ABSTRACT
We present the results of a preliminary study (the first of this kind in Algeria) in which 4 families presenting with congenital deficiency of the C1-esterase inhibitor (C1-INH) responsible for hereditary angioneurotic oedema were biologically explored. The complement fractions C1-INH, C4 and C3d were assayed in 38 subjects of the 4 families. Extending this biological evaluation to all members of theses families enabled us to identify all asymptomatic subjects (46 percent in our series). In 2 patients the congenital disease was associated with systemic lupus erythematosus. Some clinico-biological discordances are reported and discussed in the light of data from the literature.
Subject(s)
Angioedema/congenital , Complement C1 Inactivator Proteins/deficiency , Adolescent , Adult , Algeria , Angioedema/genetics , Child , Child, Preschool , Complement C1 Inactivator Proteins/genetics , Complement C3d/analysis , Complement C4/analysis , Female , Humans , Male , Middle Aged , PedigreeABSTRACT
The titration of haemolytic complement in biological liquid cause inconvenient in calculating concentration, it was long and fastidious. We report a fast technic based on measure of hemolysis in microplate++ method, which exploitation of results, it does with an appropriate algorithm.