Article
Primary Ciliary Dyskinesia: Longitudinal Study of Lung Disease by Ultrastructure Defect and Genotype.
American journal of respiratory and critical care medicine - 15 Jan 2019
Davis Stephanie D, Rosenfeld Margaret, Lee Hye-Seung, Ferkol Thomas W, Sagel Scott D, Dell Sharon D, Milla Carlos, Pittman Jessica E, Shapiro Adam J, Sullivan Kelli M, Nykamp Keith R, Krischer Jeffrey P, Zariwala Maimoona A, Knowles Michael R, Leigh Margaret W
Abstract excerpt
RATIONALE: In primary ciliary dyskinesia, factors leading to disease heterogeneity are poorly understood. OBJECTIVES: To describe early lung disease progression in primary ciliary dyskinesia and identify associations between ultrastructural defects and genotypes with clinical phenotype. METHODS: This was a prospective, longitudinal (5 yr), multicenter, observational study. Inclusion criteria were less than 19...
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