title: [Clinical findings in children with selective deficiency of Clq, a subunit of the first component of complement].
abstract: Recently we diagnosed a new complement defect, namely, selective deficiency of the Clq subcomponent of Cl. This defect was manifested by recurrent skin lesions, bouts of serious infections and membranoproliferative glomerulonephritis [4,5]. Within the past two years, we have diagnosed two new similar cases by paying attention to recurrent skin disease and associated symptomatology [7]. These patients had a bad prognosis due to severe bacterial infections and chronic kidney disease. In addition, discovery of new cases should be of help in explaining the importance of complement for host-parasite interactions and whether it is important for their management that an underlying deficiency of Clq be detected in similar cases. It is the purpose of this report to analyze common clinical findings and symptomatology in patients with selective complete Clq deficiency [4-7] and to compare them with other reported cases of Clq defects.
mesh_terms: D000419:Albuminuria / Q000209:etiology / Q000276:immunology; D001424:Bacterial Infections / Q000209:etiology / Q000276:immunology*; D002648:Child; D002675:Child, Preschool; D003872:Dermatitis / Q000209:etiology / Q000276:immunology; D005260:Female; D005921:Glomerulonephritis / Q000209:etiology / Q000276:immunology*; D006801:Humans; D008177:Lupus Vulgaris / Q000209:etiology / Q000276:immunology; D008297:Male
vernacular_title: Klinische Befunde bei Kindern mit selektivem Mangel an Clq, einer Untereinheit der erstem Komplementkomponente.