Acute renal cortical necrosis due to acquired antiprotein S antibodies |
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Authors: | Anis Skander Larakeb Solène Evrard Férielle Louillet Thérésa Kwon Hadji Djaffar Brigitte Llanas Georges Deschênes Marie-Françoise Hurtaud-Roux Véronique Baudouin |
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Institution: | 1. Department of Pediatric Nephrology, Assistance Publique-H?pitaux de Paris, H?pital Robert Debré, 48 Boulevard Sérurier, 75019, Paris, France 2. Department of Biological Hemobiology, Assistance Publique-H?pitaux de Paris, H?pital Robert Debré, 75019, Paris, France 3. Department of Pediatrics, H?pital Saint Camille, 94360, Bry sur Marne, France 4. Department of Pediatrics, H?pital Pellegrin, 33000, Bordeaux, France 5. University Paris VII-Denis Diderot, 75010, Paris, France
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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. |
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Keywords: | Acute renal failure Antiprotein S antibody Plasma exchange Protein S deficiency Varicella |
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