Literature DB >> 3969086

Pancreatic fluid secretion and protein hyperconcentration in cystic fibrosis.

H Kopelman, P Durie, K Gaskin, Z Weizman, G Forstner.   

Abstract

To study pancreatic protein and water secretion in 28 patients with cystic fibrosis and 21 controls matched for pancreatic acinar function as defined by trypsin secretion, we used a quantitative-marker perfusion technique and continuous intravenous secretin-pancreozymin stimulation. Regardless of the level of pancreatic acinar function, secretions from the patients contained significantly higher concentrations of protein than those from the controls. Total protein output and albumin:protein ratios were not increased in secretions from the patients, but their fluid secretion was significantly decreased at any level of pancreatic function. A significant linear correlation was found between protein and volume secretion in the patients (r = 0.86, P less than 0.001), most of whom had a fluid output of less than 4.2 ml per kilogram of body weight per hour. No such relation was found in the control subjects, whose flow was always above 4.2 ml per kilogram per hour. We conclude that fluid secretion in patients with cystic fibrosis may be a rate-limiting factor in protein output and that a limited flow of hyperconcentrated protein secretions may predispose to protein precipitation and ductal obstruction in the pancreas.

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Year:  1985        PMID: 3969086     DOI: 10.1056/NEJM198502073120601

Source DB:  PubMed          Journal:  N Engl J Med        ISSN: 0028-4793            Impact factor:   91.245


  49 in total

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Authors:  H Kopelman
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Review 2.  Pathophysiology of the exocrine pancreas in cystic fibrosis.

Authors:  P R Durie; G G Forstner
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Review 3.  Human pancreatic exocrine response to nutrients in health and disease.

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Journal:  Gut       Date:  2005-07       Impact factor: 23.059

Review 4.  Patterns of GI disease in adulthood associated with mutations in the CFTR gene.

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Review 5.  Gastrointestinal Manifestations of Cystic Fibrosis.

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6.  Antisense oligodeoxynucleotide to the cystic fibrosis transmembrane conductance regulator inhibits cyclic AMP-activated but not calcium-activated cell volume reduction in a human pancreatic duct cell line.

Authors:  H Kopelman; C Gauthier; M Bornstein
Journal:  J Clin Invest       Date:  1993-03       Impact factor: 14.808

7.  Cystic fibrosis-related diabetes: from CFTR dysfunction to oxidative stress.

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Journal:  Clin Biochem Rev       Date:  2009-11

Review 8.  Mechanisms of bicarbonate secretion: lessons from the airways.

Authors:  Robert J Bridges
Journal:  Cold Spring Harb Perspect Med       Date:  2012-08-01       Impact factor: 6.915

9.  Molecular consequences of cystic fibrosis transmembrane regulator (CFTR) gene mutations in the exocrine pancreas.

Authors:  N Ahmed; M Corey; G Forstner; J Zielenski; L-C Tsui; L Ellis; E Tullis; P Durie
Journal:  Gut       Date:  2003-08       Impact factor: 23.059

10.  Abnormal surface liquid pH regulation by cultured cystic fibrosis bronchial epithelium.

Authors:  Raymond D Coakley; Barbara R Grubb; Anthony M Paradiso; John T Gatzy; Larry G Johnson; Sylvia M Kreda; Wanda K O'Neal; Richard C Boucher
Journal:  Proc Natl Acad Sci U S A       Date:  2003-12-10       Impact factor: 11.205

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