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Alport syndrome (AS) is a leading hereditary cause of KF in children. Angiotensin-converting enzyme inhibitors (ACEi) have demonstrated efficacy in delaying KF in young people living with AS, but non-adherence can compromise their therapeutic benefits. To investigate the adherence to ACEi in children and adolescents with AS, a liquid chromatography-mass spectrometry (LCMS)-based method was developed for objective verification of recent medication intake at two different time points in this cohort study.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Urine samples from 58 children enrolled in the EARLY PRO-TECT Alport trial were analyzed. An LCMS-based method was established and validated to simultaneously screen and quantify both ramipril and ramiprilat in urine samples. Participants were not informed in advance of the medication intake measurements.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      A total of 106 urine samples from 58 patients with early stages of chronic kidney disease (mean estimated glomerular filtration rate, 130\u2009\u00b1\u200932\u00a0mL\/min\/1.73\u00a0m\n                      <jats:sup>2<\/jats:sup>\n                      ) were analyzed at two different time points. All 13 negative control samples (100%; 95% confidence intervals [CI] 75.7% to 100%) were identified correctly. Adherence to ACEi at the time of sampling was consistently high, with 96% (47\/49; 95% CI 86% to 99.5%) and 95% (42\/44; 95% CI 84.5% to 99.4%) of children showing confirmed drug intake at initial and second adherence measurements.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This study demonstrated that children with chronic kidney disease, when treated with ACEi within a clinical trial, show high adherence to the prescribed medication.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Graphical abstract<\/jats:title>\n                  <\/jats:sec>","DOI":"10.1007\/s00467-025-07053-0","type":"journal-article","created":{"date-parts":[[2025,11,22]],"date-time":"2025-11-22T03:28:30Z","timestamp":1763782110000},"page":"1035-1044","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["High adherence to angiotensin-converting enzyme inhibitor in children and adolescents with Alport syndrome: objective verification using liquid chromatography-mass spectrometry"],"prefix":"10.1007","volume":"41","author":[{"ORCID":"https:\/\/orcid.org\/0009-0006-2021-9255","authenticated-orcid":false,"given":"Jan","family":"Boeckhaus","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Burkhard","family":"T\u00f6nshoff","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lutz T.","family":"Weber","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dieter","family":"Haffner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lars","family":"Pape","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kay","family":"Latta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henry","family":"Fehrenbach","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Baerbel","family":"Lange-Sperandio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthias","family":"Kettwig","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabine","family":"K\u00f6nig","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ulrike","family":"John-Kroegel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jutta","family":"Gellermann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthias","family":"Galiano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angelika","family":"Hafke","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frank","family":"Streit","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Oliver","family":"Gross","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,11,22]]},"reference":[{"key":"7053_CR1","doi-asserted-by":"publisher","first-page":"846","DOI":"10.1007\/s00467-006-0081-y","volume":"21","author":"SL Goldstein","year":"2006","unstructured":"Goldstein SL, Graham N, Burwinkle T, Warady B, Farrah R, Varni JW (2006) Health-related quality of life in pediatric patients with ESRD. 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