(13)CARBON MIXED TRIGLYCERIDE BREATH TEST AND PANCREATIC-ENZYME SUPPLEMENTATION IN CYSTIC-FIBROSIS

Children with cystic fibrosis have variable degrees of exocrine pancre atic insufficiency which, if untreated, is the main cause of fat malab sorption. The impact of pancreatic enzyme supplementation on fat diges tion was measured in 41 children with cystic fibrosis, 11 healthy cont rols, and five children with mucosal diseases by a non-invasive test o f intraluminal Lipolysis using (13)carbon (C-13) labelled mixed trigly ceride (1,3-distearyl, 2[C-13] octanoyl glycerol). The children with c ystic fibrosis without pancreatic supplements had a median (range) C-1 3 cumulative percentage dose recovered over six hours (cPDR) of 3.1% ( 0-31.7), the controls 31.0% (21.8-41.1), and the subjects with mucosal disease 27.8% (19.7-32.5). In 23 subjects with cystic fibrosis the us ual dose of pancreatic enzyme supplements increased the cPDR to a medi an of 23.9% (0-45.6), and twice the usual dose of enteric coated micro spheres increased the cPDR to 31.1% (11.1-47.8). There was no signific ant difference between the median cPDR of normal controls and children with mucosal disease, but there was a highly significant difference b etween these groups and children with untreated cystic fibrosis. Thirt een children with cystic fibrosis had no C-13 recovery in their breath without enzymes and 10 showed marked increases with regular enzymes. In eight children doubling the dose of enzymes caused no or minimal im provement. The mixed triglyceride breath test offers a simple, non-inv asive way of assessing the need for pancreatic enzyme supplementation in children with cystic fibrosis and could be used to optimise treatme nt.