Pediatr Pulmonol. 2026 Jul;61(7):e71740. doi: 10.1002/ppul.71740.
ABSTRACT
BACKGROUND: Advanced cystic fibrosis lung disease (ACFLD) remains a major cause of morbidity, particularly in individuals requiring lung transplantation. Gastrointestinal-related aspiration (GRASP), including reflux and motility disorders, may contribute to adverse respiratory outcomes, yet diagnostic and management strategies vary across specialties.
OBJECTIVE: To evaluate practice patterns in foregut testing and pharmacologic management of GRASP among pulmonologists and gastroenterologists caring for individuals with ACFLD, with and without transplantation.
METHODS: A cross-sectional survey of clinicians at cystic fibrosis centers was distributed via a national listserv. Respondents involved in ACFLD care were included. Descriptive statistics were generated, and comparisons by specialty were performed using Fisher’s exact and Mann-Whitney U tests (α = 0.05).
RESULTS: Forty-five complete responses were analyzed (82% pulmonologists, 18% gastroenterologists). Eighty percent reported access to gastrointestinal motility testing. Overall use of reflux, motility, and gastric emptying testing was similar between specialties; however, modality selection differed. Gastroenterologists favored pH multichannel intraluminal impedance (75%) and BRAVO capsule testing (25%), while pulmonologists demonstrated heterogeneous practices and frequently deferred testing (37.8%). High-resolution manometry was universally preferred by gastroenterologists but variably used by pulmonologists. Acid suppression was widely utilized, with proton pump inhibitors most common, though pulmonologists showed greater variability, particularly post-transplant.
CONCLUSIONS: Substantial variability exists in GRASP evaluation and management in ACFLD. Standardized, multidisciplinary approaches may improve diagnostic consistency and optimize outcomes, especially in the transplant population.
PMID:42473831 | DOI:10.1002/ppul.71740
