title: Acute renal cortical necrosis due to acquired antiprotein S antibodies.
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.
authors.affiliation: Department of Pediatric Nephrology, Assistance Publique-Hôpitaux de Paris, Hôpital Robert Debré, 48 Boulevard Sérurier, 75019, Paris, France.
authors.forename: Anis Skander
authors.initials: AS
authors.lastname: Larakeb
authors.forename: Solène
authors.initials: S
authors.lastname: Evrard
authors.forename: Férielle
authors.initials: F
authors.lastname: Louillet
authors.forename: Thérésa
authors.initials: T
authors.lastname: Kwon
authors.forename: Hadji
authors.initials: H
authors.lastname: Djaffar
authors.forename: Brigitte
authors.initials: B
authors.lastname: Llanas
authors.forename: Georges
authors.initials: G
authors.lastname: Deschênes
authors.forename: Marie-Françoise
authors.initials: MF
authors.lastname: Hurtaud-Roux
authors.forename: Véronique
authors.initials: V
authors.lastname: Baudouin
mesh_terms: 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
chemical_list: D000925:Anticoagulants; D001323:Autoantibodies; D017984:Enoxaparin; D005938:Glucocorticoids; D017293:Protein S
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