Renal oligohydramnios and Potter sequence with cystic kidney disease

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...

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Main Authors: E. F. Andreeva, N. D. Savenkova
Format: Article
Language:Russian
Published: Ltd. “The National Academy of Pediatric Science and Innovation” 2021-03-01
Series:Rossijskij Vestnik Perinatologii i Pediatrii
Subjects:
Online Access:https://www.ped-perinatology.ru/jour/article/view/1337
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author E. F. Andreeva
N. D. Savenkova
author_facet E. F. Andreeva
N. D. Savenkova
author_sort E. F. Andreeva
collection DOAJ
description 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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spelling doaj.art-df404630359d427aa65cd1666edb94fc2023-03-13T09:12:50ZrusLtd. “The National Academy of Pediatric Science and Innovation”Rossijskij Vestnik Perinatologii i Pediatrii1027-40652500-22282021-03-01661475110.21508/1027-4065-2021-66-1-47-511000Renal oligohydramnios and Potter sequence with cystic kidney diseaseE. F. Andreeva0N. D. Savenkova1ФГБОУ ВО «Санкт-Петербургский государственный педиатрический медицинский университет» Минздрава РоссииФГБОУ ВО «Санкт-Петербургский государственный педиатрический медицинский университет» Минздрава РоссииFor 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).https://www.ped-perinatology.ru/jour/article/view/1337новорожденныепоттер-фенотиппоттер-последовательностьренальное маловодиекистозы почек
spellingShingle E. F. Andreeva
N. D. Savenkova
Renal oligohydramnios and Potter sequence with cystic kidney disease
Rossijskij Vestnik Perinatologii i Pediatrii
новорожденные
поттер-фенотип
поттер-последовательность
ренальное маловодие
кистозы почек
title Renal oligohydramnios and Potter sequence with cystic kidney disease
title_full Renal oligohydramnios and Potter sequence with cystic kidney disease
title_fullStr Renal oligohydramnios and Potter sequence with cystic kidney disease
title_full_unstemmed Renal oligohydramnios and Potter sequence with cystic kidney disease
title_short Renal oligohydramnios and Potter sequence with cystic kidney disease
title_sort renal oligohydramnios and potter sequence with cystic kidney disease
topic новорожденные
поттер-фенотип
поттер-последовательность
ренальное маловодие
кистозы почек
url https://www.ped-perinatology.ru/jour/article/view/1337
work_keys_str_mv AT efandreeva renaloligohydramniosandpottersequencewithcystickidneydisease
AT ndsavenkova renaloligohydramniosandpottersequencewithcystickidneydisease