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Updated: Aug 27, 2026

The WinCF Model - An Inexpensive and Tractable Microcosm of a Mucus Plugged Bronchiole to Study the Microbiology of Lung Infections
Published on: May 8, 2017
[Digestive diseases and nutrition in cystic fibrosis]
Dominique Turck1, Laurent Michaud, Nathalie Wizla-Derambure
1Centre de soins pour la mucoviscidose, Unité de gastro-entérologie, hépatologie et nutrition Clinique de pédiatrie Hôpital Jeanne-de-Flandre, Faculté de médecine 59037 Lille. dturck@chru-lille.fr
Abstract:
Present in 85-90% of patients, pancreatic exocrine insufficiency contributes to growth retardation and delayed puberty in the child, and low weight in the adult. The incidence of hepatic disease varies according to whether one considers the discovery of histological abnormalities at autopsy of patients who have died from other complications (20-70%) or the presence of a focal or multilobular biliary cirrhosis complicated or not by portal hypertension (2-10%). Gastro-oesophageal reflux can contribute to the degradation of the nutritional state and exacerbate the respiratory symptomatology. All deviations of anthropometric parameters (weight/height ratio, body mass index) from standard references are a warning sign and justify nutritional assistance, of which one distinguishes 3 successive stages by chronological order: fractionated oral supplementation, nasogastric enteral nutrition and parenteral nutrition.
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