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The kidney concentrating capacity was further impaired within the animal model of autosomal recessive polycystic kidney disease (ARPKD). To date, only one small study has investigated it in children having ARPKD. Therefore, we aimed to study the kidney concentrating ability in a larger cohort of children with ARPKD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Eighteen children (median age 8.5\u00a0years, range 1.3\u201316.8) were retrospectively investigated. A standardized kidney concentrating capacity test was performed after the application of a nasal drop of desmopressin (urine osmolality\u2009&gt;\u2009900 mOsmol\/kg). The glomerular filtration rate was estimated using the Schwartz formula (eGFR) and blood pressure (BP) was measured as office BP.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Kidney concentrating capacity was decreased (urine osmolality\u2009&lt;\u2009900 mOsmol\/kg) in 100% of children with ARPKD. The median urine osmolality after desmopressin application was 389 (range 235\u2013601) mOsmol\/kg. Sixteen patients (89%) were defined as hypertensive based on their actual BP level or their use of antihypertensive drugs. The maximum amounts of urinary concentration correlated significantly with eGFR (<jats:italic>r<\/jats:italic>\u2009=\u20090.72,<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.0001) and hypertensive scores (<jats:italic>r<\/jats:italic>\u2009=\u20090.50,<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.05), but not with kidney size. Twelve patients (67%) were defined as having CKD stages 2\u20134. The median concentrating capacity was significantly lower in children within this group, when compared to children with CKD stage 1 possessing a normal eGFR (544 mOsmol\/kg, range 413\u2013600 mOsmol\/kg vs. 327 mOsmol\/kg, range 235\u2013417 mOsmol\/l,<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Impaired kidney concentrating capacity is present in most children with ARPKD and is associated with decreased eGFR and hypertension.<\/jats:p><\/jats:sec><jats:sec><jats:title>Graphical abstract<\/jats:title><\/jats:sec>","DOI":"10.1007\/s00467-022-05834-5","type":"journal-article","created":{"date-parts":[[2022,12,20]],"date-time":"2022-12-20T15:03:46Z","timestamp":1671548626000},"page":"2093-2100","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Kidney concentrating capacity in children with autosomal recessive polycystic kidney disease is linked to glomerular filtration and hypertension"],"prefix":"10.1007","volume":"38","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1371-3096","authenticated-orcid":false,"given":"Tom\u00e1\u0161","family":"Seeman","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kveta","family":"Bl\u00e1hov\u00e1","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Filip","family":"Fencl","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard","family":"Klaus","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B\u00e4rbel","family":"Lange-Sperandio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gabriela","family":"Hr\u010dkov\u00e1","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u0139udmila","family":"Podrack\u00e1","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2022,12,20]]},"reference":[{"key":"5834_CR1","doi-asserted-by":"publisher","first-page":"3841","DOI":"10.1007\/s00467-021-05192-8","volume":"36","author":"C Gimpel","year":"2020","unstructured":"Gimpel C, Liebau MC, Schaefer F (2020) Systematic review on outcomes used in clinical research on autosomal recessive polycystic kidney disease-are patient-centered outcomes our blind spot? 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