Table II.
Biological data
| Plasma kallikrein (Vmax, nmol/min/mL) |
|||||||
|---|---|---|---|---|---|---|---|
| Spontaneous kallikrein activity | Proenzyme activation | CPN (Vmax, nmol/min/mL)∗ | APP (Vmax, nmol/min/mL) | ACE (IU) | |||
| Reference population, 5th-95th percentile interval (median) | |||||||
| Male (n = 98) | 3.1-9.2 (5.8) | 1830-2765 (2253) | 70.9-105.9 (92.0) | 0.25-3.08 (1.04) | 43-95 (69) | ||
| Female (n = 101) | 3.2-10.6 (6.4) | 1870-2985 (2351) | 79.6-100.0 (90.7) | 0.50-5.93 (1.59) | 42-85 (62) | ||
| Family ID | Patient | Sex | |||||
| A | I.2 | F | 65.1 ± 11.1 (n = 2)† | ||||
| II.1‡ | F | 1.7 | 3237 ± 136 (n = 2); NS | 27.2 ± 0.9 (n = 3)§ | 2.68 ± 0.18 (n = 3) | 42 ± 4 (n = 3) | |
| II.2 | M | 46.4 ± 7.9 (n = 2)|| | |||||
| II.3 | F | 82.5 ± 6.8 (n = 2); NS | |||||
| III.1 | F | 86.7 ± 3.8 (n = 3); NS | |||||
| B | I.1 | M | 9.1 | 1819 ± 434 (n = 2)† | 60.4 ± 1.2 (n = 2)|| | ||
| I.2 | F | 5.7 | 1316 ± 65 (n = 2)¶ | 61.8 ± 1.7 (n = 2)¶ | |||
| II.1† | F | 7.7 | 1233 ± 48 (n = 2)¶ | 42.6 ± 1.5 (n = 2)|| | 0.82 | 53 | |
| III.1 | F | 3.2 | 1156 ± 43 (n = 2)¶ | 62.4 ± 4.8 (n = 2)¶ | 0.67 | 47 ± 7 (n = 2) | |
| III.3 | F | 10.6 | 1896 ± 15 (n = 2); NS | 59.7 ± 4.7 (n = 2)|| | 1.87 | 37.5 ± 3.5 (n = 2) | |
| IV.1 | M | 2.7 | 1185 ± 106 (n = 2)|| | 50.7 ± 4.2 (n = 2)|| | 0.46 | 78 | |
| C | II.2 | F | 10.5 | 3313 | 38.4 ± 7.6 (n = 2)|| | 38.5 ± 0.7 (n = 2) | |
| III.1 | F | 58.7 ± 1.1 (n = 2)|| | |||||
| III.2† | F | 10.1 | 2808 | 33.4 ± 1.5 (n = 2)|| | 0.90 | 41 ± 1.4 (n = 2) | |
| D | I.1 | M | 3.9 | 3010 | 70.6 ± 2.6 (n = 2)† | 1.56 | 43 |
| I.2 | F | 4.2 | 2554 | 63.7 ± 0.9 (n = 2)† | 5.07 | 63 | |
| II.1† | M | 8.4 | 2883 | 54.6 ± 0.4 (n = 2)|| | 1.89 | 75 | |
Enzymatic continuous variables in patient samples are presented as mean ± SD. Reference intervals with medians generated in healthy donors are presented (95th percentile). Enzymatic activity outside the reference interval is highlighted in bold.
F, Female; M, male; NS, not significant.
Lower threshold value 0.7 nmol/min/mL.
When compared with reference interval, significance of data is shown: P ≤ .05 (Mann-Whitney U test).
Family proband.
When compared with reference interval, significance of data is shown: P ≤ .0001 (Mann-Whitney U test).
When compared with reference interval, significance of data is shown: P ≤ .001 (Mann-Whitney U test).
When compared with reference interval, significance of data is shown: ∗∗P ≤ .01 (Mann-Whitney U test).