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. 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.

Background: Chronic rhinosinusitis with nasal polyps (CRSwNP) in children is a clinically significant inflammatory disorder characterized by persistent symptoms and complex underlying mechanisms. This study used multi-omics approaches to investigate potential microbial and metabolic associations in pediatric CRSwNP. Methods: Nasal secretions from 20 children with CRSwNP and 19 healthy controls were analyzed using metagenomics, untargeted metabolomics, and proteomics. Results: CRSwNP patients showed higher microbial diversity and altered microbial communities, with increased Streptococcus abundance. Metabolomic sequencing revealed that 13′-Hydroxy-alpha-tocopherol was significantly upregulated in the CRSwNP group and exhibited a positive correlation with the abundance of Streptococcus. Proteomic sequencing revealed that proteins involved in glutathione metabolism were significantly downregulated in the CRSwNP group, with GCLM and GGCT showing a significant negative correlation with 13′-Hydroxy-alpha-tocopherol. Conclusion: These associative findings suggest potential links among Streptococcus, 13′-Hydroxy-α-tocopherol, and glutathione metabolism, indicating that oxidative stress–related imbalance may contribute to pediatric CRSwNP. These results provide preliminary evidence that 13′-Hydroxy-α-tocopherol may serve as a potential biomarker for pediatric CRSwNP.

Median palatine cysts (MPCs) and nasopalatine duct cysts (NPDCs) are rare non-odontogenic cystic lesions in the pediatric population. Due to their low incidence and often asymptomatic nature in children, these cysts are frequently overlooked or misdiagnosed. To the best of our knowledge, few comparative pediatric cases have been reported. The novelty lies in the detailed histopathological differentiation and the analysis of their impact on maxillofacial development, providing a crucial clinical reference for early intervention. In this report, two pediatric patients presented with similar clinical manifestations and obvious dental malalignment. Initial imaging suggested NPDCs in both cases. Both patients underwent complete cyst removal via endoscopic nasal surgery. Postoperative histopathological examination confirmed the distinct diagnoses: one as an MPC and the other as an NPDC. The primary histopathological differentiator was the composition of the fibrous cyst wall; the NPDC contained specialized structures (blood vessels, nerves, and mucous glands), which were absent in the MPC. At the two-year follow-up, both patients showed favorable maxillofacial development and no signs of recurrence. These cases illustrate that while MPCs and NPDCs may share clinical similarities, they possess distinct histopathological characteristics. The endoscopic approach appears to be a feasible surgical option in these cases. Early and accurate differential diagnosis can aid in surgical planning and may help mitigate potential impacts on pediatric maxillofacial development, offering useful insights for otolaryngologists and pediatric dentists.

Chronic Rhinosinusitis is a common disease in children. The main function of CFTR is to maintain the thickness of the mucous layer on the surface of the nasal mucosa. CFTR disease-causing variant can cause CFTR protein dysfunction and induce or aggravate chronic infection. However, the carrying status of the CFTR variants in the Chinese population is not clear. To study the frequency and variants of CFTR in Chinese children with CRS and to analyze the CFTR variants and the clinical characteristics and susceptibility to CRS. Whole Exome Sequencing was performed to analyze the CFTR genes in a total of 106 CRS children from the Chinese mainland area. The CFTR variants, frequency and clinical data were summarized and analyzed. A total of 31 CFTR variants were detected, of which the carrying rate of 7 sites was significantly higher than that of the population database. 88 patients carried more than 2 variants. 37 people carried variants (MAF < 0.05), of which 91.89% had a history of recurrent upper respiratory infections, 16 had nasal polyps, 5 had bronchiectasis, and 1 was diagnosed with CF-related disorders. The carrying rate of CFTR variants in Chinese CRS children increased, and the highest rates of variants (MAF < 0.05) are p.I556V, p. E217G, c.1210-12[T]. Carrying multiple CFTR variants, especially p.E217G, p.I807 M, p.V920L and c.1210-12[T] may lead to increased susceptibility to CRS. There are CF-related disorders in patients with CRS.