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Practical approach to the gastrointestinal manifestations of cystic fibrosis

Rishi Bolia1, Chee Y Ooi2,3, Peter Lewindon4

  • 1Department of Gastroenterology and Clinical Nutrition, Royal Children's Hospital, Melbourne, Victoria, Australia.

Journal of Paediatrics and Child Health
|May 17, 2018
PubMed

Insights

Cystic fibrosis (CF) is a common genetic disorder affecting multiple organs. This review offers evidence-based guidance for managing CF

Area of Science:

  • Medical Genetics
  • Pediatric Gastroenterology
  • Pulmonology

Background:

  • Cystic fibrosis (CF) is the most prevalent life-limiting genetic disorder in Australian and New Zealand children.
  • The genetic defect in CF leads to aberrant anion transport in epithelial cells, impacting organs like the lungs, GI tract, liver, and genitourinary system.
  • While respiratory complications dominate morbidity and mortality, significant gastrointestinal, hepatic, pancreatic, and nutritional issues require proactive management.

Purpose of the Study:

  • To furnish evidence-based information and clinical algorithms for the nutritional and gastrointestinal management of cystic fibrosis patients.
  • To support timely and structured detection and management of CF-related multi-systemic manifestations.

Main Methods:

  • This review synthesizes current evidence on the gastrointestinal and nutritional aspects of cystic fibrosis.
  • Clinical algorithms are developed based on established guidelines and expert consensus.

Main Results:

  • Key gastrointestinal and nutritional challenges in CF patients are identified.
  • Practical, evidence-based strategies for managing these manifestations are presented.

Conclusions:

  • Comprehensive, multi-disciplinary management is crucial for addressing the multi-systemic nature of cystic fibrosis.
  • Structured nutritional and gastrointestinal care is essential for improving outcomes in CF patients.

Keywords:
abdominal paincystic fibrosis-related liver diseasegastro-oesophageal refluxnutrition

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