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Background: IgA-dominant infection-associated glomerulonephritis is well-documented in adults but has not been studied in depth in children. We assessed the incidence of pediatric IgA-dominant infection-associated glomerulonephritis and clinical and kidney biopsy findings., Methods: Pediatric native kidney biopsies over a 10-year period with IgA dominance, strong C3, and findings indicative of infection-associated etiology were identified., Results: We identified 9 cases of IgA-dominant infection-associated glomerulonephritis, 0.8% of pediatric native kidney biopsies. Seven patients presented with elevated creatinine. All had hematuria and proteinuria. Eight patients had clinical evidence of infection: one each with central port infection by methicillin-sensitive Staphylococcus aureus, recurrent streptococcal pharyngitis and recent otitis media, streptococcal pharyngitis demonstrated 8 months after biopsy, suspected streptococcal scalded skin syndrome, and viral gastroenteritis, and three with serologic evidence of Streptococcal infection but no identified site of infection. All but one patient experienced short-term normalization of creatinine and resolution of proteinuria, though two eventually progressed to kidney failure: one 3 years later due to progressive disease and one 11 years later due to focal segmental glomerulosclerosis without concurrent immune deposits., Conclusions: Pediatric IgA-dominant infection-associated glomerulonephritis is rare, and generally has a favorable prognosis, contrasting that seen in adults with severe comorbidities. A higher resolution version of the Graphical abstract is available as Supplementary., (© 2021. IPNA.)

Streptococcus pyogenes is the most common cause of post-infectious glomerulonephritis. There have been isolated case reports of nephritis following infections with Streptococcus pneumoniae. We report here the case of a 6-year-old white female who presented with blood culture-confirmed pneumococcal pneumonia associated with glomerulonephritis. Her acute renal failure improved over several days, and renal function was normal by 8 weeks post-hospitalization. This case serves to reinforce the concept that other organisms besides Streptococcus pyogenes can trigger a similar post-infectious glomerulonephritis and should be considered in the differential diagnosis of any child who presents with acute glomerulonephritis and respiratory findings. Additionally, pneumococcus group 7 may be a nephritogenic strain and requires further investigation.

A 10-year-old African-American female was evaluated for asymptomatic proteinuria. Initial investigation did not reveal the etiology of her renal disease. She subsequently underwent a percutaneous renal biopsy, which was consistent with fibrillary glomerulopathy. This condition is rare in children and is identified histologically by a solid, randomly arranged, amyloid-like fibrillar structure with a diameter of about 18-22 nm. It is a diagnosis of exclusion, the clinical presentation of which can be quite varied. Children usually present with nephrotic syndrome. There is no effective therapy for this condition. Therapy with steroids, cytotoxic agents, and plasmapheresis does not confer any real benefit in stabilizing or improving renal function. However, angiotensin converting enzyme inhibitors can decrease the proteinuria. End-stage renal disease is known to occur in 50% of patients within 4 years of diagnosis. The nephrotic-range proteinuria in our patient significantly decreased during a 4-year follow-up without any therapy, while maintaining normal renal function.