Skip to main content
. 2022 Mar 27;190(1):89–101. doi: 10.1002/ajmg.c.31968

TABLE 2.

Clinical features of adults and children in this cohort

Adults Children
n/mean (%)/±SD n/mean (%)/±SD
Findings chest imaging a N = 16 N = 16
Atelectasis 5 (31%) 7 (44%)
Infiltration 0 (0%) 2 (13%)
Bronchiectasis 13 (81%) 3 (19%)
Other 2 (13%) 12 (75%)
Normal 2 (13%) 2 (13%)
BMI N = 15 N = 13
kg/m2 22.7 ±3.8 19.4 ±3.2
z‐score b 0.20 ±1.1 −0.14 ±0.9
Underweight 0 (0%) 0 (0%)
Normal 14 (93%) 13 (100%)
Overweight 1 (7%) 0 (0%)
FEV1 N = 15 N = 13
L 2.8 ±0.9 3.1 ±1.1
%predicted 77.9 ±14.8 88.8 ±13.8
z‐score −1.7 ±1.0 −0.9 ±1.1
FVC N = 15 N = 13
L 3.8 ±1.0 4.3 ±1.5
%predicted 90.7 ±15.0 105.2 ±9.3
z‐score −0.7 ±1.1 0.4 ±0.8

Note: Data are presented as n(%) or mean ± SD.

Abbreviations: BMI, body mass index; FEV1, forced expiratory volume in 1 s; FVC, forced vital capacity.

a

Patients can be part of multiple subgroups.

b

z‐scores based on reference values from the World Health Organization, adjusted for age and sex.