CFTR modulators increase risk of acute pancreatitis in pancreatic insufficient patients with cystic fibrosis.

Patients with pancreatic insufficient cystic fibrosis rarely develop acute pancreatitis due to insufficient acinar reserve. We describe a series of five patients under the age of 18 (range 8-16 years) with pancreatic insufficient cystic fibrosis who developed a phenotype in keeping with acute pancreatitis following initiation of CFTR modulator therapy. This occurred at a median of 30 months following CFTR modulator initiation.

Crohn's disease and cheilitis.

A five-year-old boy presented to his family physician with painless swelling of both lips. One year later he developed abdominal pain, nonbloody diarrhea, weight loss and joint pains. Colonoscopic examination demonstrated patchy erythema, friability and multiple aph-thous ulcers consistent with the appearance of Crohn's colitis, and treatment with prednisone was initiated.