs from January 1, 2009, to June 20, 2018. PATIENTS: One-thousand six-hundred thirty-three children with cystic fibrosis accounting for 2,893 PICU admissions were studied.
INTERVENTIONS: None.
MEASUREMENTS AND MAIN RESULTS: The primary outcome was mortality during PICU admission. Predictors included demographics, anthropometrics, diagnoses, clinical characteristics, and critical care interventions. Odds ratios of mortality were calculated in univariate and multivariable analyses to assess differences in mortality associated with predictor variables. Generalized estimating equation models were used to account for multiple admissions per patient. The overall PICU mortality rate was 6.6%. Factors associated with increased odds of mortality included hemoptysis/pulmonary hemorrhage, pneumothorax, gastrointestinal bleeding, bacterial/fungal infections, lower body mass index/malnutrition, and need for noninvasive or invasive respiratory support. Intubation/mechanical ventilation occurred in 26.4% of the 2,893 admissions and was associated with a 19.1% mortality rate. Of the nonsurvivors, 20.7% died without receiving mechanical ventilation.
CONCLUSIONS: The mortality rate during PICU admissions for patients with cystic fibrosis is lower than has been reported in prior studies, both in the overall cohort and in the subset requiring invasive mechanical ventilation. These data provide updated insight into the prognosis for cystic fibrosis patients requiring critical care.

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Year:  2020        PMID: 32304511      PMCID: PMC7597545          DOI: 10.1097/PCC.0000000000002358

Source DB:  PubMed          Journal:  Pediatr Crit Care Med        ISSN: 1529-7535            Impact factor:   3.971


  26 in total

1.  Patients with cystic fibrosis should not be intubated and ventilated.

Authors:  Mark Rosenthal
Journal:  J R Soc Med       Date:  2010-07       Impact factor: 5.344

2.  Patients with cystic fibrosis should be intubated and ventilated.

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3.  Nutritional Status in Childhood as a Prognostic Factor in Patients with Cystic Fibrosis.

Authors:  Moshe Ashkenazi; N Nathan; I Sarouk; B E Bar Aluma; A Dagan; Y Bezalel; S Keler; D Vilozni; O Efrati
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4.  One-year outcome after severe pulmonary exacerbation in adults with cystic fibrosis.

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5.  PRISM III: an updated Pediatric Risk of Mortality score.

Authors:  M M Pollack; K M Patel; U E Ruttimann
Journal:  Crit Care Med       Date:  1996-05       Impact factor: 7.598

6.  Predictors of outcome in patients with cystic fibrosis requiring endotracheal intubation.

Authors:  Andrew Jones; Diana Bilton; Timothy W Evans; Simon J Finney
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Review 7.  Intensive care management of the patient with cystic fibrosis.

Authors:  Ted M Kremer; Robert G Zwerdling; Peter H Michelson; P O'Sullivan
Journal:  J Intensive Care Med       Date:  2008-04-28       Impact factor: 3.510

8.  Improved outcomes of patients with cystic fibrosis admitted to the intensive care unit.

Authors:  Hima Vedam; Carmel Moriarty; Paul J Torzillo; David McWilliam; Peter T P Bye
Journal:  J Cyst Fibros       Date:  2004-03       Impact factor: 5.482

9.  The Cystic Fibrosis Foundation Patient Registry. Design and Methods of a National Observational Disease Registry.

Authors:  Emily A Knapp; Aliza K Fink; Christopher H Goss; Ase Sewall; Josh Ostrenga; Christopher Dowd; Alexander Elbert; Kristofer M Petren; Bruce C Marshall
Journal:  Ann Am Thorac Soc       Date:  2016-07

10.  Determinants of mortality for adults with cystic fibrosis admitted in Intensive Care Unit: a multicenter study.

Authors:  Joëlle Texereau; Dany Jamal; Gérald Choukroun; Pierre-Régis Burgel; Jean-Luc Diehl; Antoine Rabbat; Philippe Loirat; Antoine Parrot; Alexandre Duguet; Joël Coste; Daniel Dusser; Dominique Hubert; Jean-Paul Mira
Journal:  Respir Res       Date:  2006-01-26
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