C3 deposition glomerulopathy due to a functional factor H defect
S Habbig, MJ Mihatsch, S Heinen, B Beck… - Kidney …, 2009 - kidney-international.org
CASE PRESENTATION Two female siblings (patient 1: 12 6/12; Patient 2: 7 3/12 years old)
of consanguineous parents, presented with hematuria and proteinuria at five years (Patient
1) and six months (Patient 2) of age, respectively. Clinical examination, renal
ultrasonography and laboratory analyses resulted normal, and both patients were
normotensive. Detailed complement analyses, however, demonstrated activation of the
alternative complement pathway reflected by decreased C3 and Factor B (FB), and …
of consanguineous parents, presented with hematuria and proteinuria at five years (Patient
1) and six months (Patient 2) of age, respectively. Clinical examination, renal
ultrasonography and laboratory analyses resulted normal, and both patients were
normotensive. Detailed complement analyses, however, demonstrated activation of the
alternative complement pathway reflected by decreased C3 and Factor B (FB), and …