Exocrine Pancreas Insufficiency is usually diagnosed in the first year of life. Due to the lack of pancreas enzyme fat and fat-soluble vitamins are not absorbed in the gut.

There is no doubt that pancreatic enzyme replacement therapy (PERT) is indicated in patients with EPI.

If PERT is conducted properly, no fat restrictions are necessary. Patients should take Pancreatin along with a normal diet instead of changing their diet. If there are deficits in fat-soluble vitamins (A, D, E), prothrombin time as a surrogate for vitamin K and trace elements should be recorded, these should be substituted accordingly.

Blood levels of fat-soluble vitamins should be measured every 6 to 12 months in young children, and supplementary therapy should be started if values are low. It is important to ensure compliance with pancreatic enzyme supplementation, as deficiencies of these vitamins are an indirect marker of fat malabsorption.