Pancreatic insufficiency in cystic fibrosis

Damage to the exocrine pancreas in cystic fibrosis patients due to viscous secretions and ductal obstruction. Develops in 85% of CF patients and requires lifelong pancreatic enzyme replacement therapy.

ICD-10 E84.81 drug

Also known as: CF-related pancreatic insufficiency

Drugs used for this condition

APancreatinFirst line · Digestives. Multienzyme preparations85% of cystic fibrosis patients develop exocrine insufficiency from childhood. PERT is lifelong, started immediately after diagnosis. Doses are based on lipase per kilogram of body weight: 500–1000 IU lipase/kg/meal in children, 500–2500 IU lipase/kg/meal in adults. Maximum 10 000 IU lipase/kg/day to prevent fibrosing colonopathy.

How to read tiers A–F

Evidence tiers belong to the drug-indication pair, not to the drug as a whole: the same substance can be A for one condition and F for another.

AStrong evidence – first or second line in international guidelines
BRecommended in selected cases
CStudy data contradict each other
DBenefit not proven, no documented harm
FDocumented harm, or risk without benefit