Chronic pancreatitis and cystic fibrosis.

Abstract:

:Recent discoveries of trypsinogen and trypsin inhibitor mutations in patients with chronic pancreatitis (CP) support the hypothesis that an inappropriate activation of pancreatic zymogens to active enzymes within the pancreatic parenchyma starts the inflammatory process. Current data suggest that CP may be inherited dominant, recessive, or complex as a result of mutations in the above mentioned or yet unidentified genes. Evaluation of patients with CP should include genetic testing. Cystic fibrosis (CF) is an autosomal recessive inherited disorder caused by mutations in the CF transmembrane conductance regulator (CFTR) gene and is characterised by pancreatic insufficiency and chronic bronchopulmonary infection. The progression and severity of pulmonary disease differs considerably between people with identical CFTR mutations and does not seem to correlate with the type or class of the CFTR mutation. The identification of further disease modifying genetic factors will increase the pathophysiological understanding and may help to identify new therapeutic targets.

journal_name

Gut

journal_title

Gut

authors

Witt H

doi

10.1136/gut.52.suppl_2.ii31

keywords:

subject

Has Abstract

pub_date

2003-05-01 00:00:00

pages

ii31-41

eissn

0017-5749

issn

1468-3288

journal_volume

52 Suppl 2

pub_type

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