Inherited Causes of Exocrine Pancreatic Dysfunction

The exocrine pancreas is functionally immature at birth. Protease function is probably adequate, but lipase activity approximates 5% to 10% of adult values in the newborn and remains low i...

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Main Author: Peter R Durie
Format: Article
Language:English
Published: Wiley 1997-01-01
Series:Canadian Journal of Gastroenterology
Online Access:http://dx.doi.org/10.1155/1997/137618
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author Peter R Durie
author_facet Peter R Durie
author_sort Peter R Durie
collection DOAJ
description The exocrine pancreas is functionally immature at birth. Protease function is probably adequate, but lipase activity approximates 5% to 10% of adult values in the newborn and remains low in infancy. Pancreatic amylase secretion is essentially absent at birth and remains low through the first years of life. Functional disturbances of the exocrine pancreas are less frequent in childhood than in adult life. Causes of pancreatic dysfunction in childhood can be divided in two general categories: hereditary conditions, which directly affect the pancreas; and acquired disorders, in which loss of pancreatic function is a secondary phenomenon. Most inherited causes of pancreatic dysfunction are due to a generalized disorder. Cystic fibrosis is, by far, the most common inherited cause of disturbed pancreatic function among Caucasian children. All other inherited causes of exocrine pancreatic dysfunction (eg, Johanson-Blizzard syndrome) are uncommon or rare.
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series Canadian Journal of Gastroenterology
spelling doaj-art-98d47d41b66c4675b7f19a7f2ab653302025-02-03T05:53:03ZengWileyCanadian Journal of Gastroenterology0835-79001997-01-0111214515210.1155/1997/137618Inherited Causes of Exocrine Pancreatic DysfunctionPeter R Durie0Department of Pediatrics, University of Toronto, CanadaThe exocrine pancreas is functionally immature at birth. Protease function is probably adequate, but lipase activity approximates 5% to 10% of adult values in the newborn and remains low in infancy. Pancreatic amylase secretion is essentially absent at birth and remains low through the first years of life. Functional disturbances of the exocrine pancreas are less frequent in childhood than in adult life. Causes of pancreatic dysfunction in childhood can be divided in two general categories: hereditary conditions, which directly affect the pancreas; and acquired disorders, in which loss of pancreatic function is a secondary phenomenon. Most inherited causes of pancreatic dysfunction are due to a generalized disorder. Cystic fibrosis is, by far, the most common inherited cause of disturbed pancreatic function among Caucasian children. All other inherited causes of exocrine pancreatic dysfunction (eg, Johanson-Blizzard syndrome) are uncommon or rare.http://dx.doi.org/10.1155/1997/137618
spellingShingle Peter R Durie
Inherited Causes of Exocrine Pancreatic Dysfunction
Canadian Journal of Gastroenterology
title Inherited Causes of Exocrine Pancreatic Dysfunction
title_full Inherited Causes of Exocrine Pancreatic Dysfunction
title_fullStr Inherited Causes of Exocrine Pancreatic Dysfunction
title_full_unstemmed Inherited Causes of Exocrine Pancreatic Dysfunction
title_short Inherited Causes of Exocrine Pancreatic Dysfunction
title_sort inherited causes of exocrine pancreatic dysfunction
url http://dx.doi.org/10.1155/1997/137618
work_keys_str_mv AT peterrdurie inheritedcausesofexocrinepancreaticdysfunction