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. 2018 Jan 31;9:76. doi: 10.3389/fimmu.2018.00076

Table 1.

Patient characteristics.

FPF HPSPF URel p-Value
Age (years) 63.4 ± 3.2 42.0 ± 2.9 43.3 ± 9.3 <0.001a
0.060b
0.86c
Gender (M/F) 1/4 2/10 3/1
FVC (% predicted) 89.0 ± 10.5 67.8 ± 5.7 120.5 ± 3.6 0.073a
0.037b
<0.001c
TLC (% predicted) 87.0 ± 7.6 73.6 ± 6.1 115.3 ± 5.0 0.23a
0.022b
0.002c
DLCOa (% predicted) 55.4 ± 11.8 43.2 ± 4.6 92.5 ± 8.2 0.25a
0.045b
<0.001c
HRCT scan Fibrosis Fibrosis No fibrosis

FPF, familial pulmonary fibrosis; HPSPF, Hermansky–Pudlak syndrome pulmonary fibrosis; URel, unaffected relative; M, male; F, female; FVC, forced vital capacity; TLC, total lung capacity; DLCOa, diffusion capacity (adjusted); HRCT, high-resolution computed tomography.

aFPF vs HPSPF.

bFPF vs URel.

cHPSPF vs URel.