Skip to main content

Table 1 Clinical data of patients with HAE-nlC1-INH according to the identification of mutation

From: Abstracts of 11th C1-inhibitor Deficiency & Angioedema Workshop

 

HAE-F12

HAE-unknown

Patients

183

38

Families

62

15

Family history

170/183

92.9%

38/38

100%

Sex

153F:30M

83.6%:16.4%

33F:5M

86.8%:13.2%

Onset of symptoms (age)

2–68 (136/141) median = 18.5

2–52 (31/38) median = 16

Site of edema

 Subcutaneous

84/141

59.6%

26/38

68.4%

 Facial

119/141

84.4%

31/38

81.6%

 Genital

21/141

14.9%

10/38

26.3%

 Abdominal

109/141

77.3%

28/38

73.7%

 Tongue

32/141

22.7%

5/38

13.2%

 Upper airways

65/141

46.1%

17/38

44.7%

Trigger factors

Hormones (symptomatic women) (n = 128)

 Oral contraceptives

87/128

68.0%

17/33

51.5%

 Pregnancy

25/128

19.5%

6/33

18.2%

 Menses

1/128

0.8%

3/33

9.1%

 HRT

2/128

1.6%

1/33

3.0%

All symptomatic patients

 Stress

87/141

61.7%

18/38

47.4%

 Trauma

70/141

49.6%

16/38

42.1%

 Surgery

11/141

7.8%

4/38

10.5%

 Dental procedure

21/141

14.9%

5/38

13.2%

 Infection

6/141

4.3%

4/38

10.5%

 Weather

11/141

7.8%

4/38

10.5%

 Exercise

4/141

2.8%

5/38

13.2%

  1. HRT: hormonal reposition therapy