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.