Giant Bilateral Renal Angiomyolipomas and Lymphangioleiomyomatosis Presenting after Two Successive Pregnancies Successfully Treated with Surgery and Rapamycin
Table 1
Effect of rapamycin on pulmonary function in a patient with LAM treated for 12 months.
Variable
−5 months (November 2009)
Baseline (March 2010)
+6 months rapamycin (October 2010)
+12 months rapamycin (April 2011)
FEV1
Volume (mL)
2920
2810
3140
3080
% of predicted value
83.5
80.9
90.5
89.5
FVC
Volume (mL)
3870
3440
3810
4010
% of predicted value
96.7
86.5
95.8
101.4
FEV1/FVC (%)
75.4
81.6
82.4
77.0
TLC (mL)
5140
5380
5530
6110
% of predicted value
92.4
96.7
99.5
109.9
RV (mL)
2120
2490
1860
2140
% of predicted value
135.7
157.7
117.6
134.6
RV/TLC (%)
41.24 (144.8%)
46.24 (160.4% )
33.53 (116.4%)
35.08 (120.3%)
DLCO
Diffusing capacity (mL/mm Hg/min)
8.46
9.19
8.84
8.52
% of predicted value
85.0
92.7
89.2
86.4
DLCO/VA (%)
2.00 (111.8%)
2.21 (124.2%)
1.66 (93.3%)
1.61 (90.7%)
FEV1 : Forced expiratory volume in 1 second. FVC: Forced vital capacity. TLC: Total lung capacity. RV: Residual volume. DLCO: Diffusing capacity for carbon monoxide.