Table 1.

Clinical features of the 27 patients with hereditary angioedema (HAE)

Clinical features	HAE-1/2 (n = 19)	HAE-nC1-INH (n = 8)	p-value	Total (n = 27)
Females(%)	14 (73.68%)	4 (50%)	0.375	18 (66.67%)
At first event(years), mean ± SD	19.79 ± 8.88	39.95 ± 14.41	0.034*	25.76 ± 14.09
At diagnosis(years), mean ± SD	32.14 ± 14.01	46.08 ± 15.68	0.789	36.27 ± 15.62
Delay in diagnosis(years), median(IQR)	12 (1,22)	1.75 (0.90,1.83)	0.333	8 (1,19)
Family history of HAE(%)	14 (73.68%)	1(12.5%)	0.008*	15 (55.56%)
Genomic variant
SERPING1	12	0	/	12
FXII	0	1	/	1
KNG1	0	2	/	2
MYOF	1	4	/	5
HS3ST6	0	1	/	1
Not checked	7	0	/	7
Clinical manifestations
Skin/extremities/trunk	19	8	/	27
Gastrointestinal tract	11	3	0.420	14
Laryngeal	12	3	0.398	15
Persistent swelling	0	3	0.019*	3
Prodromal symptoms
Erythema or erythema marginatum	5	1	0.633	6
Tiredness and fatigue	2	0	1.000	2
Trigger factors
Trauma/compression	7	0	0.062	7
Stress/emotional factors	6	3	1.000	9
Infection	2	0	1.000	2
Cold weather	3	0	0.532	3
Around menstruation/Oral contraceptive	4	0	0.285	4
Exposure to specific foods or chemicals (i.e. persimmons, seafood, alcohol)	3	1	1.000	4
Comorbidities
Allergic diseases	5	6	0.033*	11
Autoimmune diseases	5	2	1.000	7
Simultaneous attacks of edema and pruritic rashes (i.e. urticaria)	1	3	0.081	4

*For the categorical variables, the p-vaule was obtained from chi-square test, with Fisher’s exact test applied when expected frequencies in any cell were less than five. For continuous variables, the p-vaule was obtained from independent samples t-tests for normally distributed data or the Mann-Whitney U test for non-normally distributed data