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Difficulties in diagnostics of atypical hemolytic uremic syndrome in the intensive care unit

https://doi.org/10.21292/2078-5658-2019-16-4-65-76

Abstract

Atypical hemolytic uremic syndrome (aHUS) is an orphan disease, representing one of thrombotic microangiopathies, the natural course of which leads to an unfavorable outcome often accompanied by multiple organ failure in the onset of the disease. Anesthesiologists and emergency physicians play an important role in the multi-disciplinary team of specialists managing such patients. However, this disease is rare and its symptoms are similar to the ones of some other disorders including sepsis and disseminated intravascular coagulation syndrome, thus often aHUS is diagnosed late and specific treatment is not started in time. This article aims to present main notions related to thrombotic microangiopathies and discuss topical issues of differential diagnostics of aHUS in the intensive care units giving two clinical cases as an example. 

About the Authors

N. L. Kozlovskаya
City Clinical Hospital named after A.K. Eramishantsev; Peoples’ Friendship University of Russia
Russian Federation

Natalya L. Kozlovskaya - Doctor of Medical Sciences, Professor, Head of the Center for Pregnant Women with Renal and Urinary Tract Pathology.

15, Lenskaya St., Moscow, 129327


G. M. Gаlstyan
National Medical Research Center of Hematology
Russian Federation

Gennady M. Galstyan - Doctor of Medical Sciences, Head of Anesthesiology and Intensive Care Department.

4, Noby Zykovskiy Lane, Moscow, 125167, Phone.: +7 (495) 612‒48‒59


V. N. Stepаnyuk
Primorskaya Regional Clinical Hospital no. 1
Russian Federation

Vladimir N. Stepanyuk - Anesthesiologist and Emergency Physician of Primorsky Anesthesiology and Intensive Care Center.

57, Aleutskaya St., Vladivostok, 690091, Phone/Fax: +7 (4232) 40‒05‒19



References

1. Kozlovskaya N., Prokopenko E., Emirova Kh. et al. Clinical recommendation for diagnostics and treatment of atypical hemolytic uremic syndrome. Nefrologiya i Dializ, 2015, vol. 17, no. 3, pp. 242-264. (In Russ.)

2. Polushin Yu.S., Shlyk I.V., Dobronravov V.А. Atypical hemolytic uremic syndrome - is it something exotic for intensive care unit or not? Vestnik Anesteziologii I Reanimatologii, 2016, no. 3, pp. 71-73. (In Russ.)

3. Azoulay E., Knoebl P., Garnacho-Montero J. et al. Expert statements on the standard of care in critically ill adult patients with atypical hemolytic uremic syndrome. Chest, 2017, vol. 152, no. 2, pp. 424-434.

4. Bommer M., Wölfle-Guter M., Bohl S. et al. The differential diagnosis and treatment of thrombotic microangiopathies. Deutsches Arzteblatt International, 2018, vol. 115, pp. 327-334.

5. Bresin E., Pinto S., Goodship T.H.J. et al. Combined complement gene mutations in atypical hemolytic uremic syndrome influence clinical phenotype. J. Amer. Society Nephrol., 2013, vol. 24, no. 3, pp. 475-486.

6. Campistol J.M., Arias M., Ariceta G. et al. An update for atypical haemolytic uraemic syndrome: diagnosis and treatment. A consensus document. Nefrologia, 2013, vol. 33, no. 1, pp. 27-45.

7. Coppo P. Predictive features of severe acquired ADAMTS13 deficiency in idiopathic thrombotic microangiopathies: The French TMA reference center experience. PLoS ONE, 2010, vol. 5, no. 4, pp. e10208. DOI:https://doi.org/10.1371/journal.pone.0010208.

8. Coppo P., Adrie C., Azoulay E. et al. Infectious diseases as a trigger in thrombotic microangiopathies in intensive care unit (ICU) patients? Intens. Care Med., 2003, vol. 29, pp. 564-569.

9. Douglas K.W., Pollock K.G.J., Young D. et al. Infection frequently triggers thrombotic microangiopathy in patients with preexisting risk factors: A single-institution experience. J. Clin. Apheresis, 2010, vol. 25, no. 2, pp. 47-53.

10. Duburcq T., Tournoys A., Gnemmi V. et al. Impact of obesity on endotoxin-induced disseminated intravascular coagulation. Shock, 2015, vol. 44, no. 4, pp. 341-347.

11. Erickson Y.O., Samia N.I., Bedell B. et al. Elevated procalcitonin and C-reactive protein as potential biomarkers of sepsis in a subpopulation of thrombotic microangiopathy patients. J. Clin. Apheresis, 2009, vol. 24, no. 4, pp. 150-154.

12. Fakhouri F., Zuber J., Frémeaux-Bacchi V. et al. Haemolytic uraemic syndrome. Lancet, 2017, vol. 390, pp. 681-696.

13. Fremeaux-Bacchi V. Genetics and outcome of atypical hemolytic uremic syndrome: A nationwide french series comparing children and adults. Clin. J. Amer. Society Nephrol., 2013, vol. 8, no. 4, pp. 554-562.

14. Gando S., Levi M. and Toh C.-H. Disseminated Intravascular Coagulation. Nat Rev Dis Primers, 2016, vol. 2, no. 2, pp. doi: https://doi.org/10.1016/B978-0-12-385157-4.00327-4.

15. George J.N., Nester C.M. Syndromes of Thrombotic Microangiopathy. New Engl. J. Med., 2014, vol. 371, pp. 654-666.

16. Gupta M., Feinberg B.B., Burwick R.M. Thrombotic microangiopathies of pregnancy: Differential diagnosis. Pregnancy Hypertension, 2018, vol. 12, pp. 29-34.

17. Java A., Edwards A., Rossi A et al. Cytomegalovirus-induced thrombotic microangiopathy after renal transplant successfully treated with eculizumab: Case report and review of the literature. Transpl. Int., 2015, vol. 28, no. 9, pp. 1121-1125.

18. Jodele S., Dandoy C.E., Danziger-Isakov L. et al. Terminal complement blockade after hematopoietic stem cell transplantation is safe without meningococcal vaccination. Biol. Blood Marrow Transplant., 2016, vol. 22, no. 7, pp. 1337-1340.

19. Larsen F.F. and Petersen J.A. Novel biomarkers for sepsis: A narrative review. Eur. J. Intern. Med., 2017, vol. 45, pp. 46-50.

20. Laudes I.J., Chu J.C., Sikranth S. et al. Anti-C5a ameliorates coagulation/fibrinolytic protein changes in a rat model of sepsis. Am. J. Pathology, 2002, vol. 160, no. 5, pp. 1867-1875.

21. Laurence J., Haller H., Mannucci P.M. et al. Atypical hemolytic uremic syndrome (ahus): essential aspects of an accurate diagnosis. Clin. Advan. Hematol. Oncol., 2016, vol. 14, no. 11, pp. 2-15.

22. Levi M., Toh C.H., Thachil J. et al. Guidelines for the diagnosis and management of disseminated intravascular coagulation. Brit. J. Haematol., 2009, vol. 145, pp. 24-33.

23. Noris M. Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype. Clin. J. Am. Society Nephrol., 2010, vol. 5, no. 10, pp. 1844-1859.

24. Orpha-net 2019. Atypical hemolytic-uremic syndrome. Portal for rare diseasse and orphan drugs. (2019). doi: https://doi.org/.1037//0033-2909.I26.1.78.

25. Pène F., Papo T., Brudy-Gulphe L. et al. Septic shock and thrombotic microangiopathy

26. due to mycobacterium tuberculosis in a nonimmunocompromised patient. Arch. Intern. Med., 2001, vol. 161, pp. 1347-1348.

27. Van der Poll T., de Jonge E., Levi M. Regulatory role of cytokines in disseminated intravascular coagulation. Semin. Thrombosis and Haemostasis, 2001, vol. 27, no. 6, pp. 639-651.

28. Prucha M., Bellingan G., Zazula R. Sepsis biomarkers. Clin. Chim. Acta., 2015, vol. 440, pp. 97-103.

29. Regal J.F., Gilbert J.S., Burwick R.M. The complement system and adverse pregnancy outcomes. Molec. Immunol., 2015, vol. 67, no. 1, pp. 56-70.

30. Salvadori M. Update on hemolytic uremic syndrome: Diagnostic and therapeutic recommendations. World J. Nephrol., 2013, vol. 2, no. 3, pp. 56-76.

31. Sarmiento M., Balcells M.E., Ramirez P. Thrombotic microangiopathy as first manifestation of acute human immunodeficiency virus infection: A case report and review of the literature. J. Med. Case Reports, 2016, vol. 10, pp. 152.

32. Seymour C.W., Liu V.X., Iwashyna T.J. et al. Assessment of clinical criteria for sepsis for the third international consensus definitions for sepsis and septic shock (sepsis-3). JAMA, 2016, vol. 315, no. 8, pp. 762-774.

33. Sircar M., Thadhani R., Karumanchi S. A. Pathogenesis of preeclampsia. Cur. Opin. Nephrol. Hypertens., 2015, vol. 24, pp. 31-138.

34. Taylor Jr.F., Toh C.H., Hoots W.K. et al. Towards definition, clinical and laboratory criteria, and a scoring system for disseminated intravascular coagulation. Thromb. Haemost., 2001, vol. 86, pp. 1327-1330.

35. Vincent J.L., Castro P., Hunt B.J. et al. Thrombocytopenia in the ICU: disseminated intravascular coagulation and thrombotic microangiopathies – what intensivists need to know. Crit. Care, 2018, vol. 22, no. 1, pp. 158,

36. Wada H., Matsumoto T., Suzuki K. et al. Differences and similarities between disseminated intravascular coagulation and thrombotic microangiopathy. Thrombosis J., 2018, vol. 16, no. 14, doi: https://doi.org/10.1186/s12959-018-0168-2.

37. Williams L.A., Marques M.B. Pathology consultation on the diagnosis and treatment of thrombotic microangiopathies (TMAs). Am. J. Clin. Pathol., 2016, vol. 145, pp. 158-165.

38. Zhaoyue W., Ziqiang Y., Jian S. et al. Sepsis-induced disseminated intravascular coagulation with features of thrombotic thrombocytopenic purpura: A fatal fulminant syndrome. Clin. Applied Thrombosis/Hemostasis, 2011, vol. 17, no. 3, pp. 251-253.


Review

For citations:


Kozlovskаya N.L., Gаlstyan G.M., Stepаnyuk V.N. Difficulties in diagnostics of atypical hemolytic uremic syndrome in the intensive care unit. Messenger of ANESTHESIOLOGY AND RESUSCITATION. 2019;16(4):65-76. (In Russ.) https://doi.org/10.21292/2078-5658-2019-16-4-65-76



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