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Diffuse microcystic pancreatic enlargement in a cystic fibrosis patient causing severe gastrointestinal symptoms and successfully treated by total pancreatectomy

Diffuse microcystic pancreatic enlargement in a cystic fibrosis patient causing severe gastrointestinal symptoms and successfully treated by total pancreatectomy
Diffuse microcystic pancreatic enlargement in a cystic fibrosis patient causing severe gastrointestinal symptoms and successfully treated by total pancreatectomy
Excerpt
The typical morphologic changes within the pancreas of patients with cystic fibrosis are inter- and intralobular fibrosis and destruction of the pancreatic parenchyma. The small ducts become obstructed by secretions and cellular debris, leading to formation of microcysts. Eventually, the pancreas becomes shrunken and fibrosed, with fatty infiltration of the parenchyma(1-3). On rare occasions, it contains epithelia-lined macrocysts and calcification (4). The pancreatic changes in cystic fibrosis rarely cause abdominal pain except in small numbers of patients who suffer acute pancreatitis. In most of these cases, patients have sufficient pancreatic function(5,6). We report a child with pancreatic insufficiency with cystic fibrosis, in whom severe gastrointestinal symptoms developed, associated with a previously undescribed complication of the pancreas.
0277-2116
454-457
Dewar, Ann L.
73302ecb-a869-49aa-b9b2-3422b3176d37
Connett, Gary J.
55d5676c-90d8-46bf-a508-62eded276516
Dewar, Ann L.
73302ecb-a869-49aa-b9b2-3422b3176d37
Connett, Gary J.
55d5676c-90d8-46bf-a508-62eded276516

Dewar, Ann L. and Connett, Gary J. (1998) Diffuse microcystic pancreatic enlargement in a cystic fibrosis patient causing severe gastrointestinal symptoms and successfully treated by total pancreatectomy. Journal of Pediatric Gastroenterology & Nutrition, 26 (4), 454-457. (doi:10.1097/00005176-199804000-00017).

Record type: Article

Abstract

Excerpt
The typical morphologic changes within the pancreas of patients with cystic fibrosis are inter- and intralobular fibrosis and destruction of the pancreatic parenchyma. The small ducts become obstructed by secretions and cellular debris, leading to formation of microcysts. Eventually, the pancreas becomes shrunken and fibrosed, with fatty infiltration of the parenchyma(1-3). On rare occasions, it contains epithelia-lined macrocysts and calcification (4). The pancreatic changes in cystic fibrosis rarely cause abdominal pain except in small numbers of patients who suffer acute pancreatitis. In most of these cases, patients have sufficient pancreatic function(5,6). We report a child with pancreatic insufficiency with cystic fibrosis, in whom severe gastrointestinal symptoms developed, associated with a previously undescribed complication of the pancreas.

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Published date: 1 April 1998

Identifiers

Local EPrints ID: 420694
URI: https://eprints.soton.ac.uk/id/eprint/420694
ISSN: 0277-2116
PURE UUID: 14c9dd70-7a17-4078-8524-dd5b3c47cbd4
ORCID for Gary J. Connett: ORCID iD orcid.org/0000-0003-1310-3239

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Date deposited: 11 May 2018 16:30
Last modified: 14 Mar 2019 01:22

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Contributors

Author: Ann L. Dewar
Author: Gary J. Connett ORCID iD

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