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 |