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囊性纤维化中的胰液分泌与蛋白质高浓度

Pancreatic fluid secretion and protein hyperconcentration in cystic fibrosis.

作者信息

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

出版信息

N Engl J Med. 1985 Feb 7;312(6):329-34. doi: 10.1056/NEJM198502073120601.

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.

摘要

为研究28例囊性纤维化患者及21例根据胰蛋白酶分泌定义的胰腺腺泡功能相匹配的对照者的胰腺蛋白质和水分泌情况,我们采用了定量标记灌注技术及持续静脉注射促胰液素-胰酶泌素刺激法。无论胰腺腺泡功能水平如何,患者的分泌物中蛋白质浓度均显著高于对照者。患者分泌物中的总蛋白输出量及白蛋白与蛋白质比值并未增加,但在任何胰腺功能水平下,其液体分泌均显著减少。患者的蛋白质分泌与液体分泌之间存在显著的线性相关性(r = 0.86,P < 0.001),其中大多数患者每千克体重每小时的液体输出量少于4.2毫升。在对照者中未发现此类关系,其液体流量始终高于每千克每小时4.2毫升。我们得出结论,囊性纤维化患者的液体分泌可能是蛋白质输出的限速因素,且高浓度蛋白质分泌物的有限流量可能易导致胰腺内蛋白质沉淀和导管阻塞。

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