Pediatric Nephrology

, 24:207

Acute renal cortical necrosis due to acquired antiprotein S antibodies

  • Anis Skander Larakeb
  • Solène Evrard
  • Férielle Louillet
  • Thérésa Kwon
  • Hadji Djaffar
  • Brigitte Llanas
  • Georges Deschênes
  • Marie-Françoise Hurtaud-Roux
  • Véronique Baudouin
Brief Report

DOI: 10.1007/s00467-008-0967-y

Cite this article as:
Larakeb, A.S., Evrard, S., Louillet, F. et al. Pediatr Nephrol (2009) 24: 207. doi:10.1007/s00467-008-0967-y

Abstract

Although varicella is a common disease of childhood, renal complications are quite rare. We report here the interesting case of a-22 month-old boy exhibiting renal cortical necrosis related to an acquired protein S deficiency following varicella. Ten days after the vesicle eruption appearance, he presented with ecchymosed heels, oligoanuric kidney failure, anemia [hemoglobin (Hb) 78 g/L], schizocytosis (2.5%), but normal platelet count. Kidney sonography and magnetic resonance imaging evoked renal cortical necrosis. All together, these features suggested acquired protein S deficiency secondary to varicella. Strikingly, it was confirmed by a dramatic decrease in protein S plasma activity and a huge increase in immunoglobulin (Ig)G antibodies against protein S in the plasma. Anticoagulation therapy in addition with plasmapheresis and steroid pulses allowed a dramatic decrease in the antibodies against protein S and recovery of normal protein S activity. Undelayed diagnosis and treatment did not avoid kidney insufficiency but prevented life-threatening complications. In the light of this case report, protein S deficiency due to antibody inhibition should be carefully monitored anytime in the context of varicella when kidney insufficiency or necrosis occurs.

Keywords

Acute renal failureAntiprotein S antibodyPlasma exchangeProtein S deficiencyVaricella

Copyright information

© IPNA 2008

Authors and Affiliations

  • Anis Skander Larakeb
    • 1
  • Solène Evrard
    • 2
  • Férielle Louillet
    • 1
  • Thérésa Kwon
    • 1
  • Hadji Djaffar
    • 3
  • Brigitte Llanas
    • 4
  • Georges Deschênes
    • 1
    • 5
  • Marie-Françoise Hurtaud-Roux
    • 2
  • Véronique Baudouin
    • 1
  1. 1.Department of Pediatric NephrologyAssistance Publique-Hôpitaux de Paris, Hôpital Robert DebréParisFrance
  2. 2.Department of Biological HemobiologyAssistance Publique-Hôpitaux de Paris, Hôpital Robert DebréParisFrance
  3. 3.Department of PediatricsHôpital Saint CamilleBry sur MarneFrance
  4. 4.Department of PediatricsHôpital PellegrinBordeauxFrance
  5. 5.University Paris VII-Denis DiderotParisFrance