Missing or Medicated Pancreas Means Missing Key Enzymes

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HI Hope

Children with congenital hyperinsulinism (HI) may be at risk for exocrine pancreatic insufficiency, which affects the ability of the pancreas to make and secrete digestive enzymes, as well as pancreatic juice.

Both of these exocrine pancreas products are important for the digestion and absorption of nutrients. In particular, absorption of fat and fat-soluble vitamins (vitamins A, D, E and K) relies on normal exocrine pancreas structure and function. Pancreatic juice is also important because it boosts the effectiveness of these pancreatic enzymes and creates the ideal environment for the digestion and absorption of other vitamins and minerals, including vitamin B12.

HI patients may have exocrine pancreatic insufficiency because of medications used to manage HI or when they’ve had a neartotal pancreatectomy. The end result is children with HI are at risk for deficiencies of essential fatty acids, fat-soluble vitamins, vitamin B12 and other essential nutrients.

Subtle signs of pancreatic insufficiency

While symptoms of exocrine pancreatic insufficiency may be subtle and go unnoticed, they have significant and profound potential to adversely affect nutrition and growth. CHOP’s Center for Pancreatic Disorders follows children carefully to prevent, screen for, detect and correct any nutritional deficiencies that may happen. Using specific supplemental vitamins, pancreatic enzyme replacement therapy and sometimes adding acid blockers may help compensate for impaired pancreas function and optimize health.

Advanced Care for Our Patients

We now have a multidisciplinary clinic to care for children who have undergone pancreatectomy for HI. Endocrinologists, gastroenterologists and nutritionists offer a comprehensive evaluation of glycemic control, exocrine pancreatic function and growth. To schedule an appointment for your child, call 215-590-3174.


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