gastro-jРоссийский журнал гастроэнтерологии, гепатологии, колопроктологииRussian Journal of Gastroenterology, Hepatology, Coloproctology1382-43762658-6673«Gastro» LLC10.22416/1382-4376-2016-26-1-44-53gastro-j-20Research ArticleГЕПАТОЛОГИЯHEPATOLOGYПеченочная энцефалопатия: патогенез, клиника, диагностика, терапияHepatic encephalopathy: pathogenesis, clinical presentation, diagnostics, treatmentПавловЧ. С.PavlovCh. S.chpavlov@mail.ruДамулинИ. В.DamulinI. V.damulin@mmascience.ruИвашкинВ. Т.IvashkinV. T.noemail@neicon.ruНаучно-образовательный клинический центр «Инновационная терапия» ГБОУ ВПО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава РФ, г. МоскваРоссияScientific and educational clinical center of innovative therapy, State educational government-financed institution of higher professional education «Sechenov First Moscow state medical university», Ministry of Healthcare of the Russian Federation, MoscowRussian FederationГБОУ ВПО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава РФ, г. Москва, Российская ФедерацияРоссияState educational government-financed institution of higher professional education «Sechenov First Moscow state medical university», Ministry of Healthcare of the Russian Federation, Moscow, the Russian FederationRussian Federation2016120820162614453Copyright © Павлов Ч.С., Дамулин И.В., Ивашкин В.Т., 20162016Павлов Ч.С., Дамулин И.В., Ивашкин В.Т.Pavlov C.S., Damulin I.V., Ivashkin V.T.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.gastro-j.ru/jour/article/view/20

Цель обзора. Отразить современные представления о патогенезе, клинических проявлениях, диагностике и лечении печеночной энцефалопатии (ПЭ). Основные положения. ПЭ - комплекс нейропсихических расстройств, возникающих на фоне тяжелых поражений печени, в основе развития которых лежат шунтирование портальной крови и нарушение дезинтоксикационной функции. Мнения экспертов о ведущем механизме патогенеза ПЭ расходятся. Некоторые считают, что ведущую роль играет влияние гипераммониемии на нейрональные функции, другие основное значение придают изменению аминокислотного состава и нейротрансмиттерным нарушениям, третьи больше внимания уделяют повышению концентрации меркаптана и уровня короткоцепочечных жирных кислот, изменению функции ГАМК-бензодиазепинового комплекса. ПЭ характеризуется гетерогенностью проявлений. В клинической картине доминируют когнитивные (варьирующие от минимальных изменений до комы) и двигательные (ригидность мышц, затрудненная речь, тремор и др.) нарушения. Направления терапевтических воздействий при ПЭ следующие: выявление и устранение факторов, вызывающих повреждение печени и провоцирующих развитие/нарастание ПЭ; уменьшение образования и абсорбции аммиака или увеличение его элиминации. При часто рецидивирующей и прогрессирующей печеночной недостаточности выполняют трансплантацию печени. Заключение. Поражение центральной нервной системы при печеночной недостаточности - важнейшая проблема как в отношении своевременной и правильной диагностики, лечения, так и в плане социальной адаптации пациентов. Многие аспекты ПЭ, в частности ее патогенез, недостаточно изучены.

The aim of review. To present modern concept on pathogenesis, clinical presentation, diagnostics and treatment of hepatic encephalopathy (HE). Summary. HE is a complex of neurological and psychiatric disorders that develop on a background of severe liver diseases due to portal blood shunting and impaired liver detoxication function. Expert opinions on the leading mechanism of HE pathogenesis are contradictory. Some believe, that the leading part is played by the effect of hyperammoniemia on neuronal functions; the others give major importance to the change in aminoacid spectrum and neurotransmitter disorders; the third give the most attention to elevation of mercaptan concentration and level of shortchain fatty acids, change of GABA-benzodiazepine complex function. HE is characterized by heterogeneity of manifestations. Cognitive (varying from the minimal changes to coma) and motor (muscles stiffness, baryphonia, tremor, etc.) disorders prevail in the clinical presentation. The basic approaches in treatment of HE include: detection and elimination liver damaging factors that provoke onset or stimulate progression of HE; reduction of production and absorption of ammonia or increase of its elimination. At frequently relapsing and progressing liver failure liver transplantation is performed. Conclusion. Involvement of the central nervous system at liver failure is the major problem from the point of view of duly and correct diagnostics, treatment, and social adaptation of patients. Many aspects of HE in particular its pathogenesis are insufficiently investigated.

печеночная энцефалопатияпатогенезклиникадиагностикатерапиярифаксимин-aлактулозаL-орнитин-L-аспартатReferencesИвашкин В.Т., Маевская М.В., Федосьина Е.А. Лечение осложнений цирроза печени: Методические рекомендации для врачей. М.: Литтерра; 2011.Ивашкин В.Т., Маевская М.В., Федосьина Е.А. Лечение осложнений цирроза печени: Методические рекомендации для врачей. М.: Литтерра; 2011.Ивашкин В.Т., Надинская М.Ю., Буеверов А.О. Печеночная энцефалопатия и методы ее метаболической коррекции. Болезни органов пищеварения 2001;1:25-7.Ивашкин В.Т., Надинская М.Ю., Буеверов А.О. Печеночная энцефалопатия и методы ее метаболической коррекции. Болезни органов пищеварения 2001;1:25-7.Шульпекова Ю.О., Маевская М.В. Специальные средства парентерального питания для коррекции метаболических нарушений при печеночной энцефалопатии. Фарматека: Международный медицинский журнал 2006; 1:55-60.Шульпекова Ю.О., Маевская М.В. Специальные средства парентерального питания для коррекции метаболических нарушений при печеночной энцефалопатии. Фарматека: Международный медицинский журнал 2006; 1:55-60.Lockwood A.H. Hepatic Encephalopathy. In: Neurology and General Medicine. Fourth ed. Ed. by M.J.Aminoff. Ch. 14. Philadelphia; 2008. Р. 265-79.Lockwood A.H. Hepatic Encephalopathy. In: Neurology and General Medicine. Fourth ed. Ed. by M.J.Aminoff. Ch. 14. Philadelphia; 2008. Р. 265-79.Stewart C.A., Menon K.V.N., Kamath P.S. Hepatic encephalopathy - diagnosis and management. In: Neurological Therapeutics Principles and Practice. Second ed. Vol. 2. Ed. by J.H.Noseworthy. Ch. 119. Abingdon: Informa Healthcare; 2006. P. 1432-40.Stewart C.A., Menon K.V.N., Kamath P.S. Hepatic encephalopathy - diagnosis and management. In: Neurological Therapeutics Principles and Practice. Second ed. Vol. 2. Ed. by J.H.Noseworthy. Ch. 119. Abingdon: Informa Healthcare; 2006. P. 1432-40.Alonso J., Cordoba J., Rovira A. Brain magnetic resonance in hepatic encephalopathy. Semin Ultrasound CT MRI 2014; 35:136-52.Alonso J., Cordoba J., Rovira A. Brain magnetic resonance in hepatic encephalopathy. Semin Ultrasound CT MRI 2014; 35:136-52.Hassan E.A., Abd El-Rehim A.S., Seifeldein G.S., Shehata G. Minimal hepatic encephalopathy in patients with liver cirrhosis: Magnetic resonance spectroscopic brain findings versus neuropsychological changes. Arab J Gastroenterol 2014; 15:108-13.Hassan E.A., Abd El-Rehim A.S., Seifeldein G.S., Shehata G. Minimal hepatic encephalopathy in patients with liver cirrhosis: Magnetic resonance spectroscopic brain findings versus neuropsychological changes. Arab J Gastroenterol 2014; 15:108-13.Butterworth R.F. Pathophysiology of brain dysfunction in hyperammonemic syndromes: The many faces of glutamine. Mol Gen Metab 2014; 113:113-7.Butterworth R.F. Pathophysiology of brain dysfunction in hyperammonemic syndromes: The many faces of glutamine. Mol Gen Metab 2014; 113:113-7.Chavarria L., Alonso J., Garcıa-Martınez R. et al. Brain magnetic resonance spectroscopy in episodic hepatic encephalopathy. J Cerebr Blood Flow Metab 2013; 33:272-7.Chavarria L., Alonso J., Garcıa-Martınez R. et al. Brain magnetic resonance spectroscopy in episodic hepatic encephalopathy. J Cerebr Blood Flow Metab 2013; 33:272-7.Chavarria L., Cordoba J. Magnetic resonance of the brain in chronic and acute liver failure Metab. Brain Dis 2014; 29:937-44.Chavarria L., Cordoba J. Magnetic resonance of the brain in chronic and acute liver failure Metab. Brain Dis 2014; 29:937-44.Razek A.A.K.A., Abdalla A., Ezzat A. et al. Minimal hepatic encephalopathy in children with liver cirrhosis: diffusion-weighted MR imaging and proton MR spectroscopy of the brain. Neuroradiology 2014; 56:885-91.Razek A.A.K.A., Abdalla A., Ezzat A. et al. Minimal hepatic encephalopathy in children with liver cirrhosis: diffusion-weighted MR imaging and proton MR spectroscopy of the brain. Neuroradiology 2014; 56:885-91.Ahluwalia V., Wade J.B., Heuman D.M. et al. Enhancement of functional connectivity, working memoryand inhibitory control on multi-modal brain MR imaging with Rifaximin in Cirrhosis: Implications for the gutliver-brain axis. Metab Brain Dis 2014; 29:1017-25.Ahluwalia V., Wade J.B., Heuman D.M. et al. Enhancement of functional connectivity, working memoryand inhibitory control on multi-modal brain MR imaging with Rifaximin in Cirrhosis: Implications for the gutliver-brain axis. Metab Brain Dis 2014; 29:1017-25.Bathla G., Hegde A.N. MRI and CT appearances in metabolic encephalopathies due to systemic diseases in adults. Clin Radiol 2013; 68:545-54.Bathla G., Hegde A.N. MRI and CT appearances in metabolic encephalopathies due to systemic diseases in adults. Clin Radiol 2013; 68:545-54.Ciecko-Michalska I., Dziedzic T., Banys R. et al. Does magnetic resonance spectroscopy identify patients with minimal hepatic encephalopathy? Neurol Neurochirurg Polska 2012; 46(5):436-42.Ciecko-Michalska I., Dziedzic T., Banys R. et al. Does magnetic resonance spectroscopy identify patients with minimal hepatic encephalopathy? Neurol Neurochirurg Polska 2012; 46(5):436-42.Conn H.O., Leevy C.M., Vlahcevic Z.R. et al. Comparison of lactulose and neomycin in the treatment of chronic portalsystemic encephalopathy: a double blind controlled trial. Gastroenterology 1977; 72:573-83.Conn H.O., Leevy C.M., Vlahcevic Z.R. et al. Comparison of lactulose and neomycin in the treatment of chronic portalsystemic encephalopathy: a double blind controlled trial. Gastroenterology 1977; 72:573-83.Orlandi F., Freddara U., Candelaresi M.T. et al. Comparison between neomycin and lactulose in 173 patients with hepatic encephalopathy: a randomized clinical study. Dig Dis Sci 1981; 26:498-506.Orlandi F., Freddara U., Candelaresi M.T. et al. Comparison between neomycin and lactulose in 173 patients with hepatic encephalopathy: a randomized clinical study. Dig Dis Sci 1981; 26:498-506.Strauss E., Tramote R., Silva E.P. et al. Double-blind randomized clinical trial comparing neomycin and placebo in the treatment of exogenous hepatic encephalopathy. Hepatogastroenterology 1992; 39:542-5.Strauss E., Tramote R., Silva E.P. et al. Double-blind randomized clinical trial comparing neomycin and placebo in the treatment of exogenous hepatic encephalopathy. Hepatogastroenterology 1992; 39:542-5.Tromm A., Griga T., Greving I. et al. Orthograde whole gut irrigation with mannite versus paromomycine + lactulose as prophylaxis of hepatic encephalopathy in patients with cirrhosis and upper gastrointestinal bleeding: results of a controlled randomized trial. Hepatogastroenterology 2000; 47:473-7.Tromm A., Griga T., Greving I. et al. Orthograde whole gut irrigation with mannite versus paromomycine + lactulose as prophylaxis of hepatic encephalopathy in patients with cirrhosis and upper gastrointestinal bleeding: results of a controlled randomized trial. Hepatogastroenterology 2000; 47:473-7.Morgan M.H., Read A.E., Speller D.C. Treatment of hepatic encephalopathy with metronidazole. Gut 1982; 23:1-7.Morgan M.H., Read A.E., Speller D.C. Treatment of hepatic encephalopathy with metronidazole. Gut 1982; 23:1-7.Tarao K., Ikeda T., Hayashi K. et al. Successful use of vancomycin hydrochloride in the treatment of lactulose resistant chronic hepatic encephalopathy. Gut 1990; 31:702-6.Tarao K., Ikeda T., Hayashi K. et al. Successful use of vancomycin hydrochloride in the treatment of lactulose resistant chronic hepatic encephalopathy. Gut 1990; 31:702-6.Di Piazza S., Gabriella F.M., Valenza L.M. et al. Rifaximine versus neomycin in the treatment of portosystemic encephalopathy. Ital J Gastroenterol 1991; 23:403-7.Di Piazza S., Gabriella F.M., Valenza L.M. et al. Rifaximine versus neomycin in the treatment of portosystemic encephalopathy. Ital J Gastroenterol 1991; 23:403-7.Pedretti G., Calzetti C., Missale G., Fiaccadori F. Rifaximin versus neomycin on hyperammonemia in chronic portal systemic encephalopathy of cirrhotics: a double-blind, randomized trial. Ital J Gastroenterol 1991; 23:175-8.Pedretti G., Calzetti C., Missale G., Fiaccadori F. Rifaximin versus neomycin on hyperammonemia in chronic portal systemic encephalopathy of cirrhotics: a double-blind, randomized trial. Ital J Gastroenterol 1991; 23:175-8.Bucci L., Palmieri G.C. Double-blind, double-dummy comparison between treatment with rifaximin and lactulose in patients with medium to severe degree hepatic encephalopathy. Curr Med Res Opin 1993; 13:109-18.Bucci L., Palmieri G.C. Double-blind, double-dummy comparison between treatment with rifaximin and lactulose in patients with medium to severe degree hepatic encephalopathy. Curr Med Res Opin 1993; 13:109-18.Mas A., Rodes J., Sunyer L. et al. Comparison of rifaximin and lactitol in the treatment of acute hepatic encephalopathy: results of a randomized, double-blind, double-dummy, controlled clinical trial. J Hepatol 2003; 38:51-8.Mas A., Rodes J., Sunyer L. et al. Comparison of rifaximin and lactitol in the treatment of acute hepatic encephalopathy: results of a randomized, double-blind, double-dummy, controlled clinical trial. J Hepatol 2003; 38:51-8.Leevy C.B., Phillips J.A. Hospitalizations during the use of rifaximin versus lactulose for the treatment of hepatic encephalopathy. Dig Dis Sci 2007; 52:737-41.Leevy C.B., Phillips J.A. Hospitalizations during the use of rifaximin versus lactulose for the treatment of hepatic encephalopathy. Dig Dis Sci 2007; 52:737-41.Neff G.W., Kemmer N., Zacharias V.C. et al. Analysis of hospitalizations comparing rifaximin versus lactulose in the management of hepatic encephalopathy. Transplant Proc 2006; 38:3552-5.Neff G.W., Kemmer N., Zacharias V.C. et al. Analysis of hospitalizations comparing rifaximin versus lactulose in the management of hepatic encephalopathy. Transplant Proc 2006; 38:3552-5.Spiegel B., Huang E., Esrailian E. Is rifaximin costeffective in the management of hepatic encephalopathy? Gastroenterology 2006; 130:A-806. Abstract M1031.Spiegel B., Huang E., Esrailian E. Is rifaximin costeffective in the management of hepatic encephalopathy? Gastroenterology 2006; 130:A-806. Abstract M1031.

The authors declare that there are no conflicts of interest present.