Cystic Fibrosis Transmembrane Conductance Regulator Modulator Use Is Associated With Reduced Pancreatitis Hospitalizations in Patients With Cystic Fibrosis.
Adolescent
Adult
Child
Child, Preschool
Cross-Over Studies
Cystic Fibrosis
/ complications
Cystic Fibrosis Transmembrane Conductance Regulator
/ pharmacology
Female
Hospitalization
/ trends
Humans
Incidence
Infant
Infant, Newborn
Male
Pancreatitis
/ epidemiology
Prospective Studies
United States
/ epidemiology
Young Adult
Journal
The American journal of gastroenterology
ISSN: 1572-0241
Titre abrégé: Am J Gastroenterol
Pays: United States
ID NLM: 0421030
Informations de publication
Date de publication:
01 12 2021
01 12 2021
Historique:
received:
12
01
2021
accepted:
09
09
2021
pubmed:
20
10
2021
medline:
22
2
2022
entrez:
19
10
2021
Statut:
ppublish
Résumé
Acute pancreatitis (AP) occurs among patients with pancreas-sufficient cystic fibrosis (PS-CF) but is reportedly less common among patients with pancreas-insufficient cystic fibrosis (PI-CF). The incidence of AP may be influenced by cystic fibrosis transmembrane conductance regulator (CFTR) modulator use. We hypothesized that CFTR modulators would reduce AP hospitalizations, with the greatest benefit in PS-CF. MarketScan (2012-2018) was queried for AP hospitalizations and CFTR modulator use among patients with CF. Multivariable Poisson models that enabled crossover between CFTR modulator treatment groups were used to analyze the rate of AP hospitalizations on and off therapy. Pancreas insufficiency was defined by the use of pancreas enzyme replacement therapy. A total of 10,417 patients with CF were identified, including 1,795 who received a CFTR modulator. AP was more common in PS-CF than PI-CF (2.9% vs 0.9%, P = 0.007). Overall, the observed rate ratio of AP during CFTR modulator use was 0.33 (95% confidence interval [CI] 0.10, 1.11, P = 0.07) for PS-CF and 0.38 (95% CI 0.16, 0.89, P = 0.03) for PI-CF, indicating a 67% and 62% relative reduction in AP hospitalizations, respectively. In a subset analysis of 1,795 patients who all had some CFTR modulator use, the rate ratio of AP during CFTR modulator use was 0.36 (95% CI 0.13, 1.01, P = 0.05) for PS-CF and 0.53 (95% CI 0.18, 1.58, P = 0.26) for PI-CF. CFTR modulator use is associated with a reduction in AP hospitalizations among patients with CF. These observational data support the prospective study of CFTR modulators to reduce AP hospitalizations among patients with CF.
Identifiants
pubmed: 34665155
doi: 10.14309/ajg.0000000000001527
pii: 00000434-202112000-00025
pmc: PMC8900539
mid: NIHMS1781590
doi:
Substances chimiques
Cystic Fibrosis Transmembrane Conductance Regulator
126880-72-6
Types de publication
Journal Article
Multicenter Study
Research Support, N.I.H., Extramural
Langues
eng
Sous-ensembles de citation
IM
Pagination
2446-2454Subventions
Organisme : NCATS NIH HHS
ID : UL1 TR002733
Pays : United States
Informations de copyright
Copyright © 2021 by The American College of Gastroenterology.
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