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Table 1 Clinical data for five patients with lymphangioleiomyomatosis (LAM) and recurrent pneumothorax treated with sirolimus

From: Efficacy of sirolimus for the prevention of recurrent pneumothorax in patients with lymphangioleiomyomatosis: a case series

Characteristics Case 1 Case 2 Case 3 Case 4 Case 5
Age (years) 33 23 31 38 30
VEGF-D (pg/ml) 6608 2385 1139 3901 2685.8
PaO2(mmHg) 51 92 96 45.5 98
Surgery No No Yes Yes Yes
Chemical pleurodesis No No Yes No No
Number of PTX episodes before sirolimus therapy 6 3 6 6 2
Sirolimus concentration (ng/ml) 5–7 4–5 6–10 3–8 5.28a
Number of PTX episodes during sirolimus therapy 0 0 0 1 0
Follow-up 6MWT results (m) 480 550 555 510 500
  1. aOnly examined once
  2. VEGF-D vascular endothelial growth factor-D, PTX pneumothorax, PFT pulmonary function test, 6MWT 6-min walk test, FVC forced vital capacity, FEV1 forced expiratory volume in 1 s, DLCO diffusing capacity for carbon monoxide