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C1q nephropathy in the pediatric population: pathology and pathogenesis

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Abstract

C1q nephropathy was originally described nearly 25 years ago by Jennette and Hipp. Since that time there have been a limited number of publications on C1q nephropathy, most of them in the pediatric literature. Despite reported incidences as high as 16% in some pediatric biopsy series, a consensus definition on the diagnosis of C1q nephropathy is lacking and its existence as a distinct clinical disease entity remains controversial. The purpose of this review is to discuss the biology of C1q in the context of mechanisms of C1q deposition, and to provide a detailed analysis of the published pediatric case series with a focus on the pathological criteria used to establish the diagnosis of C1q nephropathy as well as long-term outcomes in children.

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Authors and Affiliations

  1. Division of Pediatric Nephrology and Hypertension, Department of Pediatrics, University of Texas School of Medicine, Houston, USA

    Scott E. Wenderfer, Rita D. Swinford & Michael C. Braun

  2. Center for Immunology and Autoimmune Diseases, The Brown Foundation Institute of Molecular Medicine, University of Texas Health Science Center, Houston, USA

    Scott E. Wenderfer & Michael C. Braun

  3. IMM/UTHSC-H, 1825 Pressler Street, Room 630C, Houston, TX, 77030, USA

    Michael C. Braun

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  1. Scott E. Wenderfer
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Correspondence to Michael C. Braun.

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Wenderfer, S.E., Swinford, R.D. & Braun, M.C. C1q nephropathy in the pediatric population: pathology and pathogenesis. Pediatr Nephrol 25, 1385–1396 (2010). https://doi.org/10.1007/s00467-009-1429-x

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