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We hypothesized that a history of macroscopic hematuria will be associated with hypertension also in children with ADPKD. We retrospectively analyzed 289 children (median age 10.2\u00a0years) with ADPKD who were followed in four European tertiary nephrology centers. The presence of\u2009\u2265\u20091 episode of macroscopic hematuria (MaHu) defines a group of MaHu+\u2009patients; the absence defines a group of MaHu\u2212\u2009patients. Hypertension was defined as the use of antihypertensive drugs upon the last investigation. Macroscopic hematuria occurred in 7.2% of children (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200921). It was the second most frequent presenting symptom of ADPKD (11 out of 51 children). The etiology of macroscopic hematuria included ruptured cyst, urinary tract infection or urolithiasis. The prevalence of hypertension at the last follow-up in MaHu+\u2009children was significantly higher than in MaHu\u2212\u2009children (14.3% vs. 3.1%,\n                    <jats:italic>p<\/jats:italic>\n                    \u2009=\u20090.017). The proportion of patients with elevated blood pressure\u2009\u2265\u200975th percentile was significantly higher in patients with hematuria as compared with patients with no hematuria (95% vs. 64%,\n                    <jats:italic>p<\/jats:italic>\n                    \u2009=\u20090.002). Children from the MaHu+\u2009group had a significantly higher number of cysts when compared to the MaHu\u2212\u2009group (20 vs. 10 cysts,\n                    <jats:italic>p<\/jats:italic>\n                    \u2009=\u20090.038). There were no significant differences between MaHu+\u2009vs. MaHu\u2212\u2009groups in other variables.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:italic>Conclusion<\/jats:italic>\n                    :\u00a0In children with ADPKD, a history of macroscopic hematuria is associated with an increased risk of developing hypertension and\/or elevated blood pressure.\n                    <jats:table-wrap>\n                      <jats:table>\n                        <jats:tbody>\n                          <jats:tr>\n                            <jats:td colspan=\"2\">\n                              <jats:bold>What is Known:<\/jats:bold>\n                              \u2022\n                              <jats:italic>Adults with autosomal dominant polycystic kidney disease have often episodes of macroscopic hematuria that predict development of hypertension.<\/jats:italic>\n                            <\/jats:td>\n                          <\/jats:tr>\n                          <jats:tr>\n                            <jats:td colspan=\"2\">\n                              <jats:bold>What is New:<\/jats:bold>\n                              \u2022\n                              <jats:italic>In children with autosomal dominant polycystic kidney disease, macroscopic hematuria is rare but is also associated with an increased risk of developing hypertension.<\/jats:italic>\n                            <\/jats:td>\n                          <\/jats:tr>\n                        <\/jats:tbody>\n                      <\/jats:table>\n                    <\/jats:table-wrap>\n                  <\/jats:p>","DOI":"10.1007\/s00431-026-07022-1","type":"journal-article","created":{"date-parts":[[2026,5,7]],"date-time":"2026-05-07T17:12:22Z","timestamp":1778173942000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Macroscopic hematuria in children with autosomal dominant polycystic kidney disease\u00a0\u2014\u00a0report from four European tertiary centers"],"prefix":"10.1007","volume":"185","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":"Kv\u011bta","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":"Ulrike","family":"John-Kroegel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Friederike","family":"Weigel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Terezie","family":"\u0160ul\u00e1kov\u00e1","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ludmila","family":"Podrack\u00e1","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janusz","family":"Feber","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,5,7]]},"reference":[{"key":"7022_CR1","doi-asserted-by":"publisher","first-page":"1356","DOI":"10.1093\/ndt\/gfw240","volume":"32","author":"CJ Willey","year":"2017","unstructured":"Willey CJ, Blais JD, Hall AK, Krasa HB, Makin AJ, Czerwiec FS (2017) Prevalence of autosomal dominant polycystic kidney disease in the European Union. 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Ethical approval was waived by the local Ethics Committee of University Hospital Motol in view of the retrospective nature of the study.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval"}},{"value":"Not applicable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent to participate"}},{"value":"Written informed consent could not be obtained from all individual participants included in the study because of the retrospective design of this trial.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Informed consent"}},{"value":"TS serves as a consultant to Otsuka Pharmaceutical and is an Editorial Board member of Pediatric Nephrology journal.  KB, FF, UJK, FW, TS, LP and JF have declared no conflicts of interest.","order":5,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"362"}}