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.
This is a preview of subscription content, log in via an institution to check access.
References
Felice KJ, DiMario FJ, Conway SR (2005) Post infectious Myasthenia gravis: report of two children. J Child Neurol 20:441–444
Apak H, Karaman S, Doğan Y, Ocak S, Celkan T, Kutlu T, Yildiz I (2006) Varicella-induced hemolytic anemia with hepatitis. Ann Hematol 85:64–65
Rand ML, Wright JF (1998) Virus-associated idiopathic thrombocytopenic purpura. Transfus Sci 19:253–259
Phillips WG, Marsden JR, Hill FG (1992) Purpura fulminans due to protein S deficiency following chickenpox. Br J Dermatol 127:30–32
D’Angelo A, Della Valle P, Crippa L, Pattarini E, Grimaldi LM, Viganò D’Angelo S (1993) Autoimmune protein S deficiency in a boy with severe thromboembolic disease. N Engl J Med 328:1753–1757
Levin M, Eley BS, Louis J, Cohen H, Young L, Heyderman RS (1995) Postinfectious purpura fulminans caused by an autoantibody directed against protein. J Pediatr 127:355–363
Manco-Johnson MJ, Nuss R, Key N, Moertel C, Jacobson L, Meech S, Weinberg A, Lefkowitz J (1996) Lupus anticoagulation and protein S deficiency in children with postvaricella purpura fulminans or thrombosis. J Pediatr 128:319–323
Josephson C, Nuss R, Jacobson L, Hacker MR, Murphy J, Weinberg A, Manco-Johnson MJ (2001) The varicella-autoantibody syndrome. Pediatr Res 50:345–352
Regnault V, Boehlen F, Ozsahin H, Wahl D, de Groot PG, Lecompte T, de Moerloose P (2005) Anti-protein S antibodies following a varicella infection: detection, characterization and influence on thrombin generation. J Thromb Haemost 3:1243–1249
Van Ommen CH, van Wijnen M, de Groot FG, van der Horst CM, Peters M (2002) Postvaricella purpura fulminans caused by acquired protein S deficiency resulting from antiprotein S antibodies: search for the epitopes. J Pediatr Hematol Oncol 24:413–416
Conflict of interest statement
None.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Larakeb, A.S., Evrard, S., Louillet, F. et al. Acute renal cortical necrosis due to acquired antiprotein S antibodies. Pediatr Nephrol 24, 207–209 (2009). https://doi.org/10.1007/s00467-008-0967-y
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00467-008-0967-y