Guy-Crotte O, Carrère J, Figarella C
Groupe de Recherche sur les Glandes Exocrines, Faculté de Médecine, Marseille, France.
Eur J Gastroenterol Hepatol. 1996 Aug;8(8):755-9.
Pancreatic dysfunction in cystic fibrosis (CF) begins in utero and, at birth, in most cases, cystic fibrosis is characterized by an elevated level of serum immunoreactive trypsin (IRT). If most patients with CF typically present insufficient pancreatic exocrine function, 10-15% of CF patients have pancreatic sufficiency and this status is genetically determined by one or two 'mild' mutations in CF transmembrane conductance regulator (CFTR). However, with age, these patients can develop pancreatic insufficiency.
囊性纤维化（CF）患者的胰腺功能障碍始于子宫内，并且在出生时，大多数情况下，囊性纤维化的特征是血清免疫反应性胰蛋白酶（IRT）水平升高。如果大多数CF患者通常存在胰腺外分泌功能不足，那么10%-15%的CF患者具有胰腺功能正常，这种状态由囊性纤维化跨膜传导调节因子（CFTR）中的一个或两个“轻度”突变在基因上决定。然而，随着年龄增长，这些患者会出现胰腺功能不全。
