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 | Â | Â | Â | Â | Â | Â |
 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 | Â | Â | Â | Â | Â | Â |
 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 | Â | Â | Â | Â | Â | Â |
 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 | Â | Â | Â | Â | Â | Â |
 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 | Â | Â | Â | Â | Â | Â |
 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 |