Clinical and Experimental Nephrology

, Volume 13, Issue 5, pp 531–532

Noonan syndrome: crossed fused ectopic kidneys and focal segmental glomerulosclerosis—a rare association

Authors

    • Department of NephrologyAll India Institute of Medical Sciences
    • AIIMS
  • Ambar Khaira
    • Department of NephrologyAll India Institute of Medical Sciences
  • Charanjit Lal
    • Department of NephrologyAll India Institute of Medical Sciences
  • Sandeep Mahajan
    • Department of NephrologyAll India Institute of Medical Sciences
  • Suresh C. Tiwari
    • Department of NephrologyAll India Institute of Medical Sciences
Letter to the Editor

DOI: 10.1007/s10157-009-0183-5

Cite this article as:
Gupta, A., Khaira, A., Lal, C. et al. Clin Exp Nephrol (2009) 13: 531. doi:10.1007/s10157-009-0183-5

Abstract

Noonan syndrome is characterised by short stature, typical facial dysmorphology and congenital heart defects. Urogenital abnormalities are reported in 10% of the cases. We present a 14-year-old girl with characteristic features of Noonan syndrome and nephrotic-range proteinuria. She had crossed fused ectopic kidneys. Renal biopsy showed focal segmental glomerulosclerosis. Oral steroids were instituted and she responded well. The case highlights this novel renal presentation of Noonan syndrome.

Keywords

Noonan syndromeCrossed fused ectopic kidneysFocal segmental glomerulosclerosis

Copyright information

© Japanese Society of Nephrology 2009