Complement factor I deficiency in a family with recurrent infections
MF Leitao, MMS Vilela, R Rutz, AS Grumach… - …, 1997 - Elsevier
Factor I deficiency causes a permanent, uncontrolled activation of the alternative pathway
resulting in an increased turnover of C3 and consumption of factor B, factor H and properdin.
Factor I deficiency is clinically associated with recurrent bacterial infections already in early
infancy, mainly affecting the upper and lower respiratory tract, or presenting as meningitis or
septicemia. We here report on a Brazilian family (n= 9) with known consanguinity, where in 3
7 children, suffering from chronic otitis, meningitis, and respiratory infections, a complete …
resulting in an increased turnover of C3 and consumption of factor B, factor H and properdin.
Factor I deficiency is clinically associated with recurrent bacterial infections already in early
infancy, mainly affecting the upper and lower respiratory tract, or presenting as meningitis or
septicemia. We here report on a Brazilian family (n= 9) with known consanguinity, where in 3
7 children, suffering from chronic otitis, meningitis, and respiratory infections, a complete …