TABLE 5.
Concentration of C1-INHt, C1-INHa and C1-INH complexes in healthy controls and C1-INH-HAE patients.
| Healthy controls (n = 6) [min–max] | Patients with C1-INH-HAE type I (n = 5) [min–max] | Patients with C1-INH-HAE type II (n = 5) [min–max] | |
| C1-INHt (nM) | 3638 [2013–5175] | 678.2∗∗ [342–1169] | 3539## [2673–5957] |
| C1-INHa (nM) | 2201 [1171–3126] | 510.9∗∗∗ [209.8–994.6] | 517.4∗∗∗ [315–812.6] |
| C1r/C1-INH (nM) | 28.59 [23.27–34.84] | 30.94 [15.58–44.04] | 28.54 [20.91–39.97] |
| C1s/C1-INH (nM) | 25.04 [13.88–36.9] | 30.46 [16.49–44.59] | 35.52 [20.85–44.91] |
| MASP-1/C1-INH (nM) | 0.38 [0.24–0.50] | 0.27 [0.20–0.39] | 0.55∗.## [0.43–0.62] |
| MASP-2/C1-INH (nM) | 0.29 [0.14–0.43] | 0.17 [0.07–0.41] | 1.27****.#### [1.00–1.69] |
| Kallikrein/C1-INH (nM) | 0.40 [0.21–0.67] | 1.54∗ [0.67–2.28] | 1.36∗ [0.51–2.59] |
| FXIIa/C1-INH (nM) | 0.62 [0.28–1.32] | 0.40 [0.29–0.50] | 0.65 [0.35–0.94] |
| FXIa/C1-INH (nM) | 1.21 [0.40–1.83] | 0.91 [0.63–1.11] | 1.79# [1.06–2.32] |
| Thrombin/C1-INH (nM) | 8.47 [3.80–14.75] | 8.31 [5.80–9.83] | 14.19 [7.30–19.27] |
C1-INHt: total C1-INH concentration. C1-INHa: active C1-INH concentration. ∗Significant difference between healthy controls and patients. #Significant difference between type I and type II patients.*/#0.01 < p < 0.05. **/##0.001 < p < 0.01. ***/###0.0001 < p < 0.001. ****/####p < 0.0001. Statistical analysis: ordinary one-way-ANOVA and multiple comparisons.