CFTR Modulator Use Is Associated with Higher Hemoglobin Levels in Individuals with Cystic Fibrosis.

ABSTRACT:

Rationale

Understanding how cystic fibrosis transmembrane conductance regulator (CFTR) modulators influence comorbid conditions like anemia is of great interest to the cystic fibrosis community.

Objectives

To test the hypothesis that CFTR modulators are associated with higher hemoglobin (Hgb) levels.

Methods

Annualized Hgb and other laboratory, demographic, and anthropometric data were abstracted from the U.S. CF Foundation Patient Registry for adult and pediatric registrants before and after therapy with ivacaftor (IVA) or lumacaftor/ivacaftor (LUM/IVA) between January 2010 and December 2016. Univariate and multivariate linear mixed models were used to examine the effect of IVA on Hgb in patients with G551D-CFTR, and the effect of LUM/IVA on Hgb in F508del-CFTR homoz

SUBMITTER: Gifford AH 

PROVIDER: S-EPMC6394125 | biostudies-literature | 2019 Mar

REPOSITORIES: biostudies-literature