Table 2.

Characteristics of untreated hereditary angio-oedema (HAE) patients with normal serum C4.

Patient	Age (years)	Sex	Normal C4 values (g/l) (≥ 0.14)	Clinical features	Low C4 (g/l) values (≥ 0.14)	C1inhA (mg/l) (> 150)	C1inhF by Quidel (%) (> 84)	C1inhF by Technochrom (units/ml) (> 0.7)
1	69	F	0.15, 0.19	Peripheral swellings	None	78	69, 93, 86, 51	0.6
2	20	M	0.16	Peripheral swellings	0.11	100	91,93, 81	< 0.3
3	11	M	0.23, 0.19	None (affected mother)	0.10	122	81, 84	< 0.3
4	36	M	0.16, 0.15, 0.15	Peripheral/facial swelling	0.11	108	81,69	0.4
5	75	M	0.21	Peripheral/facial swelling	0.13	83	37	Not performed