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”10.21508/1027-4065-2021-66-1-47-51perinatology-1337Research ArticleОБЗОРЫ ЛИТЕРАТУРЫLITERATURE REVIEWSРенальное маловодие и Поттер-последовательность при кистозных заболеваниях почекRenal oligohydramnios and Potter sequence with cystic kidney diseasehttps://orcid.org/0000-0002-8753-1415АндрееваЭ. Ф.AndreevaE. F.

Андреева Эльвира Фаатовна – кандидат медицинских наук, ассистент кафедры факультетской педиатрии 

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

Saint Petersburg

A-Elvira@yandex.ruhttps://orcid.org/0000-0002-9415-4785СавенковаН. Д.SavenkovaN. D.

Савенкова Надежда Дмитриевна – доктор медицинских наук, профессор, 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 Federation2021290320216614751Copyright © Ltd. “The National Academy of Pediatric Science and Innovation”, 20212021Ltd. “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/1337

Впервые в 1946 г. E.L. Potter (1901–1993) описала характерный внешний вид у мертворожденных и умерших новорожденных с двусторонней агенезией почек. Дальнейшие наблюдения позволили выделить Поттер-синдром (Potter syndrome, Q60.6) – совокупность характерных внешних признаков, которые формируются вследствие крайней степени маловодия и внутриутробного сдавления плода маткой. Классический Поттер-синдром диагностируют при отсутствии функции обеих почек у плода (например, при двусторонней агенезии), что приводит к летальному исходу. Широкое клиническое применение получил термин «Поттер-последовательность» (Potter sequence), или «последовательность маловодия» (oligohydramnios sequence), причины которой разнообразны. Для обозначения маловодия, развившегося в результате снижения или отсутствия функции почек плода, используют термин «почечное (ренальное) маловодие» (renal oligohydramnios, ROH). В представленном обзоре показано, что при кистозах почек у плода с формированием кист в паренхиме обеих почек (аутосомно-рецессивный поликистоз почек, аутосомно-доминантный поликистоз почек, гломерулокистозная болезнь почек, ассоциированная с мутациями гена HNF1ß/TCF2, колобомо-ренальный синдром, или renal-coloboma синдром, кистозная гипоплазия почек, кистозная дисплазия почек при мутациях гена CEP55) существует риск развития ренального маловодия и рождения ребенка с  Поттер-последовательностью.

For the first time in 1946 E.L. Potter (1901–1993) described the characteristic appearance of stillborns and deceased newborns with bilateral renal agenesis. Due to the further observations Potter distinguished the syndrome (Q60.6) – a set of characteristic external signs that are formed due to the extreme degree of oligohydramnios and intrauterine compression of the fetus. Classical Potter syndrome is diagnosed by the disfunction of both kidneys in the fetus (for example, bilateral agenesis), which leads to death. The term «Potter sequence» or oligohydramnios sequence with diverse causes has received the wide clinical use. The term «renal oligohydramnios» (ROH) is used to describe oligohydramnios resulting from a decrease or absence of fetal kidney function. The authors state that renal oligohydramnios and Potter sequence often develop in the fetus with cystic kidney disease with the formation of cysts in the parenchyma of both kidneys (autosomal recessive polycystic kidney disease, autosomal dominant polycystic kidney disease, glomerulocystic kidney disease associated with HNF1ß/TCF2 gene mutations, renal-coloboma syndrome, cystic renal hypoplasia, cystic renal dysplasia with mutations of the CEP55 gene).

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