Table VIII.

Alteration of C1-INH function measured by 4 different methods and changes in C1-INH concentration vs therapy regimen in 1 patient with HAE^∗

Therapy and parameters	Normal range	Before infusion	30 min after infusion	P value
1000 U of C1-INH concentrate per week (for 15 doses)
Concentration (%)	55-135	18.2 ± 4.9	35.2 ± 8.4	<.001
EtCO-R (%)	70-130	10.6 ± 10.5	27.1 ± 22.5	.055
MeCO-R (%)	80-125	11.9 ± 5.4	26.2 ± 10.9	.001
C1r/C1-INH (%)	70-135	29.8 ± 20.9	87.7 ± 40.0	<.001
C1s/C1-INH (%)	>68	19.3 ± 10.5	54.1 ± 16.4	<.001
500 U of C1-INH concentrate twice per week (for 16 doses)
Concentration (%)	55-135	20.5 ± 5.8	30.6 ± 6.6	<.001
EtCO-R (%)	70-130	11.9 ± 11.0	23.5 ± 14.7	.015
MeCO-R (%)	80-125	19.4 ± 9.1	26.2 ± 9.7	.012
C1r/C1-INH (%)	70-135	38.4 ± 9.8	73.6 ± 30.0	<.001
C1s/C1-INH (%)	>68	23.8 ± 13.6	43.8 ± 16.6	<.002
500 U of C1-INH concentrate twice a week (for 31 doses) + danazol 100 mg/d
Concentration (%)	55-135	23.2 ± 3.4	34.8 ± 4.7	<.001
EtCO-R (%)	70-130	13.2 ± 16.5	25.3 ± 20.3	.012
MeCO-R (%)	80-125	9.1 ± 6.2	20.1 ± 11.9	<.001
C1r/C1-INH (%)	70-135	47.3 ± 23.6	85.6 ± 25.8	<.001
C1s/C1-INH (%)	>68	24.0 ± 11.0	42.7 ± 7.2	.090

^∗A previously untreated patient received initial treatment with 1000 U of C1-INH concentrate (Aventis-Behring). Because of the patient's tendency to develop an attack before the next C1-INH concentrate infusion, the weekly dose of C1-INH was divided into two 500 U infusions. Several times, while receiving 1000 U C1-INH concentrate per week, the patient had an attack the day or a day before the next infusion.³¹⁶ The possible effect of a baseline therapy of danazol in HAE patients is best seen in samples before when comparing therapy with 500 U C1-INH concentrate per week with 500 U C1-INH concentrate per week plus danazol 100 mg/d. R = (ɛ-Cb0)-Gly-Arg-pNA.