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CFTR调节剂时代囊性纤维化中的外分泌胰腺:一篇迷你综述

The Exocrine Pancreas in Cystic Fibrosis in the Era of CFTR Modulation: A Mini Review.

作者信息

McKay Isabelle R, Ooi Chee Y

机构信息

Wagga Wagga Base Hospital, Wagga Wagga, NSW, Australia.

School of Clinical Medicine, Discipline of Paediatrics and Child Health, Randwick Clinical Campus, University of New South Wales (UNSW) Medicine and Health, University of New South Wales, Sydney, NSW, Australia.

出版信息

Front Pediatr. 2022 Jun 27;10:914790. doi: 10.3389/fped.2022.914790. eCollection 2022.

Abstract

Cystic fibrosis (CF) is a common disorder of autosomal recessive inheritance, that once conferred a life expectancy of only a few months. Over recent years, significant advances have been made to CF therapeutic approaches, changing the face of the disease, and facilitating the partial restoration of pancreatic function. This mini review summarizes the current landscape of exocrine pancreatic management in CF and explores areas for future direction and development.

摘要

囊性纤维化(CF)是一种常见的常染色体隐性遗传疾病,曾经患者的预期寿命仅有几个月。近年来,CF的治疗方法取得了重大进展,改变了这种疾病的面貌,并促进了胰腺功能的部分恢复。本综述总结了CF患者外分泌性胰腺管理的现状,并探讨了未来的发展方向和领域。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a23e/9271761/d8dd822844d0/fped-10-914790-g0001.jpg

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