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a Department of Paediatrics, University of Modena, Italy, b MRC Dunn Nutrition Unit, Cambridge, c Royal Hospital for Sick Children,
Glasgow and University of Glasgow: Department of Human Nutrition, d Department of Child Health
Correspondence to: Dr Sergio Amarri, Clinica Pediatrica, Via del Pozzo 71, 41100 Modena, Italy.
Accepted 5 November 1996
Children with cystic fibrosis have variable degrees of exocrine
pancreatic insufficiency which, if untreated, is the main cause of fat
malabsorption. The impact of pancreatic enzyme supplementation on fat
digestion was measured in 41 children with cystic fibrosis, 11 healthy
controls, and five children with mucosal diseases by a non-invasive
test of intraluminal lipolysis using 13carbon
(13C) labelled mixed triglyceride (1,3-distearyl,
2[13C] octanoyl glycerol). The children with cystic
fibrosis without pancreatic supplements had a median (range)
13C 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 usual dose of pancreatic enzyme supplements
increased the cPDR to a median of 23.9% (0-45.6), and twice the usual
dose of enteric coated microspheres increased the cPDR to 31.1%
(11.1-47.8). There was no significant difference between the median
cPDR of normal controls and children with mucosal disease, but there
was a highly significant difference between these groups and children with untreated cystic fibrosis. Thirteen children with cystic fibrosis
had no 13C 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 improvement. The
mixed triglyceride breath test offers a simple, non-invasive way of
assessing the need for pancreatic enzyme supplementation in children
with cystic fibrosis and could be used to optimise treatment.
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