PMID: 10975310Sep 7, 2000Paper

C1q nephropathy presenting as rapidly progressive crescentic glomerulonephritis

Pediatric Nephrology : Journal of the International Pediatric Nephrology Association
T SrivastavaU S Alon

Abstract

C1q nephropathy is an immune complex glomerulonephritis defined by the presence of mesangial immunoglobulins and complement deposits, most notably C1q, and the absence of clinical and laboratory evidence of systemic lupus erythematosus. Histology in C1q nephropathy is characterized by a slight to severe increase in mesangial cellularity and matrix, with or without segmental sclerosis. C1q nephropathy usually presents with nephrotic-range proteinuria in older children and young adults, and has a poor response to steroids. Patients may have decreased creatinine clearance at presentation, but progression to end-stage renal disease (ESRD) is slow. Severe crescentic glomerulonephritis has not been reported in C1q nephropathy. We describe a 3-year-old Hispanic girl who presented with renal insufficiency. Kidney biopsy showed C1q nephropathy with severe crescentic glomerulonephritis. The clinical and serological evaluation ruled out systemic lupus erythematosus or other immunological or infectious etiologies. In spite of immunosuppressive therapy, she progressed to ESRD within 14 weeks and is currently on chronic peritoneal dialysis. The atypical features of C1q nephropathy observed in our patient, which have not been described in ear...Continue Reading

Citations

Apr 14, 2005·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Keith K LauRobert J Wyatt
Mar 8, 2006·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Isabel RobertiDae Un Kim
Oct 24, 2007·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Jameela A Kari, Sawsan M Jalalah
Aug 9, 2008·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Isabel RobertiDae Un Kim
Dec 17, 2008·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Craig S WongJohn R Brandt
Aug 12, 2009·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Luke TaggartFranca Iorember
Feb 25, 2010·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Scott E WenderferMichael C Braun
Sep 13, 2003·Kidney International·Glen S MarkowitzVivette D D'Agati
Jul 25, 2008·Journal of the American Society of Nephrology : JASN·Alenka VizjakJ Charles Jennette
Apr 18, 2009·Clinical and Experimental Nephrology·Akiko MiiYuh Fukuda
Jul 25, 2008·Clinical Journal of the American Society of Nephrology : CJASN·Satoshi HisanoHiroshi Iwasaki
Feb 24, 2006·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·Yuko FukumaHiroshi Iwasaki
Mar 12, 2015·Saudi Journal of Kidney Diseases and Transplantation : an Official Publication of the Saudi Center for Organ Transplantation, Saudi Arabia·K V KanodiaH L Trivedi
Nov 28, 2013·Nephro-urology Monthly·Pavan Malleshappa, Mahesha Vankalakunti
Apr 6, 2006·Archives of Pathology & Laboratory Medicine·Clarissa S Krinsky, Alexis Harris

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