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Table 2 Patients with LAM and a history of parturition after taking sirolimus

From: Pregnancy after the diagnosis of lymphangioleiomyomatosis (LAM)

Patient no. 1 2 3 4 5 6
Diagnosis S-LAM S-LAM S-LAM S-LAM S-LAM S-LAM
Age of diagnosis 29 30 25 34 26 30
Previous pregnancy G1P1 G1P0 G1P1 G1P1 G0P0 G0P0
Baseline       
 CT grade III III III NA NA II
 VEGF-D (pg/ml) 2143.4 1089.4 1591.5 2059.7 NA 4884.4
 SGRQ total 76 15 38 14 NA 26
 FEV1 (L) 0.52 2.50 0.63 1.88 NA 2.22
 FEV1/Pred (%) 17.4 90.9 21.8 73.9 NA 73.1
 FVC (L) 2.21 3.37 1.46 3.44 NA 2.86
 FVC/Pred (%) 63.9 106.3 43.9 117.6 NA 81.7
 FEV1/FVC (%) 23.6 74.1 43.2 54.6 NA 77.7
 DLco (mmol/min/kPa) 1.22 4.80 1.26 2.84 NA 4.20
 DLco/Pred (%) 13.5 55.3 14.1 33.2 NA 46.5
 PaO2 (mmHg) 62.9 76.9 70.9 82.5 NA NA
 6MWD (m) 383 675 366 513 NA 520
 Complications None AML PTX CTX PTX/AML/CTX PTX
Sirolimus       
 Duration (months) 53 46 70 29 12 6
 Dose (mg/day) 1.5 1.5 1.0 1.0 1.0 1.0
 Serum level (ng/ml) 7.8 7.0 NA 6.0 NA NA
 Time of discontinuation (week)a + 8 + 5 + 4 − 27 − 8 − 12
Pregnancy       
 Age of pregnancy (years) 34 34 35 37 27 33
 Mode of delivery C/S C/S C/S VD VD C/S
 Gestational week 37 35 38 39 38 37
 Exacerbated dyspnea Yes Yes Yes No No Yes
 Oxygen inhalation (h/day) 24 2–5 1 1 None 1
 Complications of LAM None Rupture of AML None None PTX PTX
 Obstetric diseases None None None None None None
After parturition       
 Exacerbated dyspnea No No No No Yes Yes
 Score of SGRQ 56 16 NA 23 NA 32
 Sirolimus restart (weeks after delivery) 6 8 4 No No 24
Infants       
 Birth weight (g) 3000 2100/1850b 2600 2850 3500 2860
 Malformations No No No No No No
 NICU experience No No No No Yesc No
  1. AML angiolipoma, CTX chylothorax, C/S cesarean section, DLco diffusing capacity for carbon monoxide, FEV1 forced expiratory volume in 1 s, FVC forced vital capacity, NICU newborn intensive care unit, PaO2 partial pressure of oxygen in arterial blood, PTX pneumothorax, SGRQ St. George’s Respiratory Questionnaires, S-LAM sporadic lymphangioleiomyomatosis, VD vaginal delivery, VEGF-D vascular endothelial growth factor-D, 6MWD 6-min walking distance
  2. a‘+’ means time after pregnancy; ‘−’ means time before pregnancy
  3. bThis patient delivered twins
  4. cThe baby was sent to the NICU for aspiration pneumonia and recovered in one week