Abstract
BackgroundPancreatic destruction affects the majority of patients with cystic fibrosis. We aimed to relate ultrasound findings to exocrine pancreatic function and cystic fibrosis genotype.MethodsPatients with cystic fibrosis and a matched group of healthy controls were included. We performed transabdominal ultrasound, and recorded echo intensities of the pancreas and parenchymal characteristics according to endoscopic ultrasound based Rosemont criteria.ResultsWe included 39 patients and 29 healthy controls. The cystic fibrosis patients were grouped according to exocrine pancreatic function; Cystic fibrosis, insufficient (n = 20) and sufficient (n = 19). Echo intensity measures and visual score demonstrated hyper-echogenicity in the pancreas insufficient group compared to the pancreas sufficient groups (p<0.001). Ductal and parenchymal changes were not prevalent in any of the groups.ConclusionThe hyper-echoic pancreas was the most frequent ultrasonographic finding in exocrine pancreas insufficient cystic fibrosis patients. Pancreatic echo levels correlated to pancreatic phenotype.
Highlights
Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations in a single large gene on chromosome 7 encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein, a complex chloride channel and regulatory protein found in all exocrine tissues
We aim to correlate pancreatic ultrasound characteristics to CF genotype and exocrine pancreatic function assessed by secretin-stimulated endoscopic short test [25,26] or faecal elastase [10] in CF patients
When we evaluated the pancreas according to criteria in the endoscopic ultrasound (EUS) based Rosemont chronic pancreatitis score, we found no major and only few minor criteria in our patients
Summary
Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations in a single large gene on chromosome 7 encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein, a complex chloride channel and regulatory protein found in all exocrine tissues. The Cystic Fibrosis Mutation Database lists more than 1900 different mutations in the CFTR gene [4]. Patients with cystic fibrosis develop pancreatic damage as a result of defective ductal and acinar pancreatic secretion [7,8]. Population studies indicate that 72–88% of CF patients develop exocrine pancreatic insufficiency [9,10]. Pancreatic destruction affects the majority of patients with cystic fibrosis. We aimed to relate ultrasound findings to exocrine pancreatic function and cystic fibrosis genotype. Academic Editor: Henrik Einwaechter, Klinikum rechts der Isar der TU München, GERMANY Received: October 17, 2014 Accepted: January 28, 2015 Published: March 24, 2015
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