Analysis of cystic fibrosis gener product (CFTR) function in patients with pancreas divisum and recurrent acute pancreatitis
Analysis of cystic fibrosis gener product (CFTR) function in patients with pancreas divisum and recurrent acute pancreatitis
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DOI：
10.1111/j.1572-0241.2004.30834.x
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发表时间：
2004-08-01
影响因子：
9.8
通讯作者：
Freedman, SD
中科院分区：
文献类型：
--
作者：
Gelrud, A;Sheth, S;Freedman, SD
背景：胰腺分裂导致急性胰腺炎复发的机制尚不清楚。囊性纤维化基因产物（CFTR）的缺失与特发性慢性胰腺炎的发生有关。本研究的目的是确定胰腺分裂和复发性急性胰腺炎（PD/RAP）患者CFTR功能是否异常。方法：共纳入69例健康对照者，12例PD/RAP患者，16例CFTR单突变的专性杂合子，95例囊性纤维化患者。通过在体鼻跨上皮电位差测试来分析CFTR功能。PD/RAP患者内镜和手术治疗后的结果进行了commonly analysed.FINDINGS：直接测量CFTR功能在鼻上皮中的反应异丙肾上腺素表明，PD/RAP的值是中间的健康对照组和囊性纤维化患者之间观察到的。PD/RAP受试者的中位值为13 mV，与健康对照组有统计学差异（22 mV，p = 0.001）和胰腺囊性纤维化（-1 mV，p < 0.0001）和胰腺功能不全（-3 mV，p < 0.0001）患者。这些结果表明，胰腺分裂患者CFTR功能障碍和复发性急性胰腺炎之间存在联系，并可能解释为什么胰腺分裂患者的子集发生复发性急性胰腺炎。
BACKGROUND: The mechanism by which pancreas divisum may lead to recurrent episodes of acute pancreatitis in a subset of individuals is unknown. Abnormalities of the cystic fibrosis gene product (CFTR) have been implicated in the genesis of idiopathic chronic pancreatitis. The aim of this study was to determine if CFTR function is abnormal in patients with pancreas divisum and recurrent acute pancreatitis (PD/RAP).METHODS: A total of 69 healthy control subjects, 12 patients with PD/RAP, 16 obligate heterozygotes with a single CFTR mutation, and 95 patients with cystic fibrosis were enrolled. CFTR function was analyzed by nasal transepithelial potential difference testing in vivo. The outcomes of the PD/RAP patients following endoscopic and surgical treatments were concomitantly analyzed.FINDINGS: Direct measurement of CFTR function in nasal epithelium in response to isoproterenol demonstrated that the values for PD/RAP were intermediate between those observed for healthy controls and cystic fibrosis patients. The median value was 13 mV for PD/RAP subjects, which was statistically different from healthy controls (22 mV, p = 0.001) and cystic fibrosis pancreatic sufficient (-1 mV, p < 0.0001) and pancreatic insufficient (-3 mV, p < 0.0001) patients.INTERPRETATIONS: These results suggest a link between CFTR dysfunction and recurrent acute pancreatitis in patients with pancreas divisum and may explain why a subset of patients with pancreas divisum develops recurrent acute pancreatitis.