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Hereditary Pancreatitis and Chronic Pancreatitis
Book chapter

Hereditary Pancreatitis and Chronic Pancreatitis

Aliye Uc and Michael Wilschanski
Textbook of Pediatric Gastroenterology, Hepatology and Nutrition, pp.395-403
Springer International Publishing
10/01/2015
DOI: 10.1007/978-3-319-17169-2_34

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Abstract

Chronic pancreatitis (CP) is a progressive inflammatory disorder of the pancreas characterized by irreversible structural changes and fibrotic replacement of the pancreatic parenchyma leading to permanent loss of exocrine and endocrine function (Lowe, Curr Gastroenterol Rep 6(3):240–246, 2004; Pediatric practice gastroenterology, McGraw Hill, New York, pp. 428–440, 2010; Morinville et al., J Pediatr Gastroenterol Nutr 55(3):261–265, 2012; Nydegger et al., J Gastroenterol Hepatol 21(3):499–509, 2006). The overall incidence of CP in adults is 2–4 per 100,000 persons per year and has increased over the years (Yadav et al., Am J Gastroenterol 106(12):2192–2199, 2011; Spanier et al., World J Gastroenterol. 19(20):3018–3026, 2013). The prevalence of CP in childhood is unknown, but estimated to be low. This chapter outlines the main forms of CP in children, with an emphasis on the hereditary forms.
TIGAR-O PRSS1 SPINK1 Chronic pancreatitis Pancreatitis Hereditary pancreatitis CFTR

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