C1q nephropathy in a child presenting with recurrent gross hematuria

ArticleinPediatric Nephrology 25(1):165-8 · September 2009with1 Reads
DOI: 10.1007/s00467-009-1290-y · Source: PubMed
Abstract
C1q nephropathy is a rare glomerular disease characterized by mesangial immune deposits with dominant or codominant staining for C1q. The exact pathogenesis leading to the mesangial immune deposits of C1q remains unknown. C1q nephropathy often presents with proteinuria in the nephrotic range, with an unpredictable or poor response to corticosteroid therapy. It is seen more commonly in older children and young adults and is more common in African Americans compared with Caucasians. We present a 4-year-old African American girl who presented with recurrent gross hematuria in the absence of proteinuria or hypertension and whose renal biopsy demonstrated dominant mesangial deposits of C1q. We conclude that C1q nephropathy should be considered in patients who present with recurrent gross hematuria.
    • "Palpable abdominal masses with hematuria should be looked for the presence of tumor, polycystic kidney, or hydronephrosis; however, IgA nephropathy, thin membrane disease, Alport's syndrome may present with recurrent hematuria only. Other uncommon causes of recurrent gross hematuria can be C1q nephropathy and nutcraker syndrome [57, 58]. Hematuria of glomerular origin is usually brown, teacolored , or cola-colored, whereas macroscopic hematuria from the lower urinary tract is usually pink or red. "
    Article · Jan 2013 · Nefrologia: publicacion oficial de la Sociedad Espanola Nefrologia
    • "In each series patients were excluded if there was any clinical or serological evidence of systemic lupus erythematosus (SLE), and with the exception of a single patient all were normo-complementemic. Patients with pathological evidence of MPGN were specifically excluded from analysis in Jennette and Hipp's series, and for this reason the series of 4 patients reported by Nishida et al. is not included in the discussion [32, 39]. There are a number of case reports of C1q nephropathy in pediatric patients and as these represent unusual or atypical aspects of C1q nephropathy they will not be directly addressed404142434445464748495051. The first case series was published in 1991 by Iskandar et al. from Winston-Salem describing the clinical and pathological phenotype of C1q nephropathy in 15 children identified from a total of 91 biopsies performed over a 5-year period for nephrotic syndrome (NS), persistent glomerulonephritis , or persistent proteinuria [33]. "
    [Show abstract] [Hide abstract] 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.
    Full-text · Article · Feb 2010
  • Full-text · Article · Mar 2012
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