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This genetic disease is mainly caused by variants in the <jats:italic>PKHD1<\/jats:italic> gene and is characterized by fibrocystic hepatorenal phenotypes with major clinical variability. ARPKD frequently presents perinatally, and the management of perinatal and early disease symptoms may be challenging. This review discusses aspects of early manifestations in ARPKD and its clincial\u00a0management with a special focus on kidney disease.<\/jats:p>","DOI":"10.1007\/s00467-021-04970-8","type":"journal-article","created":{"date-parts":[[2021,2,19]],"date-time":"2021-02-19T07:08:45Z","timestamp":1613718525000},"page":"3561-3570","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":25,"title":["Early clinical management of autosomal recessive polycystic kidney disease"],"prefix":"10.1007","volume":"36","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0494-9080","authenticated-orcid":false,"given":"Max Christoph","family":"Liebau","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2021,2,17]]},"reference":[{"key":"4970_CR1","doi-asserted-by":"publisher","first-page":"e833","DOI":"10.1542\/peds.2013-3646","volume":"134","author":"EA Hartung","year":"2014","unstructured":"Hartung EA, Guay-Woodford LM (2014) Autosomal recessive polycystic kidney disease: a hepatorenal fibrocystic disorder with pleiotropic effects. 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