Demographics and Clinical Characteristics of Patients at Baseline
From January 2009 to December 2020, a total of 75 patients with definitive PCD diagnoses were admitted to the Department of Respiratory Medicine, Beijing Children’s Hospital. Clinical and genetic characteristics of patients were collected and analyzed during the follow-up period. Of the total of 75 PCD patients, 63 patients received follow-up care, 7 patients were lost to follow-up, 4 refused to participate, and 1 patient died of severe complex congenital heart disease at 1 year of age. In summary, follow-up data were obtained from a total of 63 patients, of which 35 patients were males and 28 were females; the overall median duration of the follow-up period after diagnosis was 3.1 years (ranging from four months to 9.4 years) and the average age at diagnosis was 7.6 ± 3.3 years.
Age, sex and clinical characteristics between AZM-treated and AZM-untreated groups of PCD patients were very similar at baseline (Table 1, partly reported in previous studies[12]) except for FEF25-75% predicted values, which were lower in the AZM-treated group than in the AZM-untreated group (46.9 ± 20.8 VS 70.8 ± 28.0, P = 0.008, respectively).