TABLE I.

Immunologic and clinical data from the cohort with HAE at WUSM

Patient no.	HAE phenotype	Age (y)	Sex	Onset (y)	Location of edema*	Frequency of edema†	Duration of attacks (d)	C1-INH antigen (19–37 mg/dL)	C1-INH function‡ (%)	C4 (mg/dL)
101	Type I	48	F	8	E, F, L	3/y	2	<3	0	<4§
201	Type I	55	M	18	A, E, F, L	12/y	2	8	<10	3‖
301	Type I	73	F	12	A, E, F, L	15/y	1	6	48	6§
401	Type I	58	F	16	A, E, F, L	8/wk	2	4	0	2‖
501	Type I	49	F	13	A, E, F, L	3/wk	3	10	43	3‖
502	Type I	44	M	8	A, E, F, G, L	4/wk	1	7	42	2‖
503	Type I	8	M	8	E, G	2/y	1	12	60	12‖
504	Type I	25	F	15	A, E, F, G, L	3/wk	1	8	52	<4‖
601	Type I	30	M	21	A, E, F	2/wk	1	9	63	10§
701	Type I	40	F	33	A, E, G, L	2/y	0.5	9	45	6‖
702	Type I	14	M	14	A, E	3/mo	0.5	5	38	3¶
801#	Type I	11	F	8	A, E, F, L	3/mo	1	7	27	8§
901	Type I	30	F	1.5	A, E, F, G, L	3/wk	1	4	56	10‖
902	Type I	30	F	1	A, E, F, L	1–2/wk	2	11	38	3‖
903	Type I	68	M	10	A, E, F, G, L	2–3/mo	1	14	85	16‖
1001	Type I	25	M	13	A, E, F, G, L	2–3/mo	2	5	34	3‖
1101	Type I	26	M	14	A, E, F, L	1–2/wk	1	8	68	6‖
1201	Type II	35	F	18	A, E, F	1/mo	2	29	18	NA
1301	Type II	26	M	14	A, E	3–4/mo	1	33	16	16**
1401	Type II	17	M	0.5	A, E, F, L	2–3/wk	2	24	24	2‖

All study candidates are White.

^*A, abdomen; E, extremities, F, face; G, genitals; L, larynx.

^†Frequency of edema before HAE treatment.C4 antigen levels were measured at 4 institutions with slightly different normal ranges.

^‡C1-INH function: >67%, normal; 41%–67%, equivocal; <41%, abnormal.

^§Normal level of C4: 12–54.

^‖Normal level of C4: 10–40.

^¶Normal level of C4: 14–59.

^#Except for patient 801, all other patients have a family history of HAE.

^**Normal level of C4: 17–42.