囊性纤维化查,评估和肝胆病的管理共识建议 建议

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Updated: Jul 11, 2025

Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study
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Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study

Published on: April 11, 2018

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Zachary M Sellers1, David N Assis2, Shruti M Paranjape3

  • 1Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Stanford University, Palo Alto, California, USA.

Hepatology (Baltimore, Md.)
|November 7, 2023
PubMed
概括

对囊性纤维化 (CF) 相关肝病的最新指南提供了改进的查,监测和治疗策略. 这些基于证据的建议旨在通过早期检测和治疗肝胆道并发症来改善患者的治疗结果.

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Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
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Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
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