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Management of pancreatic, gastrointestinal and liver complications in adult cystic fibrosis.
Rev Mal Respir 2015; 32(6):566-85RM

Abstract

INTRODUCTION

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.

STATE OF KNOWLEDGE

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.

CONCLUSION

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.

Authors+Show Affiliations

Assistance 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: anne.munck@rdb.aphp.fr.Département de pédiatrie médicale, Centre de ressources et de compétence de la mucoviscidose, CHU de Limoges, 87000 Limoges, France.Service de chirurgie viscérale pédiatrique - Hépatologie pédiatrique, AP-HP, CHU Necker-Enfants-Malades, 75015 Paris, France.Gastro-entérologie, Hépatologie et Nutrition Pédiatriques, Hôpital des Enfants, 33077 Bordeaux, France.CRCM Reims, American Memorial Hospital, 51092 Reims, France.Service de Pédiatrie 1, Hôpital d'Enfants, CHU de Dijon, 21079 Dijon, France.Service de Pédiatrie 1, Hôpital d'Enfants, CHU de Dijon, 21079 Dijon, France.Centre 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.Unité 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.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

26001957

Citation

Munck, A, et al. "Management of Pancreatic, Gastrointestinal and Liver Complications in Adult Cystic Fibrosis." Revue Des Maladies Respiratoires, vol. 32, no. 6, 2015, pp. 566-85.
Munck A, Languepin J, Debray D, et al. Management of pancreatic, gastrointestinal and liver complications in adult cystic fibrosis. Rev Mal Respir. 2015;32(6):566-85.
Munck, A., Languepin, J., Debray, D., Lamireau, T., Abely, M., Huet, F., ... Mas, E. (2015). Management of pancreatic, gastrointestinal and liver complications in adult cystic fibrosis. Revue Des Maladies Respiratoires, 32(6), pp. 566-85. doi:10.1016/j.rmr.2014.12.008.
Munck A, et al. Management of Pancreatic, Gastrointestinal and Liver Complications in Adult Cystic Fibrosis. Rev Mal Respir. 2015;32(6):566-85. PubMed PMID: 26001957.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Management of pancreatic, gastrointestinal and liver complications in adult cystic fibrosis. AU - Munck,A, AU - Languepin,J, AU - Debray,D, AU - Lamireau,T, AU - Abely,M, AU - Huet,F, AU - Maudinas,R, AU - Michaud,L, AU - Mas,E, Y1 - 2015/05/19/ PY - 2014/05/12/received PY - 2014/12/09/accepted PY - 2015/5/24/entrez PY - 2015/5/24/pubmed PY - 2016/5/18/medline KW - Atteinte hépatique KW - Complications gastro-intestinales KW - Cystic fibrosis KW - Gastrointestinal complications KW - Liver disease KW - Mucoviscidose KW - Pancreatitis KW - Pancréatite SP - 566 EP - 85 JF - Revue des maladies respiratoires JO - Rev Mal Respir VL - 32 IS - 6 N2 - INTRODUCTION: 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. STATE OF KNOWLEDGE: 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. CONCLUSION: 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. SN - 1776-2588 UR - https://www.unboundmedicine.com/medline/citation/26001957/Management_of_pancreatic_gastrointestinal_and_liver_complications_in_adult_cystic_fibrosis_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0761-8425(15)00189-8 DB - PRIME DP - Unbound Medicine ER -