Distal intestinal obstruction syndrome in adults with cystic fibrosis.
OBJECTIVE
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.
Dray
Xavier
X
Service d'Hépati-gastroentérologie, Hôpital Européen Georges Pompidou, Paris, France.
Bienvenu
Thierry
T
Desmazes-Dufeu
Nadine
N
Dusser
Daniel
D
Marteau
Philippe
P
Hubert
Dominique
D
D000293:Adolescent; D000328:Adult; D002429:Cecal Diseases / Q000235:genetics*; D003550:Cystic Fibrosis / Q000150:complications* / Q000235:genetics*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics*; D005260:Female; D005500:Follow-Up Studies; D005838:Genotype; D006801:Humans; D007077:Ileal Diseases / Q000235:genetics*; D007415:Intestinal Obstruction / Q000235:genetics*; D008297:Male; D008875:Middle Aged; D009154:Mutation; D012189:Retrospective Studies; D013577:Syndrome
C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator