Disorders of the Exocrine Pancreas

Published on 25/03/2015 by admin

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Chapter 341 Disorders of the Exocrine Pancreas

Steven L. Werlin

Disorders Associated with Pancreatic Insufficiency

Other than cystic fibrosis, conditions that cause pancreatic insufficiency are rare in children. They include Shwachman-Diamond syndrome, isolated enzyme deficiencies, enterokinase deficiency (Chapter 330), chronic pancreatitis, and protein-calorie malnutrition (Chapters 43 and 330).

Cystic Fibrosis (Chapter 395)

Cystic fibrosis is the most common lethal genetic disease and the most common cause of malabsorption among white American or European children. By the end of the 1st yr of life, 85-90% of children with cystic fibrosis (CF) have pancreatic insufficiency, which, if untreated, can lead to malnutrition. Treatment of the associated pancreatic insufficiency leads to improvement in absorption, better growth, and normalized stools. Pancreatic function can be monitored in children with CF with serial measurements of fecal elastase. Certain mutations in the cystic fibrosis gene have been associated with idiopathic chronic pancreatitis. CF is part of the newborn screen in every state in the United States.

Shwachman-Diamond Syndrome (Chapter 125)

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