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Disorders of the Exocrine Pancreas
Disorders Associated With Pancreatic Insufficiency
Other than cystic fibrosis (CF), conditions that cause pancreatic insufficiency are very rare in children. They include Shwachman-Diamond syndrome (SDS), Johanson-Blizzard syndrome, Ivemark syndrome, Pearson syndrome, isolated enzyme deficiencies, enterokinase deficiency (see Chapter 364 ), chronic pancreatitis, protein-calorie malnutrition (see Chapters 57 and 364 ), and IMNEPD (infantile onset multisystem neurologic, endocrine, and pancreatic disease).
Cystic Fibrosis (see Chapter 432 )
By the end of the 1st yr of life, 85–90% of children with CF have pancreatic insufficiency, which, if untreated, will lead to malnutrition. Treatment of the associated pancreatic insufficiency leads to improvement in absorption, better growth, and more normal stools. Pancreatic function can be monitored in children with CF with serial measurements of fecal elastase. Ten to 15% of children present with a neonatal intestinal obstruction called meconium ileus; in later life a common intestinal complication is distal intestinal obstruction syndrome which is unique to CF. Ten percent of CF patients develop severe liver disease. Ten to 15% of CF patients are pancreatic sufficient and their presentation tends to be later in life, including recurrent pancreatitis, male infertility, and chronic bronchiectasis. CF is part of the newborn screen in every state in the United States and in most countries in the Western world.
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