Fibrosing colonopathy is a recently described complication of
cystic fibrosis, of unknown aetiology but possibly related to treatment with high-dose pancreatic
enzyme supplements. We have used a whole gut perfusion technique to study subclinical gut
inflammation in
cystic fibrosis patients; concentrations of haemoglobin,
IgG,
albumin, alpha-1-antitrypsin,
granulocyte elastase, IL1 beta, and
IL8 were measured in whole gut lavage fluid: 23 tests were performed in 17 children with
cystic fibrosis (20 elective tests, three lavages to treat distal
intestinal obstruction syndrome (DIOS)). None has had fibrosing or haemorrhagic
colitis. There were 12 tests in control children with
constipation or precolonoscopy. Moderately abnormal results were obtained for many of the parameters studied, in specimens from all the
cystic fibrosis children; however there were no significant differences between tests on high-dose and low-dose
enzyme supplements of the same brand in the five children who had duplicate tests performed electively. The lavage fluid specimens from two
cystic fibrosis children were strikingly abnormal in all tests apart from haemoglobin and alpha-1-antitrypsin. These were two of the three children with DIOS, and were also the only cases in the series taking
Nutrizym 22. These data suggest that the majority of
cystic fibrosis children, including those on high-dose
enzyme supplements, do not have clinically significant
colitis, but that there is subclinical mucosal
inflammation in a minority (two of 17 in this series), for which DIOS and/or
Nutrizym 22 treatment may be risk factors. Alternatively,
inflammation and dysmotility in the proximal colon may be directly produced by a
drug or other agent, producing a clinical syndrome indistinguishable from DIOS. Tests for indices of
inflammation in gut lavage fluid offer a new approach to the detection and measurement of iatrogenic intestinal and colonic injury.