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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