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Titre du document / Document title

Diagnosis and treatment of intestinal malabsorption in cystic fibrosis

Auteur(s) / Author(s)

LITTLEWOOD James M. (1) ; WOLFE Susan P. (1) ; CONWAY Steven P. (1) ;

Affiliation(s) du ou des auteurs / Author(s) Affiliation(s)

(1) Regional Paediatric Cystic Fibrosis Unit, St. James's University Hospital, Leeds, ROYAUME-UNI

Résumé / Abstract

Intestinal malabsorption is severe and of early onset in virtually all people who have cystic fibrosis. The main cause is deficiency of pancreatic enzymes. Bicarbonate deficiency, abnormal bile salts, mucosal transport problems, motility differences, and anatomical structural changes are other contributory factors. Effective treatment should allow a normal to high-fat diet to be taken, control symptoms, correct malabsorption, and achieve a normal nutritional state and growth. Appropriate pancreatic enzyme replacement therapy will achieve normal or near-normal absorption in most people with cystic fibrosis. Early identification and treatment of intestinal malabsorption is critical to achieving optimal nutritional status. The occurrence of fibrosing colonopathy in a few patients on very high doses of those enzymes which have the copolymer Eudragit L30 D55 in their covering resulted in guidelines in the UK to avoid doses equivalent to more than 10,000 IU lipase per kg per day, and also to avoid preparations containing this copolymer in children and adolescents. For patients not responding to 10,000 IU lipase per kg per day review of adherence to treatment, change of enzyme preparation, variation in time of administration, and reduction in gastric acid may improve absorption. The importance of early investigation to exclude other gastrointestinal disorders as a cause of the patient's symptoms, rather than merely increasing the dose of enzymes, is stressed. With modern pancreatic enzymes in doses up to or only slightly in excess of 10,000 IU lipase per kg per day, adequate control of gastrointestinal symptoms and absorption can be achieved, and a normal nutritional state and growth rate maintained in most people with cystic fibrosis.

Revue / Journal Title

Pediatric pulmonology    ISSN  8755-6863   CODEN PEPUES 

Source / Source

2006, vol. 41, no1, pp. 35-49 [15 page(s) (article)] (129 ref.)

Langue / Language

Anglais

Editeur / Publisher

Wiley-Liss, Hoboken, NJ, ETATS-UNIS  (1985) (Revue)

Mots-clés anglais / English Keywords

Metabolic diseases

;

Genetic disease

;

Intestinal disease

;

Digestive diseases

;

Pancreatic disease

;

Pediatrics

;

Nutrition

;

Feeding

;

Enzyme

;

Treatment

;

Diagnosis

;

Respiratory disease

;

Cystic fibrosis

;

Intestinal malabsorption

;

Mots-clés français / French Keywords

Métabolisme pathologie

;

Maladie héréditaire

;

Intestin pathologie

;

Appareil digestif pathologie

;

Pancréas pathologie

;

Pédiatrie

;

Nutrition

;

Alimentation

;

Enzyme

;

Traitement

;

Diagnostic

;

Appareil respiratoire pathologie

;

Mucoviscidose

;

Malabsorption intestinale

;

Mots-clés espagnols / Spanish Keywords

Metabolismo patología

;

Enfermedad hereditaria

;

Intestino patología

;

Aparato digestivo patología

;

Páncreas patología

;

Pediatría

;

Nutrición

;

Alimentación

;

Enzima

;

Tratamiento

;

Diagnóstico

;

Aparato respiratorio patología

;

Mucoviscidosis

;

Malabsorción intestinal

;

Mots-clés d'auteur / Author Keywords

pancreatic enzymes

;

cystic fibrosis

;

malabsorption

;

nutrition

;

Localisation / Location

INIST-CNRS, Cote INIST : 21407, 35400011518728.0030

Nº notice refdoc (ud4) : 17578770



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