Sinaasappel M
Department of Paediatrics, University Hospital Rotterdam/Sophia Children's Hospital, Netherlands.
Neth J Med. 1992 Oct;41(3-4):110-4.
Cystic fibrosis (CF) is the most frequent inheritable disease with a lethal course. One of the major problems of the disease is malabsorption and malnutrition, due to pancreatic insufficiency which is already present at birth in more than 85% of the patients. Characteristically the mucoid secretion products of the epithelial tissues in lung, pancreas, liver and intestine have a high viscosity. The pathophysiology is characterized by obstruction of these organs with secondary damage and finally destruction. For a long period intestinal obstruction syndromes in CF were ascribed only to the pancreatic insufficiency. Malabsorption is not only caused by enzyme deficiency but is also related to transport processes to the surface of the enterocytes. This indicates that the intestinal disorders in CF are partly the result of mucoid plugging and not only of pancreatic insufficiency. Recently in vitro studies have shown a blockade of secretion through chloride channels in the mucosal membrane of CF tissues. In vivo measurements of chloride fluxes in the rectum showed a disturbed regulation in CF patients. The high viscosity of the mucus and plugging is directly related to the diminished chloride secretion. So it is postulated that the abnormal chloride secretion is responsible for the intestinal obstruction and partially also for the malabsorption.
囊性纤维化（CF）是最常见的具有致命病程的遗传性疾病。该疾病的主要问题之一是吸收不良和营养不良，这是由于超过85%的患者在出生时就存在胰腺功能不全。其特征是肺、胰腺、肝脏和肠道上皮组织的黏液分泌产物具有高黏性。病理生理学的特征是这些器官发生梗阻，继而造成损害，最终导致破坏。长期以来，CF中的肠梗阻综合征仅被归因于胰腺功能不全。吸收不良不仅由酶缺乏引起，还与向肠上皮细胞表面的转运过程有关。这表明CF中的肠道疾病部分是黏液阻塞的结果，而不仅仅是胰腺功能不全所致。最近的体外研究表明，CF组织黏膜膜中的氯离子通道分泌受阻。对直肠中氯离子通量的体内测量显示，CF患者存在调节紊乱。黏液的高黏性和阻塞与氯离子分泌减少直接相关。因此推测，异常的氯离子分泌是肠梗阻的原因，部分也是吸收不良的原因。
