2.2 Complement parameters
C1-INH, C3, C4, C1q were measured at diagnosis. Patients were not on
prophylactic treatment when complement parameters were measured.
C1-INH levels (normal range:
0.21–0.39 g/l) were measured using the Dade Behring BN ProSpec
(Siemens, Germany); C4 (normal range: 0.1-0.4 g/l 1-11 years and 0.2-0.5
g/l> 11 years) and C3 (normal range: 0.6-1.5 g/l 0-3 years,
0.7-1.8 g/l 3- 6 years, 0.9-1.8 g/l> 6 years), using the
Konelab Primer 60i (Thermo Scientific, USA). The Infosheet Anthos
multimode fluorometer Zenyth 1100 (Italy) was used to measure functional
C1-INH (normal range: 77.3-128.8%) and C1q (normal range: 2-20 RU/ml)
levels.