Hereditary Exocrine Disorders
There are a number of inherited disorders that can affect the exocrine and/or endocrine function of the pancreas. These include inborn errors of metabolism such as hyperlipidemia, glycogen storage disorders, or maple syrup urine disease, which can be associated with pancreatitis. The autosomal dominant disorder hereditary chronic pancreatitis is discussed in detail in this chapter, together with cystic fibrosis, which may only present in late adolescence or early adulthood, and hereditary hemochromatosis. There are also a number of inherited syndromes that are associated with an increased risk of pancreatic cancer, which are briefly mentioned. Familial pancreatic cancer is discussed in more detail, particularly with reference to the role of screening in high-risk individuals.
KeywordsPancreatic Cancer Cystic Fibrosis Chronic Pancreatitis Cystic Fibrosis Transmembrane Conductance Regulator Lynch Syndrome
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