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Rev Mal Respir. 2015 Jun;32(6):566-85. doi: 10.1016/j.rmr.2014.12.008. Epub 2015 May 19.

Management of pancreatic, gastrointestinal and liver complications in adult cystic fibrosis.

Author information

  • 1Assistance publique-Hôpitaux de Paris, Hôpital Robert-Debré, Service des maladies digestives et respiratoires de l'enfant, Centre de ressources et de compétence de la mucoviscidose, Université Paris 7, 75019 Paris, France. Electronic address:
  • 2Département de pédiatrie médicale, Centre de ressources et de compétence de la mucoviscidose, CHU de Limoges, 87000 Limoges, France.
  • 3Service de chirurgie viscérale pédiatrique - Hépatologie pédiatrique, AP-HP, CHU Necker-Enfants-Malades, 75015 Paris, France.
  • 4Gastro-entérologie, Hépatologie et Nutrition Pédiatriques, Hôpital des Enfants, 33077 Bordeaux, France.
  • 5CRCM Reims, American Memorial Hospital, 51092 Reims, France.
  • 6Service de Pédiatrie 1, Hôpital d'Enfants, CHU de Dijon, 21079 Dijon, France.
  • 7Centre de référence des affections congénitales et malformatives de l'œsophage, Pôle Enfant, Hôpital Jeanne-de-Flandre, Faculté de médecine, Université de Lille 2, 59000 Lille, France.
  • 8Unité de gastro-entérologie, hépatologie, nutrition, diabétologie et maladies héréditaires du métabolisme pédiatriques, Hôpital des Enfants, 330, avenue de Grande-Bretagne - TSA 70034, 31059 Toulouse cedex 9, France; Inserm U1043, CNRS, U5282, Université de Toulouse, UPS, Centre de Physiopathologie de Toulouse Purpan (CPTP), 31300 Toulouse, France.



The gastrointestinal tract is a major source of morbidity in adults with cystic fibrosis (CF), with a wide range of complications, some of them being specific to the underlying disease.


Abnormal CFTR function, with reduced bicarbonate and other ion transport levels through the apical surface of epithelial cells, affects the intestinal tract including the pancreas and the liver. Similarly to what is observed in the respiratory tract, gastrointestinal CFTR dysfunction leads to mucus accumulation, dysmotility, small bowel bacterial overgrowth and inflammation with alteration of innate immune responses, all of which being likely to be interrelated. In developed countries, almost half of patients with CF are adults followed in multidisciplinary CF care centres by pneumologists who often have to manage gastrointestinal complications.


It therefore appears essential that adult gastroenterologists develop the expertise needed for managing CF gastrointestinal complications in close collaboration with multidisciplinary CF care centre teams to improve the quality of life of adults with CF.


Atteinte hépatique; Complications gastro-intestinales; Cystic fibrosis; Gastrointestinal complications; Liver disease; Mucoviscidose; Pancreatitis; Pancréatite

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