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High Frequency of Acquired ADAMTS13 Deficiency After Hemolysis in Hemiscorpius Lepturus (Scorpion) Stung Children

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Abstract

Objective

To estimate the frequency of acquired ADAMTS13 deficiency in severe cases of Hemiscorpius lepturus stung patients and the frequency of acute kidney injury (AKI) in these patients.

Methods

Sixty scorpion stung children who were referred with severe hemolysis and hemoglobinuria were studied. None of them had received blood products and no one had a past medical history of renal failure.

Results

Plasma levels of ADAMTS13 and ADAMTS13 antibody (IgG) were measured using ELISA. ADAMTS13 was decreased in 91.7 % of patients and the anti-ADAMTS13 antibody (Ab) was increased in 98.3 %. ADAMTS13 decreased in all of the patients with acute kidney injury and none of those with normal levels of ADAMTS13 developed renal failure; all patients with AKI had also increased levels of ADAMTS13Ab. Acute kidney injury was found in 23.3 % and had significant association with severe anemia, thrombocytopenia, pyuria, hematuria and considerable proteinuria (p < 0.001). Disseminated intravascular coagulation (DIC) and Hemolytic uremic syndrome (HUS) developed in 6.7 % and 10 % respectively.

Conclusions

The index findings demonstrate that Hemiscorpius lepturus sting is usually associated with ADAMTS13 deficiency, and increased ADAMTS13 autoantibody. These combined mechanisms may contribute to scorpion sting-induced coagulopathies and may predispose patients to develop DIC and HUS.

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References

  1. Fujikawa K, Suzuki H, McMullen B, Chung D. Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family. Blood. 2001;98:1662–6.

    Article  CAS  PubMed  Google Scholar 

  2. Zheng X, Chung D, Takayama TK, Majerus EM, Sadler JE, Fujikawa K. Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura. J Biol Chem. 2001;276:41059–63.

    Article  CAS  PubMed  Google Scholar 

  3. Doldan-Silvero A, Acevedo-Gadea C, Habib C, Freeman J, Johari V. ADAMTS13 activity and inhibitor. Am J Hematol. 2008;83:811–4.

    Article  CAS  PubMed  Google Scholar 

  4. Tsai HM, Lian EC. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Eng J Med. 1998;339:1585–94.

    Article  CAS  Google Scholar 

  5. Feys HB, Deckmyn H, Vanhoorelbeke K. ADAMTS13 in health and disease. Acta Haematologica. 2009;121:183–5.

    Article  CAS  PubMed  Google Scholar 

  6. Franchini M, Mannucci PM. Advantages and limits of ADAMTS13 testing in thrombotic thrombocytopenic purpura. Blood Transfus. 2008;6:127–35.

    Google Scholar 

  7. Yamashita E, Okada H, Yorioka H, Fujita S, Nishi K, Komiyama Y, et al. Successful management of pregnancy-associated thrombotic thrombocytopenic purpura by monitoring ADAMTS13 activity. J Obstet Gynaecol Res. 2012;38:567–9.

    Article  PubMed  Google Scholar 

  8. Thampi S, Salmi D, Imashuku S, Ducore J, Satake N. Thrombotic thrombocytopenic purpura in a child with systemic lupus erythematosus. J Pediatr Hematol Oncol. 2011;33:221–3.

    Article  PubMed  Google Scholar 

  9. Valavi E, Ansari MJ, Hoseini S. ADAMTS-13 deficiency following Hemiscorpius lepturus scorpion sting. Saudi J Kidney Dis Transpl. 2011;22:792–5.

    PubMed  Google Scholar 

  10. Malbranque S, Piercecchi-Marti MD, Thomas L, Barbey C, Courcier D, Bucher B, et al. Fatal diffuse thrombotic microangiopathy after a bite by the “Fer-de-Lance” pit viper (Bothrops lanceolatus) of Martinique. Am J Trop Med Hyg. 2008;78:856–61.

    Google Scholar 

  11. de Mast Q, Groot E, Asih PB, Syafruddin D, Oosting M, Sebastian S, et al. ADAMTS13 deficiency with elevated levels of ultra-large and active von Willebrand factor in P. falciparum and P. vivax malaria. Am J Trop Med Hyg. 2009;80:492–8.

    Google Scholar 

  12. Pipelzadeh MH, Jalali A, Taraz M, Pourabbas R, Zaremirakabadi A. An epidemiological and a clinical study on scorpionism by the Iranian scorpion Hemiscorpius lepturus. Toxicon. 2007;50:984–92.

    Article  CAS  PubMed  Google Scholar 

  13. Valavi E, Ansari MJ. Hemolytic uremic syndrome following Hemiscorpius lepturus (scorpion) sting. Indian J Nephrol. 2008;18:166–8.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  14. Mehta RL, Kellum JA, Shah SV, Molitoris BA, Ronco C, Warnock DG, et al; Acute Kidney Injury Network. Acute Kidney Injury Network: Report of an initiative to improve outcomes in acute kidney injury. Crit Care. 2007;11:R31.

    Google Scholar 

  15. Kremer Hovinga JA, Zeerleder S, Kessler P, Romani de Wit T, van Mourik JA, Hack CE, et al. ADAMTS-13, von Willebrand factor and related parameters in severe sepsis and septic shock. J Thromb Haemost. 2007;5:2284–90.

    Article  CAS  PubMed  Google Scholar 

  16. Chion CK, Doggen CJ, Crawley JT, Lane DA, Rosendaal FR. ADAMTS13 and von Willebrand factor and the risk of myocardial infarction in men. Blood. 2007;109:1998–2000.

    Article  CAS  PubMed  Google Scholar 

  17. Matsuyama T, Uemura M, Ishikawa M, Matsumoto M, Ishizashi H, Kato S, et al. Increased von Willebrand factor over decreased ADAMTS13 activity may contribute to the development of liver disturbance and multiorgan failure in patients with alcoholic hepatitis. Alcohol Clin Exp Res. 2007;31:S27–35.

    Article  PubMed  Google Scholar 

  18. Stepanian A, Cohen-Moatti M, Sanglier T, Legendre P, Ameziane N, Tsatsaris V, et al. Von Willebrand factor and ADAMTS13: A candidate couple for preeclampsia pathophysiology. Arterioscler Thromb Vasc Biol. 2011;31:1703–9.

    Article  CAS  PubMed  Google Scholar 

  19. Austin SK, Starke RD, Lawrie AS, Cohen H, Machin SJ, Mackie IJ. The VWF/ADAMTS13 axis in the antiphospholipid syndrome: ADAMTS13 antibodies and ADAMTS13 dysfunction. Br J Haematol. 2008;141:536–44.

    Article  CAS  PubMed  Google Scholar 

  20. Feys HB, Canciani MT, Peyvandi F, Deckmyn H, Vanhoorelbeke K, Mannucci PM. ADAMTS13 activity to antigen ratio in physiological and pathological conditions associated with an increased risk of thrombosis. Br J Haematol. 2007;138:534–40.

    Article  CAS  PubMed  Google Scholar 

  21. Boyer O, Niaudet P. Hemolytic uremic syndrome: New developments in pathogenesis and treatment. Int J Nephrol. 2011;2011:908407.

    Article  PubMed Central  PubMed  Google Scholar 

  22. Moake JL. von Willebrand factor, ADAMTS-13, and thrombotic thrombocytopenic purpura. Semin Hematol. 2004;41:4–14.

    Article  CAS  PubMed  Google Scholar 

  23. Coppo P, Veyradier A. Thrombotic microangiopathies: Towards a pathophysiology-based classification. Cardiovasc Hematol Disord Drug Targets. 2009;9:36–50.

    Article  CAS  PubMed  Google Scholar 

  24. Studt JD, Kremer Hovinga JA, Antoine G, Hermann M, Rieger M, Scheiflinger F, et al. Fatal congenital thrombotic thrombocytopenic purpura with apparent ADAMTS13 inhibitor: In vitro inhibition of ADAMTS13 activity by hemoglobin. Blood. 2005;105:542–4.

    Article  CAS  PubMed  Google Scholar 

  25. Motto D. Hemoglobin versus ADAMTS13. Blood. 2005;105:436–7.

    Article  CAS  Google Scholar 

  26. Mannucci PM, Capoferri C, Canciani MT. Plasma levels of von Willebrand factor regulate ADAMTS-13, its major cleaving protease. Br J Haematol. 2004;126:213–8.

    Article  CAS  PubMed  Google Scholar 

  27. Bernardo A, Ball C, Nolasco L, Moake JF, Dong JF. Effects of inflammatory cytokines on the release and cleavage of the endothelial cell-derived ultralarge von Willebrand factor multimers under flow. Blood. 2004;104:100–6.

    Article  CAS  PubMed  Google Scholar 

  28. McDonald V, Liesner R, Grainger J, Gattens M, Machin SJ, Scully M. Acquired, noncongenital thrombotic thrombocytopenic purpura in children and adolescents: Clinical management and the use of ADAMTS13 assays. Blood Coagul Fibrinolysis. 2010;21:245–50.

    Article  PubMed  Google Scholar 

  29. Fakhouri F, Vernant JP, Veyradier A, Wolf M, Kaplanski G, Binaut R, et al. Efficiency of curative and prophylactic treatment with rituximab in ADAMTS13-deficient thrombotic thrombocytopenic purpura: A study of 11 cases. Blood. 2005;106:1932–7.

    Google Scholar 

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Acknowledgements

The authors thank the mothers, children and families that participated; the nurses, supervisors and field staff of nephrology ward for their devotion and hard work.

Conflict of Interest

None.

Role of Funding Source

Vice-chancellor of the research center of Ahvaz Jundishapur University of Medical Sciences (No: U-90027).

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Authors and Affiliations

  1. Division of Pediatric Nephrology, Abuzar Children’s Hospital, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, 6163933874, Iran

    Ehsan Valavi, Ali Ahmadzadeh & Parisa Amoori

  2. Department of Pediatrics, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran

    Aidin Daneshgar

Authors
  1. Ehsan Valavi
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  2. Ali Ahmadzadeh
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  3. Parisa Amoori
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  4. Aidin Daneshgar
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Correspondence to Ehsan Valavi.

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Valavi, E., Ahmadzadeh, A., Amoori, P. et al. High Frequency of Acquired ADAMTS13 Deficiency After Hemolysis in Hemiscorpius Lepturus (Scorpion) Stung Children. Indian J Pediatr 81, 665–669 (2014). https://doi.org/10.1007/s12098-013-1089-5

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  • DOI: https://doi.org/10.1007/s12098-013-1089-5

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