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Table 4 Efficacy of pdC1-INH in 74 swellings versus 129 untreated attacks in 12 patients with HAE-PLG

From: Treatment of patients with hereditary angioedema with the c.988A>G (p.Lys330Glu) variant in the plasminogen gene

Patient number

No. of untreated attacks*

Mean duration of untreated attacks (hrs)

No. of treated attacks

No. of attacks treated with 500 IU/1000 IU/1500 IU/3000 IU pdC1-INH

Mean time between attack onset and injection of icatibant (hrs)

Mean time to first symptom relief (hrs)

Mean duration of treated attacks (hrs)

Mean shortening of attack duration attacks (%)

No. of attacks shortened by > 50%

No. of attacks shortened by 20–50%

No. of attacks shortened by < 20%

Facial attacks

 1

10

47.6

20

4/16/0/0

1.7

2.6

45.3

4.8

0

0

20

 2

10

105.1

6

6/0/0/0

3.3

1

18

82.9

6

0

0

 14

10

67.1

1

0/1/0/0

2.5

1

24

64.2

1

0

0

Tongue swellings

 1

10

36.4

3

0/3/0/0

4

5

30

17.6

0

0

3

 4

10

17.3

4

2/0/1/1

1

0.3

54.5**

0

0

0

4

 15

10

2.7

1

1/0/0/0

2

0.5

1

62.7

1

0

0

 16

10

34.2

6

6/0/0/0

2

1

12

64.5

5

1

0

 17

3

48

2

0/1/1/0

6

1

21

56.3

2

0

0

 18

6

96

2

0/2/0/0

0.5

0.2

12

87.5

2

0

0

 19

10

24.4

1

0/1/0/0

2

1

6

75.4

1

0

0

 20

10

20.8

1

0/1/0/0

2

24

48**

0

0

0

1

Abdominal attacks

 1

10

27.7

12

2/10/0/0

0.2

3.6

26.3

5.1

0

2

10

 21

10

84.3

5

0/5/0/0

24

0.4

60.2

28.6

3

0

2

 22

10

81.9

10

0/10/0/0

2.5

1

22.2

72.9

8

2

0

Total No.

129

74

21/50/2/1

29

5

40

  1. HAE hereditary angioedema, HAE-PLG HAE with normal C1-INH and the c.988A > G (p.K330E) variant in the plasminogen gene; pdC1-INH plasma-derived C1-INH, No. number
  2. *Last 10 attacks before treatment or all if less than 10
  3. **Severe and rapidly developing attacks
  4. Note: Out of the 74 swellings, 27 were facial swellings, 20 tongue swellings, and 27 abdominal attacks