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Gastrointestinal, liver, and pancreatic involvement in adult patients with cystic fibrosis.
Pancreas 2001; 22(4):395-9P

Abstract

BACKGROUND

The clinical prevalence of cystic fibrosis (CF) in adults continues to rise, with a consequent impact on adult gastroenterology practice.

AIM

To characterize the gastrointestinal manifestations of CF in adult patients.

PATIENTS AND 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.

Authors+Show Affiliations

Digestive System Research Unit, Hospital General Vall d' Hebrón, Autonomous University of Barcelona, Spain.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

11345141

Citation

Modolell, I, et al. "Gastrointestinal, Liver, and Pancreatic Involvement in Adult Patients With Cystic Fibrosis." Pancreas, vol. 22, no. 4, 2001, pp. 395-9.
Modolell I, Alvarez A, Guarner L, et al. Gastrointestinal, liver, and pancreatic involvement in adult patients with cystic fibrosis. Pancreas. 2001;22(4):395-9.
Modolell, I., Alvarez, A., Guarner, L., De Gracia, J., & Malagelada, J. R. (2001). Gastrointestinal, liver, and pancreatic involvement in adult patients with cystic fibrosis. Pancreas, 22(4), pp. 395-9.
Modolell I, et al. Gastrointestinal, Liver, and Pancreatic Involvement in Adult Patients With Cystic Fibrosis. Pancreas. 2001;22(4):395-9. PubMed PMID: 11345141.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Gastrointestinal, liver, and pancreatic involvement in adult patients with cystic fibrosis. AU - Modolell,I, AU - Alvarez,A, AU - Guarner,L, AU - De Gracia,J, AU - Malagelada,J R, PY - 2001/5/10/pubmed PY - 2001/9/14/medline PY - 2001/5/10/entrez SP - 395 EP - 9 JF - Pancreas JO - Pancreas VL - 22 IS - 4 N2 - BACKGROUND: The clinical prevalence of cystic fibrosis (CF) in adults continues to rise, with a consequent impact on adult gastroenterology practice. AIM: To characterize the gastrointestinal manifestations of CF in adult patients. PATIENTS AND 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. SN - 0885-3177 UR - https://www.unboundmedicine.com/medline/citation/11345141/Gastrointestinal_liver_and_pancreatic_involvement_in_adult_patients_with_cystic_fibrosis_ L2 - http://Insights.ovid.com/pubmed?pmid=11345141 DB - PRIME DP - Unbound Medicine ER -