PMC:7274950 / 6713-8204
Annnotations
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In the same study, they investigated whether factors related to age, sex, tobacco, phenotype, residual α-gal activity, age at ERT initiation, Mainz Severity Score Index (MSSI) and lyso-Gb3 levels in plasma may affect change in FEV1 and FEV1/FVC over time. Median spirometric follow-up time was 7.7 years. When considering z-scores, 27 patients (51%) developed airflow limitation over time. Factors including male sex, active tobacco or past history of smoking, cardiac involvement and increased patient age at ERT initiation were associated with faster FEV1 decline in the univariate analysis. 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