AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE WITH VERY-EARLY ONSET

Q4 Medicine

Pediatriya - Zhurnal im G.N. Speranskogo Pub Date : 2023-08-10 DOI:10.24110/0031-403x-2023-102-4-177-186

M. Shumikhina, O. Chugunova, A. Gurevich

引用次数: 0

Abstract

Both autosomal dominant polycystic kidney disease and autosomal recessive polycystic kidney disease may occur at any age starting infancy and up to adulthood. Manifestations of autosomal dominant polycystic kidney disease usually occur in adult patients with a risk of development of end-stage renal failure after the fifth decade of life. However, an increase in the number of pediatric cases of a very-early onset of the disease, in utero or during the first 18 months of life, had been registered recently, that were characterized by severe clinical manifestations which in tis turn had mimic the autosomal recessive polycystic kidney disease. The three infant cases of autosomal dominant polycystic kidney disease are observed in the Article with perinatal period features, clinical manifestations of the disease, tests’ results and its interpretation given. Children with very-early onset of autosomal dominant polycystic kidney disease are in the high-risk group for nephromegaly, severe hypertension and the early end-stage renal failure.

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常染色体显性多囊肾病，发病很早

常染色体显性多囊肾病和常染色体隐性多囊肾病都可能发生在从婴儿期到成年期的任何年龄。常染色体显性多囊肾病的表现通常发生在成年患者，在生命的第五个十年后有发展为终末期肾衰竭的风险。然而，最近登记的儿童病例中，在子宫内或出生后18个月内发病的病例数量有所增加，这些病例的特点是严重的临床表现，而这又类似于常染色体隐性多囊肾病。本文观察了3例婴儿常染色体显性多囊肾病的围生期特征、临床表现、检查结果及解释。早发常染色体显性多囊肾病患儿是肾病、重度高血压和早期终末期肾功能衰竭的高危人群。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Pediatriya - Zhurnal im G.N. Speranskogo Medicine-Pediatrics, Perinatology and Child Health

CiteScore

0.60

自引率

0.00%

发文量

150

期刊介绍： Journal “Pediatria” named after G.N. Speransky (the official short names of the Journal are “Journal «Pediatria»,” “Pediatria,” and “«Pediatria,» the Journal”) is the oldest Soviet-and-Russian (in the Russian Federation, the CIS and former Soviet Union) scientific and practical medical periodical assigned for pediatricians that is published continuously since May, 1922, and distributed worldwide. Our mission statement specifies that we aim to the ‘raising the level of skills and education of pediatricians, organizers of children’s health protection services, medicine scientists, lecturers and students of medical institutes for higher education, universities and colleges worldwide with an emphasis on Russian-speaking audience and specific, topical problems of children’s healthcare in Russia, the CIS, Baltic States and former Soviet Union Countries and their determination with the use of the World’s best practices in pediatrics.’ As part of this objective, the Editorial of the Journal «Pediatria» named after G.N. Speransky itself adopts a neutral position on issues treated within the Journal. The Journal serves to further academic discussions of topics, irrespective of their nature - whether religious, racial-, gender-based, environmental, ethical, political or other potentially or topically contentious subjects. The Journal is registered with the ISSN, - the international identifier for serials and other continuing resources, in the electronic and print world: ISSN 0031-403X (Print), and ISSN 1990-2182 (Online). The Journal was founded by the Academician, Dr. Georgiy Nestorovich SPERANSKY, in May, 1922. Now (since 1973) the Journal bears his honorary name.