Acquired CFTR dysfunction and dense distribution of ionocytes in nasal mucosa of children with CRS

CRS患儿获得性CFTR功能障碍及鼻黏膜离子细胞密集分布

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作者:Yang Han #, Chao Jia #, Tieshan Wang #, Pengpeng Wang, Wenjing Liu, Yu Qin, Siyu Cai, Xiaojian Yang, Wei Zhang, Yuwei Liu, Xiao Xiao, Lejian He, Wentong Ge, Xin Ni

Background

Ionocytes are rare cells in airway epithelium characterized by a high expression of CFTR. Objectives: To investigate the morphology and distribution of ionocytes and the function of CFTR in the nasal mucosal epithelium of children.

Conclusions

The number of ionocytes and the function of CFTR in nasal mucosa of CRS patients decreased. With the expansion of inflammation, CFTR and ionocytes showed more obvious dense distribution. Some ionocytes lost the expression of CFTR and did not show the "tadpole" shape, which may be related to the occurrence of polyps.

Methods

The exfoliated cells of nasal mucosa from 101 children were detected using flow cytometry to analyze the number of ionocytes and CFTR and the difference of CFTR function. Nasal mucosa and polyps were collected from 10 children with CRSwNP. The RNAscope of FOXI1 and CFTR was detected in pathological paraffin sections. The expression and distribution of ionocytes and CFTR in nasal mucosa and polyp epithelium were observed.

Results

In CRS patients, the number of ionocytes in the nasal epithelium was lower and the number of ionocytes that did not express CFTR was higher, and the function of CFTR was also decreased. The expression of CFTR in the nasal mucosa of CRS showed the characteristics of local dense distribution and increased as the inflammation expanded. The ionocytes were "tadpole-shaped" in the epithelium and gathered in the area of high CFTR expression, the intracellular CFTR was expanded in clusters. Ionocytes that did not express CFTR was more common in the nasal polyps. Conclusions: The number of ionocytes and the function of CFTR in nasal mucosa of CRS patients decreased. With the expansion of inflammation, CFTR and ionocytes showed more obvious dense distribution. Some ionocytes lost the expression of CFTR and did not show the "tadpole" shape, which may be related to the occurrence of polyps.

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