AI Article Synopsis
- A 7-year-old Laotian boy with a lack of the complement component C3 suffered from recurrent infections and ongoing blood and protein in urine, leading to a study of his kidney tissue.
- The kidney tissue showed signs of mesangiopathic disease, with features similar to certain types of glomerulonephritis, indicating potential damage in the filtering units of the kidney.
- Immunofluorescence testing revealed the presence of various immunoglobulins and fibrinogen in specific areas, but notably no C3, reinforcing the relationship between congenital C3 deficiency and immune-related kidney diseases.
Article Abstract
The renal histopathology of a 7-year-old Laotian male with inherited deficiency of the third component of complement, recurrent infections, and persistent hematuria and proteinuria is described. The histologic changes are predominantly those of mesangiopathic disease with isolated changes resembling type I membranoproliferative glomerulonephritis and transmembranous glomerulonephritis. IgG, IgA, IgM, C4, and fibrinogen, but not C3, were detected by immunofluorescence in mesangial zones and in segments of capillary walls. A normal distribution of C3b receptors was present along all capillary walls. This report provides additional support for the association of congenital C3 deficiency and immune deposit glomerulonephritis.
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| http://dx.doi.org/10.1016/s0272-6386(85)80137-2 | DOI Listing |
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