Exocrine Pancreatic Function Testing in Cystic Fibrosis

Pancreatic Failure in Patients With Cystic Fibrosis A Multimodal Study of Exocrine Pancreatic Failure in Cystic Fibrosis

Purpose The purpose of this study is to develop and validate multimodal testing of exocrine pancreatic function (EPF). The investigators will be testing exocrine pancreatic function in patients with cystic fibrosis (CF). Exocrine pancreatic function and imaging will be correlated to age group, genotype, nutritional status and quality of life. Earlier detection of exocrine pancreatic failure in the non classical form of cystic fibrosis may be of therapeutically benefit.

Hypotheses Endoscopic short test can be applied in diagnosing and monitoring exocrine pancreatic function in patients with cystic fibrosis.

New functional testing of exocrine pancreatic function is superior to traditional testing with fecal elastase.

MRI and ultrasound methods can give volume output estimate in cystic fibrosis patients.

Contrast enhanced ultrasound can quantify reduced or delayed pancreatic perfusion and parenchymal changes in cystic fibrosis patients.

Elastography/ CEUS can be used in prediction and monitoring of fibrosis development and development of hepatocellular carcinoma in the liver of cystic fibrosis patients.

Immunohistochemical quantification of secretin/ cholecystokinin (CCK) producing cell in duodenum can be utilized as a model hormonal signaling in cystic fibrosis patients with exocrine pancreatic function.

Study Overview

• Status: Unknown
• Conditions: Cystic Fibrosis

Detailed Description

Study design: Observational, cohort studies.

Patient characterization: Age, gender, and Genetic status from electronical records.

Exocrine function testing (EPF): Secretin stimulated ultrasound and short endoscopic secretin test (EST). Faecal Elastase.

Imaging: Transabdominal ultrasound of the liver and pancreas. secretin stimulated MRI. Contrast enhanced ultrasound (CEUS) of the pancreas using SonoVue contrast.

Endpoints:

Study 1: Exocrine pancreatic function by duodenal bicarbonate/ Enzymes related to Genetics and F elastase.

Study 2: Ultrasound parenchymal changes of the pancreas related to Genetics and EPF.

Study 3/4: Pancreatic secretion by ultrasound, MRI and EST related to EPF Study 5; Perfusion of the pancreas by CEUS related to EPF

Study 6: Genotype-phenotype conciderations of the CF pancreas.

• Study Type: Observational
• Enrollment (Anticipated): 60

Contacts and Locations

Study Locations

• Norway
  • Bergen, Norway, 5020
    • Haukeland University Hospital

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 15 years and older (Child, Adult, Older Adult)
• Accepts Healthy Volunteers: Yes
• Genders Eligible for Study: All

• Sampling Method: Probability Sample

Study Population

Cystic fibrosis patients over the age of 15 years Healthy controls between 18 and 67 years

Description

Inclusion Criteria:

• Diagnosis cystic fibrosis. Age over 15 years. Assigned to routine control at department of lung diseases, Haukeland university hospital.

Exclusion Criteria:

• Unable to concent, allergies to Sonovue ultrasound contrast, pregnant or breastfeeding, contraindications against endoscopy.

Study Plan

How is the study designed?

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort
Cystic fibrosis patients; Consecutive cystic fibrosis patients attending regular Controls at the CF clinic in Bergen
Healthy controls; Age and gender matchet healthy Controls recruited by Board notice and advertising.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Time Frame
peak lipase IE	one year

Secondary Outcome Measures

Outcome Measure	Time Frame
enzyme production	2 years

Other Outcome Measures

Outcome Measure	Time Frame
emzyme production	2 years

Collaborators and Investigators

• Sponsor: Haukeland University Hospital
• Collaborators: University of Bergen
• Investigators: Principal Investigator: Georg Dimcevski, MD, PhD, Haukeland University Hospital; Study Director: Odd H Gilja, Professor, Haukeland University Hospital

Study record dates

Study Major Dates

• Study Start: January 1, 2011
• Primary Completion (Anticipated): July 1, 2018
• Study Completion (Anticipated): July 1, 2018

Study Registration Dates

• First Submitted: October 4, 2011
• First Submitted That Met QC Criteria: October 4, 2011
• First Posted (Estimate): October 5, 2011

Study Record Updates

• Last Update Posted (Estimate): August 24, 2016
• Last Update Submitted That Met QC Criteria: August 23, 2016
• Last Verified: August 1, 2016

More Information

Terms related to this study

• Keywords: Ultrasound; Cystic fibrosis; Pancreas; Contrast ultrasound; Exocrine function
• Additional Relevant MeSH Terms: Digestive System Diseases; Pathologic Processes; Respiratory Tract Diseases; Lung Diseases; Infant, Newborn, Diseases; Genetic Diseases, Inborn; Pancreatic Diseases; Fibrosis; Cystic Fibrosis
• Other Study ID Numbers: 2010/2857-7

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: YES
• IPD Plan Description: Data will be published in peer rewieved journals