Article
Longitudinal Lung Volume Changes by Ultrastructure and Genotype in Primary Ciliary Dyskinesia.
Annals of the American Thoracic Society - 1 Jun 2021
Pifferi Massimo, Bush Andrew, Mulé Giuseppe, Gracci Serena, Fonnesu Rossella, Michelucci Angela, Cangiotti Angela, Caligo Maria Adelaide, Miccoli Mario, Boner Attilio L, Peroni Diego
Abstract excerpt
Rationale: Genotype-phenotype relationships are emerging in primary ciliary dyskinesia (PCD), but little is known about lung volume changes over time. Objectives: To investigate the evolution of static lung volumes with ultrastructural defects, gene mutations, body mass index, and specific infections in PCD. Methods: Prospective, longitudinal, single-center study in children and adults evaluated twice yearly for...
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