Table 1.
—Pulmonary Function Test Results Over Time in the Proband
| Age, y | FEV1 (%)a | FVC (%)a | FEV1/FVC | TLC (%)a | FRCpleth (%)a | RV (%)a | Dlco, mL/min/mmHg (%) |
| 7.5 | 0.54 (48) | 0.66 (43) | 0.82 | … | … | … | … |
| 8.4 | 0.60 (46) | 0.68 (41) | 0.88 | … | … | … | 16.1 (79) |
| 8.9 | 0.75 (51) | 0.84 (47) | 0.90 | … | … | … | … |
| 9.9 | 0.65 (38) | 0.74 (37) | 0.89 | 2.59 (105) | … | 1.85 (366) | 10.8 (62) |
| 10.3 | 0.71 (41) | 0.87 (44) | 0.81 | 2.56 (102) | … | 1.60 (310) | … |
| 10.6 | 0.88 (49) | 0.91 (44) | 0.97 | 2.60 (102) | … | 1.69 (320) | … |
| 10.9 | 0.92 (49) | 1.11 (52) | 0.83 | 2.72 (102) | … | 1.59 (289) | … |
| 11.9 | 0.89 (48) | 0.94 (45) | 0.89 | 2.93 (97) | 2.11 (142) | 1.90 (243) | … |
| 12.9 | 0.98 (48) | 1.04 (45) | 0.94 | 3.12 (94) | 2.14 (131) | 2.05 (242) | 19.8 (103) |
| 13.9 | 1.16 (50) | 1.24 (48) | 0.93 | 3.17 (89) | 2.12 (121) | 1.87 (207) | 17.7 (88) |
| 14.9 | 1.39 (56) | 1.47 (53) | 0.95 | 3.30 (91) | 2.38 (134) | 1.83 (200) | 18.9 (92) |
| 15.9 | 1.34 (48) | 1.45 (47) | 0.93 | 3.79 (94) | 2.50 (126) | 2.34 (233) | 18.2 (82) |
| 16.9 | 1.55 (55) | 1.63 (52) | 0.96 | 3.97 (99) | 2.38 (120) | 2.26 (225) | 23.2 (105) |
| 17.5 | 1.52 (53) | 1.63 (51) | 0.93 | 4.55 (112) | 2.78 (138) | 2.52 (248) | … |
| 17.8 | 1.66 (58) | 1.81 (56) | 0.92 | 3.98 (97) | 2.41 (120) | 2.01 (197) | 23.2 (103) |
| 17.9 | 1.62 (56) | 1.77 (55) | 0.91 | 4.01 (98) | 2.47 (123) | 2.20 (216) | … |
| 18.7 | 1.45 (50) | 1.57 (49) | 0.92 | 3.93 (96) | 2.53 (126) | 2.28 (224) | 25.3 (113) |
| 18.9 | 1.53 (53) | 1.60 (50) | 0.95 | 3.82 (94) | 2.31 (115) | 2.13 (209) | … |
| 19.3 | 1.69 (54) | 1.87 (53) | 0.90 | 3.76 (80) | 2.42 (96) | 1.89 (179) | 21.5 (84) |
| 19.9 | 1.50 (48) | 1.57 (45) | 0.96 | 4.05 (85) | 2.56 (101) | 2.40 (227) | 23.2 (91) |
| 20.3 | 1.53 (49) | 1.66 (47) | 0.93 | 4.22 (89) | 2.54 (100) | 2.40 (223) | 18.7 (74) |
| 20.8 | 1.56 (50) | 1.64 (47) | 0.95 | 3.83 (81) | 2.20 (87) | 2.09 (194) | 21.3 (84) |
A significant bronchodilator response was demonstrated on two occasions (ages 15.9 y and 17.5 y) but was not present on testing at age 17.9 y (not shown). Dlco = diffusing capacity of the lung for carbon monoxide; FRCpleth = functional residual capacity by plethysmography; RV = residual volume; TLC = total lung capacity.
a
FEV1, FVC, TLC, FRCpleth, and RV in liters and the percentage of predicted.