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Malnutrition in adults with cystic fibrosis

X Dray1, R Kanaan, T Bienvenu

  • 1Service d'Hépato-Gastro-Entérologie, Hôpital Européen Georges Pompidou, Paris, France.

European Journal of Clinical Nutrition
|September 16, 2004
PubMed

Insights

Malnutrition affects nearly half of adult cystic fibrosis (CF) patients, particularly those with severe CFTR genotypes. Early diagnosis and milder CFTR genotypes are linked to better nutritional status.

Area of Science:

  • Pulmonology
  • Genetics
  • Nutritional Science

Background:

  • Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs.
  • Malnutrition is a common complication in CF patients.
  • The CFTR genotype is known to influence CF phenotype severity.

Purpose of the Study:

  • To investigate the prevalence of malnutrition in adult CF patients.
  • To identify clinical features associated with malnutrition in CF.
  • To explore the relationship between CFTR genotype and malnutrition in CF.

Main Methods:

  • Cross-sectional study design.
  • Involved 163 adult CF patients from a French hospital (1997-1999).
  • Assessed malnutrition using Body Mass Index (BMI <18.5 kg/m2) and correlated findings with CFTR genotype and clinical parameters.

Main Results:

  • Nearly half (49.7%) of adult CF patients exhibited malnutrition (BMI <18.5 kg/m2).
  • Malnutrition severity correlated with early CF diagnosis, reduced FEV1, FEV1 decline, pancreatic insufficiency, and gastro-oesophageal reflux.
  • Patients with severe CFTR genotypes had a higher prevalence of malnutrition (58.7%) compared to those with mild genotypes (28.6%).

Conclusions:

  • Malnutrition remains a significant issue in adult CF populations.
  • Milder CFTR genotypes, associated with milder CF phenotypes and later diagnosis, are linked to a lower prevalence of malnutrition.
  • Understanding these relationships can inform targeted nutritional interventions in CF care.
Abstract

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