Patients
A total of seven CF patients which belong to three families (Family 1: F1- P1, F1-P2, Family 2: F2-P1, F2-P2, Family 3: F3-P1, F3-P2, F3-P3) and three non-CF control subjects (C1, C2, C3) were diagnosed at Hacettepe University Faculty of Medicine Department of Pediatric Pulmonology. Ethical approval was obtained from Hacettepe University Non-Interventional Clinical Research Ethics Board (Project No: TYL-2019-17977). Informed written consent was obtained from the parents of all patients and non-CF control subjects. Clinical severity of siblings were determined according to recurrent lung infection, hepatic involvement, FEV1% (predicted) and CFRD parameters. Consanguinity was detected in two families (F2 and F3). The detailed clinical parameters of siblings were described in Table 1. The study also included three age matched non-CF control subjects who have no chronic disease history and no respiratory symptoms. They also have normal physical examination and do not take medications.
TABLE 1 Clinical and genetic parameters of siblings in three CF families.