SHIELD-Studie

Observational study on the radiologic and biochemical effects of choline supplementation on Cystic Fibrosis associated liver disease in children and young adults

01/05/2023 to 31/05/2025

Cholin deficiency is characteristic for Cystic Fibrosis (CF) patients suffering from exocrine pancreas insufficiency. Choline deficiency results in acute and chronic liver disease (steatosis, cholestasis, fibrosis, liver failure). CF-associated liver disease (CFALD) is frequent in CF patients. In a prospektive randomized and placebo-controlled double-blind study on 50 CF patients from 6 to 25 years with CFALD and choline deficiency we will investigate, whether a supplementation in a dosage high enough to normalize plasma choline concentrations, will improve or normalize steatosis, increased liver enzymes in plasma and altered liver structure and size. Diagnostics will be performed by routine laboratory parameters and mass spectrometric analysis of choline parameters as well as magnetic resonance spectroscopy and ultrasound/elastography of the liver.