Acute renal cortical necrosis due to acquired antiprotein S antibodies.
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.
Larakeb
Anis Skander
AS
Department of Pediatric Nephrology, Assistance Publique-Hôpitaux de Paris, Hôpital Robert Debré, 48 Boulevard Sérurier, 75019, Paris, France.
Evrard
Solène
S
Louillet
Férielle
F
Kwon
Thérésa
T
Djaffar
Hadji
H
Llanas
Brigitte
B
Deschênes
Georges
G
Hurtaud-Roux
Marie-Françoise
MF
Baudouin
Véronique
V
D000925:Anticoagulants / Q000627:therapeutic use; D001323:Autoantibodies / Q000276:immunology*; D002644:Chickenpox / Q000150:complications* / Q000473:pathology; D017984:Enoxaparin / Q000627:therapeutic use; D005938:Glucocorticoids / Q000008:administration & dosage / Q000627:therapeutic use; D006801:Humans; D007223:Infant; D007668:Kidney / Q000000981:diagnostic imaging / Q000473:pathology; D007673:Kidney Cortex Necrosis / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy; D008279:Magnetic Resonance Imaging; D008297:Male; D010956:Plasmapheresis; D017293:Protein S / Q000276:immunology*; D018455:Protein S Deficiency / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy; D020551:Pulse Therapy, Drug; D016896:Treatment Outcome; D014463:Ultrasonography
D000925:Anticoagulants; D001323:Autoantibodies; D017984:Enoxaparin; D005938:Glucocorticoids; D017293:Protein S
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