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Table 1 Demographics and clinical features of the enrolled LAM patients

From: Gene mutations in sporadic lymphangioleiomyomatosis and genotype–phenotype correlation analysis

Characteristics

Ratio (%) or median (range) or mean ± SD

Sex

Female, n (%)

22 (100.0%)

Ethnic

Asian, n (%)

22 (100.0%)

aAge, median (range) (year)

38.5 (25–57)

Postmenopause, n (%)

2 (9.1%)

Smoking history, n (%)

0 (0.0%)

Pneumothorax, n (%)

11(50.0%)

Chylous effusions, n (%)

3 (13.6%)

Renal angiomyolipoma, n (%)

4 (18.2%)

bLymphangioleiomyomas, n (%)

16 (72.7%)

cRapamycin therapy, n (%)

0 (0.0%)

Serum VEGF-D, mean ± SD (pg/ml)

1719.0 ± 1311.4

FEV1% pred, mean ± SD (%)

76.3 ± 19.0

DLCO % pred, mean ± SD (%)

59.8 ± 22.9

6MWD (meters)

493.1 ± 86.1

CT grading

Grade I, n (%)

5 (22.7%)

Grade II, n (%)

7 (31.8%)

Grade III, n (%)

10 (45.5%)

  1. SD standard deviation, VEGF-D vascular endothelial growth factor-D, FEV1%pred forced expiratory volume in first second percent predicted, DLCO %pred diffusing capacity of carbon monoxide percent predicted, 6MWD six-minute walk distance, 6MWD six-minute walk distance
  2. aAge: the age when the patient was diagnosed
  3. bAll the CT results were examined by a radiology specialist to determine the presence of retroperitoneal or pelvic masses and to confirm whether the characteristics of the masses were compatible with lymphangioleiomyoma
  4. cAll patients were rapamycin treatment-naïve at the time their blood samples were collected