Table 1.
Sibship Number | Patient | Exons Involved | Age at Peak FVC (y) | Peak FVC Level (L) |
---|---|---|---|---|
Discordant Sibships | ||||
1 | 1 | 51 | 13.42 | 1.97 |
2 | 14.17 | 2.88 | ||
3 | 14.5 | 2.71 | ||
2 | 1 | 44 | 17 | 3.62 |
2 | 11.33 | 2.67 | ||
3 | 1 | 31 | 18.33 | 3.38 |
2 | 18.17 | 4.6 | ||
Concordant Sibships | ||||
4 | 1 | 45–50 | 14.67 | 2.48 |
2 | 15.08 | 2.81 | ||
5 | 1 | 52 | 11.08 | 1.5 |
2 | 13.08 | 2.04 | ||
6 | 1 | 44 | 12.42 | 1.4 |
2 | 12.83 | 1.44 | ||
7 | 1 | 56 | 14 | 2.02 |
2 | 14.5 | 1.67 |
Mean age at peak FVC +/− S.D. = 14.31 +/− 2.18 years
Abbreviation: FVC, forced vital capacity.