Ozen Alahdab Yesim, Duman Deniz G
Department of Gastroenterology, Marmara University School of Medicine, Istanbul, Turkey -
Minerva Med. 2016 Dec;107(6):427-436. Epub 2016 Jul 14.
Cystic fibrosis (CF) is an important genetic disorder that usually manifests in early childhood. Better understanding and improved care of CF patients in our current practice with pancreatic enzyme replacement therapy and nutritional support has enabled physicians to see them more frequently from any age groups. After the lungs, pancreas is the most important organ involved with CF. Its most frequently affected component is the exocrine pancreas while endocrine functions may also be deranged. Parenchymal damage as acute or chronic pancreatitis may also develop. Herein, we aimed to outline the types of the disease, clinical picture, diagnosis and genetic aspect of the CF linked with the pancreatic involvement.
囊性纤维化（CF）是一种重要的遗传性疾病，通常在儿童早期出现。在我们目前的实践中，通过胰腺酶替代疗法和营养支持，对CF患者有了更好的理解和改善的护理，这使得医生能够更频繁地诊治来自任何年龄组的患者。除肺部外，胰腺是与CF相关的最重要器官。其最常受影响的部分是外分泌胰腺，而内分泌功能也可能紊乱。也可能发生实质性损伤，如急性或慢性胰腺炎。在此，我们旨在概述与胰腺受累相关的CF的疾病类型、临床表现、诊断和遗传学方面。
