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Distal intestinal obstruction syndrome in adults with cystic fibrosis.
Clin Gastroenterol Hepatol 2004; 2(6):498-503CG

Abstract

BACKGROUND & AIMS

With the improved survival of patients with cystic fibrosis (CF), gastrointestinal complications become more evident in adults with this condition. The aims of this study were to determine the prevalence and clinical features of distal intestinal obstruction syndrome (DIOS) and its relationship with the cystic fibrosis transmembrane conductance regulator (CFTR) genotype in an adult CF population.

METHODS

Cross-sectional study was conducted in an adult CF cohort.

RESULTS

Among 171 adults with CF (mean age, 28.9 years), 27 patients (15.8%) reported 43 episodes of DIOS. No significant association was found between DIOS and a history of meconium ileus. The first episode of DIOS occurred in adulthood in 21 cases (77.8%). DIOS recurred in 13 patients (48.1%). All patients who developed DIOS had pancreatic insufficiency. Pulmonary function was significantly more altered in patients with DIOS than in the other patients, but pancreatic insufficiency and age might act as confounding factors. DIOS occurred in 21.9% of patients with a severe CFTR genotype and in only 2.4% of patients with a mild CFTR genotype (P < 0.005).

CONCLUSIONS

DIOS is frequent in adults with CF with a severe CFTR genotype and/or advanced-stage pulmonary disease. The relative contributions of malabsorption and impaired intestinal secretion in the development of DIOS are discussed.

Authors+Show Affiliations

Service d'Hépati-gastroentérologie, Hôpital Européen Georges Pompidou, Paris, France.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

15181619

Citation

Dray, Xavier, et al. "Distal Intestinal Obstruction Syndrome in Adults With Cystic Fibrosis." Clinical Gastroenterology and Hepatology : the Official Clinical Practice Journal of the American Gastroenterological Association, vol. 2, no. 6, 2004, pp. 498-503.
Dray X, Bienvenu T, Desmazes-Dufeu N, et al. Distal intestinal obstruction syndrome in adults with cystic fibrosis. Clin Gastroenterol Hepatol. 2004;2(6):498-503.
Dray, X., Bienvenu, T., Desmazes-Dufeu, N., Dusser, D., Marteau, P., & Hubert, D. (2004). Distal intestinal obstruction syndrome in adults with cystic fibrosis. Clinical Gastroenterology and Hepatology : the Official Clinical Practice Journal of the American Gastroenterological Association, 2(6), pp. 498-503.
Dray X, et al. Distal Intestinal Obstruction Syndrome in Adults With Cystic Fibrosis. Clin Gastroenterol Hepatol. 2004;2(6):498-503. PubMed PMID: 15181619.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Distal intestinal obstruction syndrome in adults with cystic fibrosis. AU - Dray,Xavier, AU - Bienvenu,Thierry, AU - Desmazes-Dufeu,Nadine, AU - Dusser,Daniel, AU - Marteau,Philippe, AU - Hubert,Dominique, PY - 2004/6/8/pubmed PY - 2004/11/9/medline PY - 2004/6/8/entrez SP - 498 EP - 503 JF - Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association JO - Clin. Gastroenterol. Hepatol. VL - 2 IS - 6 N2 - BACKGROUND & AIMS: With the improved survival of patients with cystic fibrosis (CF), gastrointestinal complications become more evident in adults with this condition. The aims of this study were to determine the prevalence and clinical features of distal intestinal obstruction syndrome (DIOS) and its relationship with the cystic fibrosis transmembrane conductance regulator (CFTR) genotype in an adult CF population. METHODS: Cross-sectional study was conducted in an adult CF cohort. RESULTS: Among 171 adults with CF (mean age, 28.9 years), 27 patients (15.8%) reported 43 episodes of DIOS. No significant association was found between DIOS and a history of meconium ileus. The first episode of DIOS occurred in adulthood in 21 cases (77.8%). DIOS recurred in 13 patients (48.1%). All patients who developed DIOS had pancreatic insufficiency. Pulmonary function was significantly more altered in patients with DIOS than in the other patients, but pancreatic insufficiency and age might act as confounding factors. DIOS occurred in 21.9% of patients with a severe CFTR genotype and in only 2.4% of patients with a mild CFTR genotype (P < 0.005). CONCLUSIONS: DIOS is frequent in adults with CF with a severe CFTR genotype and/or advanced-stage pulmonary disease. The relative contributions of malabsorption and impaired intestinal secretion in the development of DIOS are discussed. SN - 1542-3565 UR - https://www.unboundmedicine.com/medline/citation/15181619/Distal_intestinal_obstruction_syndrome_in_adults_with_cystic_fibrosis_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1542356504001697 DB - PRIME DP - Unbound Medicine ER -