Гемолитико-уремический синдром

Делягин В.М., Плясунова С.А. Гемолитико-уремический синдром. Consilium Medicum. Педиатрия (Прил.). 2015; 3: 23–28.

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Delyagin V.M., Plyasunova S.A. Hemolytic-uremic syndrome. Consilium Medicum. Pediatrics (Suppl.). 2015; 3: 23–28.

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Аннотация

Гемолитико-уремический синдром (ГУС) – заболевание преимущественно детей раннего и младшего возраста, характеризующееся микроангиопатической гемолитической анемией, тромбоцитопенией и острой почечной недостаточностью. Спектр проявлений – от субклинических до жизнеугрожающих. Выделяют постдиарейный ГУС-(D+), типичный вариант, преимущественно педиатрический, наиболее частый; не связанный с диареей ГУС-(D-), атипичный, чаще регистрируемый у взрослых, и пневмококково-обусловленный Р-ГУС. Группа риска – дети младшего возраста; старики, контактирующие с животными, больными, страдающими диареей; обитатели закрытых учреждений, нарушающие гигиену; потребители необработанного мяса, молока, овощей, фруктов. Наряду с почечным синдромом возможны неврологические, кардиальные, абдоминальные нарушения. Лечение – восполнение объема циркулирущей крови, диализ. В практику входит применение моноклональных антител к продуктам комплемента. Обсуждается целесообразность толстокишечного лаважа полиэтиленгликолем. Антибиотикотерапия, антимотилики, адсорбенты противопоказаны. Велика угроза формирования хронической почечной болезни. Летальность в зависимости от социально-экономических условий общества и готовности медицинской службы колеблется от 3 до 72%.

Ключевые слова: гемолитико-уремический синдром, распространенность, диагностика, клиника, лечение, исходы.

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Hemolytic-uremic syndrome (HUS), a disease mostly infants and young children, is characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. The range of manifestations – from subclinical to life-threatening. There are associated with diarrhea HUS-(D +) exemplary embodiment, mainly pediatric, the most common; not associated with diarrhea HUS-(D-) atypical, often detectable in adults and pneumococcal-mediated P-HUS. At risk – young children; the elderly, contact with animals, with patients suffering from diarrhea; inhabitants of closed institutions, violating hygiene; consumers raw meat, milk, vegetables, fruits. Along with renal syndrome may be neurological, cardiac, abdominal disorders. Treatment – restores blood volume, dialysis. In practice includes the use of monoclonal antibodies to the products of complement. We discuss the feasibility of colonic lavage with polyethylene glycol. Antibiotic, antimotilik, adsorbents are contraindicated. Great danger of developing chronic kidney disease. Mortality according to the socio-economic conditions of society and the willingness of the medical service ranges from 3 to 72%.

Keywords: hemolytic uremic syndrome, prevalence, diagnosis, clinical picture, treatment, outcomes.

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Список литературы

1. Gasser C, Gattier E, Steck A et al. Hämolytisch urämische syndrome: Bilaterale Nierenridennekrosen bei akuten erworbenen hämolytischen Anämien. Schweiz Med Wochenschr 1955; 85 (38–39): 905–9.
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8. Centers for Disease Control and Prevention (CDC). National enteric disease surveillance: Shiga toxin-producing Escherichia coli (STEC) annual report, 2011. January 22, 2013. http://www.cdc.gov/ncezid/dfwed/pdfs/national-stec-surv-summ-2011-508c.pdf. Accessed August 11, 2014.
9. Doulgere J, Otto B, Nassour M, Wolters-Eisfeld G et al. Soluble plasma VE-cadherin concentrations are elevated in patients with STEC infection and haemolytic uraemic syndrome: a case-control study. BMJ Open 2015; 5(3): e005659. Doi: 10.1136/bmjopen-2014-005659. 10. Uslu-Gökceoglu A, Dogan C et al. Atypical Hemolytic Uremic Syndrome due to Factor H Autoantibody. Turk J Pediatr 2013; 55 (1): 86–9.
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12. Spinale J, Ruebner R, Kaplan B, Copelovitch L. Update on Streptococcus pneumoniae associated hemolytic uremic syndrome. Curr Opin Pediatr 2013; 25 (2): 203–8.
13. Maxvold N, Smoyer W, Custer J, Bunchman T. Amino acid loss and nitrogen balance in critically ill children with acute renal failure: a prospective comparison between classic hemofiltration and hemofiltration with dialysis. Crit Care Med 2000; 28 (4): 1161–5.
14. Ake J, Jelacic S, Ciol M et al. Relative nephroprotection during Escherichia coli O157:H7 infections: association with intravenous volume expansion. Pediatrics 2005; 115 (6): e673–680.
15. Lüth S, Fründt T, Rösch T et al. Prevention of hemolytic uremic syndrome with daily bowel lavage in patients with Shiga toxin-producing enterohemorrhagic Escherichia coli O104:H4 infection. JAMA Intern Med 2014; 174: 1003–5.
16. Gillespie R, Seidel K, Symons J. Effect of fluid overload and dose of replacement fluid on survival in hemofiltration. Pediatr Nephrol 2004; 19 (12): 1394–9.
17. Tolwani A. Continuous renal-replacement therapy for acute kidney injury. N Engl J Med 2012; 367 (26): 2505–14.
18. Lapeyraque A-L, Malina M, Freneaux-Bacchi V, Boppel T. Eculizumab in Severe Shiga-Toxin-Associated HUS. N Engl J Med 2011; 364 (26): 2561–3.
19. Murphy E. Acute pain management pharmacology for the patient with concurrent renal or hepatic disease. Anaesth Intensive Care 2005; 33 (3): 311–22.
20. Dean M. Opioids in renal failure and dialysis patients. J Pain Symptom Manage 2004; 28 (5): 497–504.
21. Wong C, Jelacic S, Habeeb R et al. The risk of the hemolytic-uremic syndrome after antibiotic treatment of Escherichia coli O157:H7 infections. N Engl J Med 2000; 342 (26): 1930–6.
22. Scheiring J, Andreoli S, Zimmerhackl L. Treatment and outcome of Shiga-toxin-associated hemolytic uremic syndrome (HUS). Pediatr Nephrol 2008; 23 (10): 1749–60.
23. Ariceta G, Besbas N, Johnson S et al. Guideline for the investigation and initial therapy of diarrhea-negative hemolytic uremic syndrome. Pediatr Nephrol 2009; 24 (4): 687–96.
24. Michael M, Elliott E, Craig J et al. Interventions for hemolytic uremic syndrome and thrombotic thrombocytopenic purpura: a systematic review of randomized controlled trials. Am J Kidney Dis 2009; 53 (2): 259–72.
25. Gruppo R, Rother R. Eculizumab for congenital atypical hemolytic-uremic syndrome. N Engl J Med 2009; 360 (5): 544–6.
26. Mejias M, Ghersi G, Craig P et al. Immunization with a chimera consisting of the B subunit of Shiga toxin type 2 and brucella lumazine synthase confers total protection against Shiga toxins in mice. J Immunol 2013; 191 (5): 2403–11.
27. Kavanach D, Goodship T, Richards A. Atypical hemolytic uremic syndrome. Seminars Nephr 2013; 33 (6): 508–30.

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1. Gasser C, Gattier E, Steck A et al. Hämolytisch urämische syndrome: Bilaterale Nierenridennekrosen bei akuten erworbenen hämolytischen Anämien. Schweiz Med Wochenschr 1955; 85 (38–39): 905–9.
2. Andrianova O.I., Manerov F.K., Churljaev Ju.A., Hamin I.G. Prichiny i lechenie ostroj pochechnoj nedostatochnosti u detej. Obshh. reanimatologija. 2007; 3 (4): 70–5. [in Russian]
3. Gillespi R. Pediatric Hemolytic Uremic Syndrome. Updated: Jun 26, 2013. eMedicine. http://emedicine.medscape.com/article/982025-overview
4. Repina I.B. Osobennosti gemolitiko-uremicheskogo sindroma pri infekcionnyh boleznjah u detej. Avtoref. dis. … kand. med. nauk. M., 1997. [in Russian]
5. Zverev D.V. Gemolitiko-uremicheskij sindrom u detej: jeffektivnost' peritoneal'nogo dializa. Nefrologija i dializ. 2000; 2 (1–2): 95–6. [in Russian]
6. Trofimov A.A. Narushenie gemostaza i lechenie gemolitiko-uremicheskogo sindroma u detej. Avtoref. dis. … kand. med. nauk. Ekaterinburg, 2003. [in Russian]
7. Noris M, Remuzzi G. Hemolytic uremic syndrome. J Am Soc Nephrol 2005; 16 (4): 1035–50.
8. Centers for Disease Control and Prevention (CDC). National enteric disease surveillance: Shiga toxin-producing Escherichia coli (STEC) annual report, 2011. January 22, 2013. http://www.cdc.gov/ncezid/dfwed/pdfs/national-stec-surv-summ-2011-508c.pdf. Accessed August 11, 2014.
9. Doulgere J, Otto B, Nassour M, Wolters-Eisfeld G et al. Soluble plasma VE-cadherin concentrations are elevated in patients with STEC infection and haemolytic uraemic syndrome: a case-control study. BMJ Open 2015; 5(3): e005659. Doi: 10.1136/bmjopen-2014-005659. 10. Uslu-Gökceoglu A, Dogan C et al. Atypical Hemolytic Uremic Syndrome due to Factor H Autoantibody. Turk J Pediatr 2013; 55 (1): 86–9.
11. Delvaeye M, Noris M, De Vriese A et al. Thrombomodulin mutations in atypical hemolytic-uremic syndrome. N Engl J Med 2009; 361(4): 345–57.
12. Spinale J, Ruebner R, Kaplan B, Copelovitch L. Update on Streptococcus pneumoniae associated hemolytic uremic syndrome. Curr Opin Pediatr 2013; 25 (2): 203–8.
13. Maxvold N, Smoyer W, Custer J, Bunchman T. Amino acid loss and nitrogen balance in critically ill children with acute renal failure: a prospective comparison between classic hemofiltration and hemofiltration with dialysis. Crit Care Med 2000; 28 (4): 1161–5.
14. Ake J, Jelacic S, Ciol M et al. Relative nephroprotection during Escherichia coli O157:H7 infections: association with intravenous volume expansion. Pediatrics 2005; 115 (6): e673–680.
15. Lüth S, Fründt T, Rösch T et al. Prevention of hemolytic uremic syndrome with daily bowel lavage in patients with Shiga toxin-producing enterohemorrhagic Escherichia coli O104:H4 infection. JAMA Intern Med 2014; 174: 1003–5.
16. Gillespie R, Seidel K, Symons J. Effect of fluid overload and dose of replacement fluid on survival in hemofiltration. Pediatr Nephrol 2004; 19 (12): 1394–9.
17. Tolwani A. Continuous renal-replacement therapy for acute kidney injury. N Engl J Med 2012; 367 (26): 2505–14.
18. Lapeyraque A-L, Malina M, Freneaux-Bacchi V, Boppel T. Eculizumab in Severe Shiga-Toxin-Associated HUS. N Engl J Med 2011; 364 (26): 2561–3.
19. Murphy E. Acute pain management pharmacology for the patient with concurrent renal or hepatic disease. Anaesth Intensive Care 2005; 33 (3): 311–22.
20. Dean M. Opioids in renal failure and dialysis patients. J Pain Symptom Manage 2004; 28 (5): 497–504.
21. Wong C, Jelacic S, Habeeb R et al. The risk of the hemolytic-uremic syndrome after antibiotic treatment of Escherichia coli O157:H7 infections. N Engl J Med 2000; 342 (26): 1930–6.
22. Scheiring J, Andreoli S, Zimmerhackl L. Treatment and outcome of Shiga-toxin-associated hemolytic uremic syndrome (HUS). Pediatr Nephrol 2008; 23 (10): 1749–60.
23. Ariceta G, Besbas N, Johnson S et al. Guideline for the investigation and initial therapy of diarrhea-negative hemolytic uremic syndrome. Pediatr Nephrol 2009; 24 (4): 687–96.
24. Michael M, Elliott E, Craig J et al. Interventions for hemolytic uremic syndrome and thrombotic thrombocytopenic purpura: a systematic review of randomized controlled trials. Am J Kidney Dis 2009; 53 (2): 259–72.
25. Gruppo R, Rother R. Eculizumab for congenital atypical hemolytic-uremic syndrome. N Engl J Med 2009; 360 (5): 544–6.
26. Mejias M, Ghersi G, Craig P et al. Immunization with a chimera consisting of the B subunit of Shiga toxin type 2 and brucella lumazine synthase confers total protection against Shiga toxins in mice. J Immunol 2013; 191 (5): 2403–11.
27. Kavanach D, Goodship T, Richards A. Atypical hemolytic uremic syndrome. Seminars Nephr 2013; 33 (6): 508–30.

Авторы

В.М.Делягин*1,2, С.А.Плясунова1

1 ФГБУ Федеральный научно-клинический центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева Минздрава России. 117997, Россия, Москва, ул. Саморы Машела, д. 1, ГСП-7;
2 ГБОУ ВПО Российский национальный исследовательский медицинский университет им. Н.И.Пирогова Минздрава России. 117997, Россия, Москва, ул. Островитянова, д. 1
*delyagin-doktor@yandex.ru

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V.M.Delyagin*1,2, S.A.Plyasunova1

1 Dmitriy Rogachev Federal Research Center of Pediatric Hematology, Oncology and Immunology of the Ministry of Health of the Russian Federation. 117997, Russian Federation, Moscow, ul. Samory Mashela, d. 1, GSP-7;
2 N.I.Pirogov Medical University of the Ministry of Health of the Russian Federation. 117997, Russian Federation, Moscow, ul. Ostrovitianova, d. 1
*delyagin-doktor@yandex.ru

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