Exocrine Pancreatic Function Testing in Cystic Fibrosis

Cystic Fibrosis

Cystic fibrosis, Pancreas, Exocrine function, Ultrasound, Contrast ultrasound

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, 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.

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