Exocrine Pancreatic Function Testing 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 Design:||Observational Model: Case Control
Time Perspective: Cross-Sectional
|Official Title:||Pancreatic Failure in Patients With Cystic Fibrosis A Multimodal Study of Exocrine Pancreatic Failure in Cystic Fibrosis|
- peak lipase IE [ Time Frame: one year ] [ Designated as safety issue: No ]
- enzyme production [ Time Frame: 2 years ] [ Designated as safety issue: No ]
- emzyme production [ Time Frame: 2 years ] [ Designated as safety issue: No ]
Biospecimen Retention: Samples With DNA
|Study Start Date:||January 2011|
|Estimated Study Completion Date:||July 2018|
|Estimated Primary Completion Date:||July 2018 (Final data collection date for primary outcome measure)|
Cystic fibrosis patients
Consecutive cystic fibrosis patients attending regular Controls at the CF clinic in Bergen
Age and gender matchet healthy Controls recruited by Board notice and advertising.
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.
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.
Please refer to this study by its ClinicalTrials.gov identifier: NCT01446861
|Haukeland University Hospital|
|Bergen, Norway, 5020|
|Principal Investigator:||Georg Dimcevski, MD, PhD||Haukeland University Hospital|
|Study Director:||Odd H Gilja, Professor||Haukeland University Hospital|