Gastrointestinal, liver, and pancreatic involvement in adult patients with cystic fibrosis.
BACKGROUND
The clinical prevalence of cystic fibrosis (CF) in adults continues to rise, with a consequent impact on adult gastroenterology practice.


OBJECTIVE
To characterize the gastrointestinal manifestations of CF in adult patients.


METHODS
The clinical records of 89 adult CF patients treated at our institution from 1992 to 1999 were reviewed. Patients were distributed into two groups: group A (39 patients), which consisted of patients who were diagnosed with CF at when they were younger than 14 years old and who survived into adulthood; and group B (50 patients), who were diagnosed with CF at the age of 14 years or older. Data on CF genetic mutations, nutritional state, evidence of pulmonary, gastrointestinal, liver, or pancreatic involvement were collected for each patient.


RESULTS
The most prevalent genetic mutation in our series was deltaF508, present in 50 patients (56.2%), 29 of whom belonged to group A and 21 who belonged to group B. In group A, the deltaF508 mutation was associated with exocrine pancreatic insufficiency (PI) in 26 of 29 patients (89.6%), whereas in group B it was associated with PI in only four patients (19%). Overall, PI was present in 33 of 39 patients (84.6%) in group A and in eight of 50 patients (16%) in group B. Four patients in group B had experienced previous episodes of acute pancreatitis; two of them had associated PI. Of the 89 patients, 12 (10 in group A) were malnourished. Malnutrition was invariably associated with PI. Hepatic and biliary tree abnormalities were particularly prevalent in patients in group A and was usually associated with PI. Intestinal manifestations were uncommon.


CONCLUSIONS
Diagnosis of CF before the age of 14 years is associated with greater gastrointestinal compromise than diagnosis at an older age, particularly with regard to PI. CF carriers of the deltaF508 mutation have an increased risk of developing gastrointestinal manifestations.
Modolell
I
I
Digestive System Research Unit, Hospital General Vall d' Hebrón, Autonomous University of Barcelona, Spain.
Alvarez
A
A
Guarner
L
L
De Gracia
J
J
Malagelada
J R
JR
D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D002769:Cholelithiasis / Q000209:etiology; D003550:Cystic Fibrosis / Q000150:complications* / Q000175:diagnosis / Q000235:genetics; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D010188:Exocrine Pancreatic Insufficiency / Q000209:etiology / Q000235:genetics; D005260:Female; D005767:Gastrointestinal Diseases / Q000209:etiology*; D006801:Humans; D008107:Liver Diseases / Q000209:etiology*; D008171:Lung Diseases / Q000209:etiology / Q000382:microbiology; D008297:Male; D008875:Middle Aged; D009154:Mutation; D009748:Nutrition Disorders / Q000209:etiology; D009752:Nutritional Status; D010182:Pancreatic Diseases / Q000209:etiology*; D010195:Pancreatitis / Q000209:etiology; D011549:Pseudomonas / Q000302:isolation & purification; D012189:Retrospective Studies
C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator