Aim There is increasing evidence to suggest the presence of chronic inflammation in the gastrointestinal (GI) tract of cystic fibrosis (CF) patients. Some CF patients continue to have very severe GI symptoms despite conventional CF treatment. In our centre, these patients are managed in a CF gastroenterology clinic, jointly with a gastroenterologist. A number of them have required GI endoscopy and biopsy. The aim of our study was to characterise these patients and determine whether endoscopy and biopsy changed their management.
Methods We reviewed all the patients seen in the CF gastroenterology clinic from 2004 to 2009, who had GI endoscopies performed. The GI symptoms these patients were experiencing ranged from abdominal pain, nausea and vomiting, rectal bleeding, severe failure to thrive, diarrhoea to constipation. These patients had already received intensive input from the dieticians and physicians in the CF clinic prior to referral to the gastroenterologist.
Results 12 patients were identified. The mean [SD] age at referral to the CF gastroenterology clinic was 4.9 (4.1) years. Their body mass index was 15.1 (1.8). 25% were homozygous delF508. Two patients had previously had meconium ileus as neonates and both had required surgery for this. One other patient had needed abdominal surgery for intussusception. 92% were pancreatic insufficient, 25% were chronically infected with Pseudomonas aeruginosa and 17% were on regularly three monthly intravenous antibiotics. Of the 10 patients who were able to perform spirometry, mean (SD) FEV1 was 95 (30)% predicted. The biopsies in three patients were normal, but the rest (nine) showed evidence of inflammation including duodenitis with mucosal eosinophilic infiltrate, duodenitis with severe partial villous atrophy, colitis and chronic inactive gastritis. In the nine patients with evidence of GI inflammation on biopsy, immunomodulators were commenced, including sodium cromoglycate, prednisolone, azathioprine, methotrexate, ketotifen, mesalazine and sulphasalazine. 4 were started on a special feed such as an elemental feed, and 1 patient had such severe symptoms associated with GI inflammation, she was commenced on TPN.
Conclusion In summary, GI endoscopy and biopsy has a significant role in the management of CF children with severe GI disease. In our study, it influenced the management in 75% of patients with severe GI symptoms. Furthermore, if severe GI inflammation is identified on biopsy, management with immunomodulatory agents may be helpful.