Management of infants with cystic fibrosis: A summary of the evidence for the cystic fibrosis foundation working group on care of infants with cystic fibrosis

Karen A Robinson, Ian J. Saldanha, Naomi A. McKoy

Research output: Contribution to journalArticle

Abstract

Objective To inform the development of Cystic Fibrosis (CF) Foundation guidelines on the care of infants with CF, we systematically reviewed the evidence for diagnosis and assessment of pancreatic and pulmonary disorders; management of pancreatic and pulmonary function; management of nutrition and nutritional disorders; and prevention and control of infections. Study design In May-June 2008, we searched The Cochrane Library for existing reviews; and MEDLINE, the National Guideline Clearinghouse, the CF Foundation Clinical Practice Guidelines and Consensus Statements, and the UK CF Trust for existing guidelines. MEDLINE, Cumulative Index to Nursing and Allied Health Literature (CINAHL) and the Excerpta Medica Database (EMBASE) were searched for primary studies in January 2008. Bibliographies of eligible articles were searched and expert input was sought. We selected English-language articles of any study design that provided original data on any of our questions on infants up to 2 years of age. Results We identified 14 relevant guidelines and 3 Cochrane reviews. Fifty-nine articles (55 primary studies) were included. Only four of these were randomized controlled trials. Sample sizes of infants ranged from 2 to 768 study participants; the median sample size was 24. Of our 21 review topics, 5 topics had only one study while for 5 we identified no relevant studies. We identified one or no primary studies for 20 of 32 review questions. Conclusions There is a paucity of evidence on the care of infants diagnosed with CF. For several of the review questions no guidelines or primary studies were identified, but for other questions, studies limited by weak design and small sample sizes were the only studies identified.With increasing numbers of infants with CF being diagnosed by newborn screening there is an opportunity to study the management of infants diagnosed with CF.

Original languageEnglish (US)
JournalJournal of Pediatrics
Volume155
Issue number6 SUPPL.
DOIs
StatePublished - Dec 2009

Fingerprint

Infant Care
Cystic Fibrosis
Guidelines
Nutrition Disorders
Sample Size
MEDLINE
Lung
Bibliography
Infection Control
Practice Guidelines
Libraries
Consensus
Nursing
Language
Randomized Controlled Trials
Newborn Infant
Databases
Health

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Medicine(all)

Cite this

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title = "Management of infants with cystic fibrosis: A summary of the evidence for the cystic fibrosis foundation working group on care of infants with cystic fibrosis",
abstract = "Objective To inform the development of Cystic Fibrosis (CF) Foundation guidelines on the care of infants with CF, we systematically reviewed the evidence for diagnosis and assessment of pancreatic and pulmonary disorders; management of pancreatic and pulmonary function; management of nutrition and nutritional disorders; and prevention and control of infections. Study design In May-June 2008, we searched The Cochrane Library for existing reviews; and MEDLINE, the National Guideline Clearinghouse, the CF Foundation Clinical Practice Guidelines and Consensus Statements, and the UK CF Trust for existing guidelines. MEDLINE, Cumulative Index to Nursing and Allied Health Literature (CINAHL) and the Excerpta Medica Database (EMBASE) were searched for primary studies in January 2008. Bibliographies of eligible articles were searched and expert input was sought. We selected English-language articles of any study design that provided original data on any of our questions on infants up to 2 years of age. Results We identified 14 relevant guidelines and 3 Cochrane reviews. Fifty-nine articles (55 primary studies) were included. Only four of these were randomized controlled trials. Sample sizes of infants ranged from 2 to 768 study participants; the median sample size was 24. Of our 21 review topics, 5 topics had only one study while for 5 we identified no relevant studies. We identified one or no primary studies for 20 of 32 review questions. Conclusions There is a paucity of evidence on the care of infants diagnosed with CF. For several of the review questions no guidelines or primary studies were identified, but for other questions, studies limited by weak design and small sample sizes were the only studies identified.With increasing numbers of infants with CF being diagnosed by newborn screening there is an opportunity to study the management of infants diagnosed with CF.",
author = "Robinson, {Karen A} and Saldanha, {Ian J.} and McKoy, {Naomi A.}",
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AU - Robinson, Karen A

AU - Saldanha, Ian J.

AU - McKoy, Naomi A.

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N2 - Objective To inform the development of Cystic Fibrosis (CF) Foundation guidelines on the care of infants with CF, we systematically reviewed the evidence for diagnosis and assessment of pancreatic and pulmonary disorders; management of pancreatic and pulmonary function; management of nutrition and nutritional disorders; and prevention and control of infections. Study design In May-June 2008, we searched The Cochrane Library for existing reviews; and MEDLINE, the National Guideline Clearinghouse, the CF Foundation Clinical Practice Guidelines and Consensus Statements, and the UK CF Trust for existing guidelines. MEDLINE, Cumulative Index to Nursing and Allied Health Literature (CINAHL) and the Excerpta Medica Database (EMBASE) were searched for primary studies in January 2008. Bibliographies of eligible articles were searched and expert input was sought. We selected English-language articles of any study design that provided original data on any of our questions on infants up to 2 years of age. Results We identified 14 relevant guidelines and 3 Cochrane reviews. Fifty-nine articles (55 primary studies) were included. Only four of these were randomized controlled trials. Sample sizes of infants ranged from 2 to 768 study participants; the median sample size was 24. Of our 21 review topics, 5 topics had only one study while for 5 we identified no relevant studies. We identified one or no primary studies for 20 of 32 review questions. Conclusions There is a paucity of evidence on the care of infants diagnosed with CF. For several of the review questions no guidelines or primary studies were identified, but for other questions, studies limited by weak design and small sample sizes were the only studies identified.With increasing numbers of infants with CF being diagnosed by newborn screening there is an opportunity to study the management of infants diagnosed with CF.

AB - Objective To inform the development of Cystic Fibrosis (CF) Foundation guidelines on the care of infants with CF, we systematically reviewed the evidence for diagnosis and assessment of pancreatic and pulmonary disorders; management of pancreatic and pulmonary function; management of nutrition and nutritional disorders; and prevention and control of infections. Study design In May-June 2008, we searched The Cochrane Library for existing reviews; and MEDLINE, the National Guideline Clearinghouse, the CF Foundation Clinical Practice Guidelines and Consensus Statements, and the UK CF Trust for existing guidelines. MEDLINE, Cumulative Index to Nursing and Allied Health Literature (CINAHL) and the Excerpta Medica Database (EMBASE) were searched for primary studies in January 2008. Bibliographies of eligible articles were searched and expert input was sought. We selected English-language articles of any study design that provided original data on any of our questions on infants up to 2 years of age. Results We identified 14 relevant guidelines and 3 Cochrane reviews. Fifty-nine articles (55 primary studies) were included. Only four of these were randomized controlled trials. Sample sizes of infants ranged from 2 to 768 study participants; the median sample size was 24. Of our 21 review topics, 5 topics had only one study while for 5 we identified no relevant studies. We identified one or no primary studies for 20 of 32 review questions. Conclusions There is a paucity of evidence on the care of infants diagnosed with CF. For several of the review questions no guidelines or primary studies were identified, but for other questions, studies limited by weak design and small sample sizes were the only studies identified.With increasing numbers of infants with CF being diagnosed by newborn screening there is an opportunity to study the management of infants diagnosed with CF.

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