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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