Abstract

[...]significant obstruction (>50% of diameter) was identified along the main trachea and left bronchus during expiration, and some branches of the right bronchus also demonstrated obstruction (basal segment of the right lower lobe) [Figure 1A–1C]. The two mutations of c.6049C>G (Mutation Taster 0.944) and c.3754C>T (Mutation Taster 1.0) were both predicted to have protein-damaging and disease-causing effects. [...]for this case, the compound heterozygous mutations on PIEZO2 would lead to the loss-of-function of this gene, indicating insensitivity to mechanical stress-inducing cartilage maturation disorder. [...]the inactivity of the PIEZO2 channel would lead to deformation of the mechanical structure of chondrocyte-enriched tissues.

Details

Title
A rare case with compound heterozygous mutations of piezo-type mechanosensitive ion channel component 2 (PIEZO2) induced tracheobronchomalacia
Author
Wu, Juan 1 ; Lin, Zhong 2 ; Chen, Ting 2 ; Qiu, Li 2 ; Yi-Fei, Li 3 

 Key Laboratory of Birth Defects and Related Diseases of Women and Children of MOE, West China Second University Hospital, Sichuan University, Chengdu, Sichuan 610041, China; Department of Nursing, West China Second University Hospital, Sichuan University, Chengdu, Sichuan 610041, China 
 Department of Pediatrics, West China Second University Hospital, Sichuan University, Chengdu, Sichuan 610041, China 
 Key Laboratory of Birth Defects and Related Diseases of Women and Children of MOE, West China Second University Hospital, Sichuan University, Chengdu, Sichuan 610041, China; Department of Pediatrics, West China Second University Hospital, Sichuan University, Chengdu, Sichuan 610041, China 
Pages
1254-1256
Section
Correspondence
Publication year
2021
Publication date
May 2021
Publisher
Lippincott Williams & Wilkins Ovid Technologies
ISSN
03666999
Source type
Scholarly Journal
Language of publication
English
ProQuest document ID
2849448044
Copyright
Copyright © 2021 The Chinese Medical Association, produced by Wolters Kluwer, Inc. under the CC-BY-NC-ND license. This work is published under http://creativecommons.org/licenses/by-nc-nd/4.0 (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.