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Editor,—We read with interest the report by Bansi and colleagues (OpenUrlPubMedWeb of Science) describing fibrosing colonopathy secondary to high dose pancreatic enzyme therapy in an adult patient. Some details of the patient's history—cholangitis, pancreatitis, and pancreatic insufficiency—are strikingly similar to symptoms displayed by our adult patient with cystic fibrosis and fibrosing colonopathy described previously.1 In this patient with cystic fibrosis, chronic cholangitis and cholelithiasis required repeated endoscopic retrograde cholangiopancreaticography, and severe pancreatic insufficiency was the reason for high dose pancreatic enzyme supplementation. Bansi et al assume that their patient was not suffering from cystic fibrosis. As previously discussed in the commentary by Dodge in the same issue,2 negative results after even extensive mutation analysis of the cystic fibrosis transmembrane regulator gene cannot rule out cystic fibrosis. Furthermore, as outlined by the Cystic Fibrosis Foundation Consensus Panel,3 sweat testing is the standard test for diagnosis of cystic fibrosis. In patients with typical clinical symptoms but normal or borderline sweat chloride concentrations and normal genetic findings, nasal potential difference measurements should be performed. The clinical symptoms of the patient described by Bansi et al are highly indicative of cystic fibrosis with exclusive involvement of the gastrointestinal tract. Moreover, the histopathology of the pancreatic tissue, showing glandular fibrosis and atrophy, is also typical of cystic fibrosis, as are frequent bowel actions.4
Proof of fibrosing colonopathy in a patient not suffering from cystic fibrosis may contribute considerably to a better understanding of the pathogenesis of fibrosing colonopathy which is still a matter of discussion.5 ,6 It would underline the aetiological impact of toxic effects of high dose pancreatic enzyme supplementation but caution against overestimating the contribution of factors possibly related to the cystic fibrosis transmembrane regulator gene mutation, such as increased intestinal absorption.2 We would therefore be interested in the patient's sweat chloride concentration and, if normal, in the result of nasal potential difference measurements. This paper strongly advocates well thought out supplementation of pancreatic enzymes in adults who, like infants and children, are at risk of developing fibrosing colonopathy.
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