perinatologyРоссийский вестник перинатологии и педиатрииRossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)1027-40652500-2228Ltd. “The National Academy of Pediatric Science and Innovation”10.21508/1027-4065-2023-68-3-61-67perinatology-1827Research ArticleОРИГИНАЛЬНЫЕ СТАТЬИORIGINAL ARTICLESСиндром портальной гипертензии у детей с аутосомно-рецессивной поликистозной болезнью почек, кистозом и фиброзом печениPortal hypertension syndrome in children with autosomal recessive polycystic kidney disease with liver cysts and hepatic fibrosishttps://orcid.org/0000-0002-8753-1415АндрееваЭ. Ф.AndreevaE. F.

Андреева Эльвира Фаатовна - к.м.н., доц. кафедры факультетской педиатрии

194100 Санкт-Петербург, ул. Литовская, д. 2

Saint Petersburg

A-Elvira@yandex.ruhttps://orcid.org/0000-0002-3998-6407ДюгИ. В.DyugI. V.

Дюг Игорь Витальевич - врач-хирург микрохирургического отделения клиники

194100 Санкт-Петербург, ул. Литовская, д. 2

Saint Petersburg

https://orcid.org/0000-0001-7890-733XГорячеваЛ. Г.GoryachevaL. G.

Горячева Лариса Георгиевна - д.м.н., проф. кафедры инфекционныхзаболеваний у детей факультета послевузовского дополнительного профессионального образования; рук. отдела вирусных гепатитов и заболеваний печени

194100 Санкт-Петербург, ул. Литовская, д. 2

Saint Petersburg

https://orcid.org/0000-0002-9415-4785СавенковаН. Д.SavenkovaN. D.

Савенкова Надежда Дмитриевна -д.м.н., проф., зав. кафедрой факультетской педиатрии

194100 Санкт-Петербург, ул. Литовская, д. 2

Saint Petersburg

ФГБОУ ВО «Санкт-Петербургский государственный педиатрический медицинский университет» Минздрава РоссииРоссияSaint Petersburg State Pediatric Medical UniversityRussian FederationФГБОУ ВО «Санкт-Петербургский государственный педиатрический медицинский университет» Минздрава России; ФГБУ «Детский научно-клинический центр инфекционных болезней Федерального медико-биологического агентства»РоссияSaint Petersburg State Pediatric Medical University; Pediatric Research and Clinical Center for Infectious DiseasesRussian Federation2023150720236836167Copyright © Ltd. “The National Academy of Pediatric Science and Innovation”, 20232023Ltd. “The National Academy of Pediatric Science and Innovation”Ltd. “The National Academy of Pediatric Science and Innovation”https://www.ped-perinatology.ru/jour/about/submissions#copyrightNoticehttps://www.ped-perinatology.ru/jour/article/view/1827

Фиброз и кистоз печени, синдром портальной гипертензии — экстраренальные проявления, определяющие тяжесть течения и прогноз аутосомно-рецессивной поликистозной болезни почек у детей.Цель исследования. В катамнезе детей с аутосомно-рецессивной поликистозной болезнью почек оценить особенности манифестации и течения кистоза и фиброза печени, развития синдрома портальной гипертензии.Пациенты и методы. Обследованы 27 детей с аутосомно-рецессивной поликистозной болезнью почек, из них 2 с летальным исходом в неонатальном периоде исключены из последующих этапов исследования; 25 детей в возрасте 1–17 лет разделены на 2 группы по наличию синдрома портальной гипертензии. Из 25 детей с аутосомно-рецессивной поликистозной болезнью почек у 10 (40%) на момент катамнеза отсутствовали признаки синдрома портальной гипертензии (1-я группа), у 15 (60%) детей выявлен синдром портальной гипертензии (2-я группа). В работе использованы катамнестический, клинический, генеалогический, лабораторный и инструментальный (ультразвуковое исследование, магнитно-резонансная/компьютерная томография почек и органов брюшной полости, эластография печени, по показаниям – эзофагогастродуоденоскопия) методы исследования. У 3 детей диагноз подтвержден при аутопсии.Результаты. При ультразвуковом исследовании пренатально не выявлено характерных для аутосомно-рецессивной поликистозной болезни почек, изменений печени и желчных протоков. Из 27 обследованных детей у 10 (37%) диагностирован в катамнезе фиброз печени, у 22 (81%) — расширение внутрипеченочных желчных протоков, из них у 15 (68%) — поликистоз печени, у 3 (14%) — болезнь Кароли. При аутосомно-рецессивной поликистозной болезни почек у всех детей с синдромом портальной гипертензии установлено варикозное расширение вен пищевода и желудка по результатам эзофагодуоденоскопии, в 53% случаев с показаниями к лигированию флебэктазии, в 47% диагностирована тромбоцитопения, в 67% – анемия, в 100% спленомегалия, в 13% – пищеводно-желудочное кровотечение.Заключение. В 2 сравниваемых группах у детей выявлены различия в начальных проявлениях аутосомно-рецессивной поликистозной болезни почек, не установлено различий по частоте развития фиброза и кистоза печени в катамнезе.

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.

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