Inherited C3 deficiency with recurrent infections and glomerulonephritis
M. S. Borzy, A. Gewurz, L. Wolff, D. Houghton and E. Lovrien
Department of Pediatrics, School of Medicine, Doernbecher Memorial Hospital for Children, Oregon Health Sciences University, Portland 97201.
A 10-year-old Laotian boy had homozygous deficiency of the third component
of complement and recurrent bacterial infections beginning at age 5 months.
Cellular and humoral immunity were normal, as were polymorphonuclear
leukocyte chemotaxis and bactericidal activities. Serum complement-mediated
hemolytic, chemotactic, and opsonic activities were deficient. In vitro
addition of purified C3 to patient serum restored hemolytic complement to
normal levels, and plasma infusion during each of four episodes of
pneumonia significantly enhanced serum opsonic activity for as long as 36
hours. A renal biopsy specimen revealed mesangiopathic glomerulonephritis,
although significant levels of circulating IgG immune complexes were not
detected. These findings further support the association of C3 deficiency
with immune-complex disease and suggest that plasma infusion may be an
adjunct to antibiotic therapy in the management of severe pyogenic
infections in patients with C3 deficiency.