C1q nephropathy in two young sisters

Pediatric Nephrology : Journal of the International Pediatric Nephrology Association
Jameela A Kari, Sawsan M Jalalah

Abstract

C1q nephropathy (C1qNP) is a controversial and uncommon form of glomerulonephritis, characterized by mesangial immunoglobulin and complement deposits, predominantly C1q, with no evidence of systemic lupus erythematosus. Clinically, it may present as nephrotic syndrome and non-nephrotic proteinuria per se or associated with microhematuria, hypertension, or renal insufficiency. We describe two sisters with C1qNP, who presented with steroid-resistant nephrotic syndrome. Both sisters presented before the age of 2 years, and they showed a poor response to other immunosuppressive therapy. Both girls had normal serum complement levels, negative antinuclear antibodies (ANAs) and negative hepatitis B antigen. Renal biopsy in both patients showed histological features of mesangioproliferative glomerulonephritis, with diffuse "full-house" positive immunofluorescence reaction in the mesangial area. The immunofluorescence reaction for C1q was most intense and co-dominant with IgG in both patients. Correspondingly, electron microscopy demonstrated dense deposits mainly in the mesangial areas too. We report on two young sisters with the characteristic features of C1qNP presented in early childhood. To the best of our knowledge, this is the fi...Continue Reading

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Citations

Jun 6, 2013·ISRN Dentistry·Istabrak HasanFriedhelm Heinemann
Dec 17, 2008·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Craig S WongJohn R Brandt
Feb 25, 2010·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Scott E WenderferMichael C Braun
Apr 18, 2009·Clinical and Experimental Nephrology·Akiko MiiYuh Fukuda

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