Daniel S Grosser1, Paul Persad2, Romualdo V Talento3, Lawrence R Shoemaker4, Tracy E Hunley5, Guillermo Hidalgo6,7, Mihail M Subtirelu8, Susan Coventry9, Radhakrishna Baliga10, Agnes B Fogo11. 1. Department of Pathology and Laboratory Medicine, University of Texas Health Science Center At San Antonio, San Antonio, TX, USA. grosser@uthscsa.edu. 2. Laboratory for Kidney Pathology, Nashville, TN, USA. 3. Eastern Carolina Pathology, Wilson, NC, USA. 4. Department of Pediatrics, Division of Pediatric Nephrology, University of Florida College of Medicine, Gainesville, FL, USA. 5. Division of Pediatric Nephrology, Monroe Carell Jr Children's Hospital At Vanderbilt, Nashville, TN, USA. 6. Pediatric Nephrology, East Carolina University, Greenville, NC, USA. 7. Pediatric Nephrology, Hackensack Meridian Health, Neptune, NJ, USA. 8. Pediatric Nephrology and Hypertension, East Tennessee Children's Hospital, Knoxville, TN, USA. 9. Department of Pathology, Norton Children's Hospital, Louisville, KY, USA. 10. Tulane University School of Medicine, New Orleans, LA, USA. 11. Department of Pathology, Microbiology and Immunology, Vanderbilt University Medical Center, Nashville, TN, USA.
Abstract
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.
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.
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