1 ФГБУ Детский научно-клинический центр инфекционных болезней Федерального медико-биологического агентства, Санкт-Петербург, Россия 2 Управление Федеральной службы Роспотребнадзора по городу Санкт-Петербургу, Санкт-Петербург, РоссияПредставлен анализ серологической эффективности и длительности сохранения специфического иммунитета у детей, привитых различными вакцинами против гепатита В в рамках национального календаря прививок на территории Санкт-Петербурга. Выявлено, что высокие титры антител (более 100мМЕ/мл) чаще наблюдались у детей в первые 3 го-да после вакцинации (68,2%). С течением времени уровни антител снижались, и через 5 лет защитные титры сохрани-лись у 84,5% обследуемых, число пациентов с титром антител менее 10 Мме/мл выросло с 8,5 до 15,5%.

Hepatic fibrosis, liver cysts, and portal hypertension are extrarenal manifestations that determine the prognosis of autosomal recessive polycystic kidney disease in children.Purpose. To assess the features of the manifestation and course of liver cystic disease and fibrosis, the development of portal hypertension in the follow-up medical history of children with autosomal recessive polycystic kidney disease.Material and methods. We studied 27 children with autosomal recessive polycystic kidney disease, with two children with a fatal outcome in the neonatal period excluded. 25 children 1–17 years old with autosomal recessive polycystic kidney disease were divided into 2 groups depending on the presence of portal hypertension syndrome. In the long-term follow-up 10 (40%) of 25 children with autosomal recessive polycystic kidney disease had no signs of portal hypertension (group 1), 15 (60%) children had portal hypertension syndrome (group 2). The long-term follow-up, clinical, genealogical, laboratory and instrumental (US, MRI/CT of kidney and abdominal cavity, liver elastography) research methods were used in the study. The diagnosis was confirmed by autopsy for 3 children.Results. There were no cases of changes in the liver and bile ducts characteristic of autosomal recessive polycystic kidney disease by prenatal ultrasound examination. From 27 children with autosomal recessive polycystic kidney disease, in 10 (37%) were diagnosed the liver fibrosis in the long-term follow-up, 22 (81%) had cystic enlargement of intrahepatic veins, of which 15 (68%) had polycystic liver disease, 3 (14%) had Caroli disease. All children with autosomal recessive polycystic kidney disease and portal hypertension syndrome had varicose veins of the esophagus and stomach according to esophagoduodenoscopy, 53% with indications for ligation of phlebectasia, 47% were diagnosed with thrombocytopenia, 67% with anemia, 100% with splenomegaly, 13% with esophageal-gastric bleeding.Conclusion. The differences in the initial manifestations of autosomal recessive polycystic kidney disease were revealed, while no differences in the incidence of hepatic fibrosis and liver cysts were found in 2 compared groups of children.

Aim. To determine the number of children with chronic hepatitis C in the Russian Federation, including, who have received antiviral treatment, taking into account their age, the genotype of the virus, as well as the therapy regimens used.Materials and methods. The analysis of specially developed statistical reporting forms was carried out, the filling of which was carried out in September 2020 by specialists from 268 medical organizations from 37 constituent entities of the Russian Federation, which are part of 8 federal districts.Results. In September 2020, 2,160 children with chronic hepatitis C virus infection aged 0 to 17 years were under observation in 268 medical organizations, including 50.7% females and 49.3% of males. The number of children in the age group from 12 to 17 years was 42.9%, from 6 to 11 years – 34.5%, from 3 to 5 years – 16.2% and from 0 to 2 years – 6.4%. The genotype of the virus was determined in 1388 (64.3%) children. The proportion of children with genotype 1 was 58.6%, with genotype 3 – 37.2%, with genotype 2 – 4%. Only 141 (8.8%) children with chronic hepatitis C virus infection have been received antiviral therapy. 1465 (91.2%) children were not treated, but 153 (9.5%) of them received therapy earlier, without achieving a sustained virological response. Direct-acting antiviral agents treatment was carried out to 120 children (85.1%), of whom glecaprevir + pibrentasvir was received by 85 children (70.8%) in 20 regions, sofosbuvir + ledipasvir – 14 children (11.7%) in 6 regions, sofosbuvir – 14 children (11.7%) in 6 regions, daklatasvir – 7 children (5.8%) in 4 regions. Children are removed from dispensary observation after achieving a stable virological response in accordance with the current regulatory documents in 26 regions of the Russian Federation (70.3%).Conclusion. In 2020, less than 10% of children under management received antiviral therapy for chronic hepatitis C virus infection in the Russian Federation. It is necessary to approve the state program for the treatment of viral hepatitis, one of the directions of which should be the provision of all children with chronic hepatitis C virus infection with modern highly effective antiviral drugs. It is also necessary to conduct clinical trials to assess the safety and efficacy of direct-acting antiviral agents for children with chronic hepatitis C virus infection in order to ensure the possibility of their earlier prescription.

Liver transplantation today is the only radical method of treatment decompensating fulminant and chronic liver failure. The operation technic and patient care improvement made it possible to achieve a high survival rate. Transplantation has become an available and safe method for children, including patients weighing less than 10 kg. However, at the moment there are a number of unsolved problems. Postoperative complications can significantly affect the results of transplantation. This article provides a Russian and foreign literature overview, reflecting the achievements in the field of liver, identifying current problems and solutions.

Введение. Хронический гепатит С (ХГС) занимает лидирующую позицию среди всех хронических гепатитов. Около 71 миллиона человек в мире инфицированы вирусом гепатита С (ВГС), среди них, по разным оценкам, от 2,1 до 5 млн составляют дети до 15 лет. В большинстве случаев заболевание в детском возрасте протекает бессимптомно, однако с возрастом повышается риск развития фиброза и связанных с ним осложнений, что требует динамического контроля и своевременного назначения противовирусной терапии. Цель исследования. Определить эпидемиологические особенности и характер течения вирусного гепатита С у детей, инфицированных в раннем возрасте; проанализировать эффективность новых схем терапии препаратами прямого противовирусного действия в сравнении с терапией препаратами интерферона. Материалы и методы. В группу наблюдательного исследования вошли 559 детей с установленным диагнозом ХГС. Диагноз был подтвержден серологически (методом ИФА) и молекулярно-генетически (методом полимеразной цепной реакции - ПЦР). Степень фиброза оценивалась по результатам эластографии печени (Fibroscan). Результаты. Преобладающее большинство детей были инфицированы ВГС в результате перинатального контакта. Спонтанный клиренс отмечался не более чем в 20% случаев. По полученным данным, девочки заражались чаще мальчиков, однако с большей частотой элиминировали вирус. ХГС в детском возрасте характеризовался гладким, малосимптомным течением, однако 12% пациентов имели начальную стадию фиброза. УВО при использовании интерфероновых (ИФН) схем был достигнут у 40-53% пациентов с 1-м генотипом, у 92-100% пациентов со 2-м и 3-м генотипами. Эффективность терапии α-ИФН с рибавирином (РВ) составила 72%, пег-ИФН с РВ - 81,8%, комбинацией пег-ИФН + РВ в сочетании с каскадной плазмофильтрацией - 89,2%. Применение препаратов интерферона сопровождалось развитием нежелательных явлений, наиболее тяжело переносили введение препарата подростки старше 12 лет. Среди побочных реакций чаще всего наблюдался гриппоподобный, астеновегетативный синдромы (до 97%), несколько реже отмечались диспептические расстройства, снижение массы тела, алопеция, нервно-психические расстройства. В единичных случаях был спровоцирован дебют аутоиммунных заболеваний. На базе нашего центра подтверждено влияние полиморфизма генов rs8099917 и rs12979860 IL28B на вероятность спонтанной элиминации вируса гепатита С, а также на эффективность ПВТ. С 2019 г. начата терапия препаратами прямого противовирусного действия (ПППД), которая показала 100% эффективность при развитии минимальных нежелательных явлений. Выводы. Заболеваемость ХГС в настоящее время остается на высоком уровне. Несмотря на малосимптомное течение заболевания у детей, с возрастом значительно повышается риск развития прогрессирующего фиброза печени, жизнеугрожающих осложнений. Применение препаратов прямого противовирусного действия у детей позволяет не только своевременно начать терапию ХГС, но также избежать серьезных нежелательных явлений, связанных с лечением препаратами интерферона. Introduction. Chronic hepatitis C (CHC) occupies a leading position among all chronic hepatitis. There are about 71 million people in the world infected with the hepatitis C virus (HCV). Among them there are 2.1-5 million children under 15 years of age according to various estimates. In most cases, the disease in childhood is asymptomatic, however, with age, the risk of fibrosis and associated complications increases, that`s why requires dynamic control and timely administration of antiviral therapy. Purpose of the study. To determine the epidemiological features and nature of the course of viral hepatitis C in children infected at an early age; to analyze the effectiveness of new regimens of therapy with direct antiviral drugs in comparison with interferon therapy. Materials and methods. There are 559 children with an established diagnosis of CHC included in the observational study group. The diagnosis was confirmed serologically (by ELISA) and molecular- genetically (by polymerase chain reaction - PCR). The degree of fibrosis was assessed by the results of liver elastography (Fibroscan). Results. The vast majority of children were infected with HCV as a result of perinatal contact. Spontaneous clearance was noted in no more than 20% of cases. According to the data obtained, girls became infected more often than boys, but eliminated the virus with a greater frequency. CHC in childhood was characterized by a smooth, low-symptom course, but 12% of patients had an initial stage of fibrosis. SVR after using interferon (IFN) regimens was achieved in 40-53% of patients with genotype 1, 92-100% in patients with genotypes 2 and 3. The effectiveness of therapy for a-IFN with ribavirin (RV) was 72%, peg-IFN with RV - 81.8%, the combination of peg-IFN + RV in combination with cascade plasma filtration - 89.2%. The use of interferon preparations was accompanied by the development of undesirable phenomena; adolescents over 12 years of age hardly tolerated the administration of the drug. More often observed adverse events were flu-like, asthenovegetative syndromes (up to 97%), dyspeptic disorders, weight loss, alopecia, and neuropsychiatric disorders were noted somewhat less frequently. In isolated cases, the debut of autoimmune diseases was provoked. On the basis of our center, the influence of the polymorphism of the rs8099917 and rs12979860 IL28B genes on the probability of spontaneous elimination of the hepatitis C virus, as well as on the effectiveness of the AVT, has been confirmed. Since 2019 therapy when direct antiviral therapy (DAA) was started, it showed 100% efficiency with minimal adverse events. Conclusions. The incidence of CHC currently remains at a high level. Despite the low-symptomatic course of the disease in children, the risk of developing progressive liver fibrosis and life-threatening complications increases significantly with age. The use of drugs with direct antiviral action in children allows not only to start therapy for CHC in a timely manner, but also to avoid serious adverse events associated with treatment with interferon drugs.