# Pharmacological Improvement of Cystic Fibrosis Transmembrane Conductance Regulator Function Rescues Airway Epithelial Homeostasis and Host Defense in Children with Cystic Fibrosis

*PRI Rank #9 · Topics and Trends in Most Cited Pediatric health and respiratory diseases Papers, Class of 2026*

*Canonical URL: https://pri.pepkio.com/top-papers/pediatric-health-and-respiratory-diseases/2026/rank-9*

| Field | Value |
| --- | --- |
| Rank | #9 |
| 18m citations | 28 |
| Journal | American Journal of Respiratory and Critical Care Medicine |
| Year | 2024 |
| DOI | 10.1164/rccm.202310-1836oc |
| Corresponding authors | Jennifer Loske |
| Institution | Berlin Institute of Health at Charité - Universitätsmedizin Berlin, Germany |

**Ranking page:** [Topics and Trends in Most Cited Pediatric health and respiratory diseases Papers, Class of 2026](https://pri.pepkio.com/top-papers/pediatric-health-and-respiratory-diseases/2026)

**Paper link:** [10.1164/rccm.202310-1836oc](https://doi.org/10.1164/rccm.202310-1836oc)

## Topics

CFTR · CFTR · Elexacaftor/tezacaftor/ivacaftor · Elexacaftor/tezacaftor/ivacaftor · F508del mutation · nasal epithelial cells · single-cell RNA sequencing · ionocytes · Airway basal cells · club cells · goblet cells · IFN signaling · major histocompatibility complex class I · major histocompatibility complex class II · neutrophils · macrophages · Airway mucosal homeostasis · innate mucosal immunity · CFTR-positive cells · immune cell inflammatory responses

## Cite this ranking

```
Pepkio Research Index (PRI). Topics and Trends in Most Cited Pediatric health and respiratory diseases Papers, Class of 2026. https://pri.pepkio.com/top-papers/pediatric-health-and-respiratory-diseases/2026. Accessed 2026-07-14.

Zheng Su, Tinsley Li, Thematic Shifts in Early-High-Impact Cancer Genomics and Diagnostics Research: A Bibliometric and Semantic Analysis. bioRxiv 2026.07.04.736459; doi: https://doi.org/10.64898/2026.07.04.736459
```