{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,21]],"date-time":"2026-07-21T09:04:19Z","timestamp":1784624659708,"version":"3.55.0"},"reference-count":22,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2026,4,16]],"date-time":"2026-04-16T00:00:00Z","timestamp":1776297600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,4,16]],"date-time":"2026-04-16T00:00:00Z","timestamp":1776297600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2026,5]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Phenylketonuria (PKU) is a rare metabolic disorder causing elevated phenylalanine (PHE) levels requiring lifelong dietary or pharmacological management and regular monitoring. Current PHE monitoring methods, such as tandem mass spectrometry (FIA\u2010MS\/MS), are laboratory and sample transport\u2010dependent, leading to delays in obtaining results. This highlights the need for accessible point\u2010of\u2010care (PoC) solutions. Building on a previously developed bioluminescence\u2010based PHE quantification method, we created a PoC PHE sensor integrated with a smartphone app (\n                    <jats:italic>phenyx<\/jats:italic>\n                    app) for real\u2010time monitoring and data sharing. In a clinical study with PKU patients, we evaluated the reliability of the sensor and the app by comparing at\u2010home PHE measurements with dried blood samples (DBS) analyzed using the gold standard FIA\u2010MS\/MS. We also assessed time differences between sampling and results, sensor practicality, and user experience. Forty\u2010seven PKU patients (median age 10.0\u2009years, range 0.7\u201353.4) performed one supervised and four at\u2010home measurements using the PHE sensor, alongside DBS sampling for FIA\u2010MS\/MS. The sensor showed higher PHE values than FIA\u2010MS\/MS, with a mean difference of 84.8\u2009\u03bcmol\/L (\n                    <jats:italic>p<\/jats:italic>\n                    \u2009=\u20090.0017). Variability was influenced by test supervision (clinic vs. home) and test kit age (greater difference in older batches,\n                    <jats:italic>p<\/jats:italic>\n                    \u2009=\u20090.001). Usability of the PHE sensor and\n                    <jats:italic>phenyx<\/jats:italic>\n                    app was rated favorably; all patients preferred the PHE sensor over laboratory testing. PoC measurement using the PHE sensor and\n                    <jats:italic>phenyx<\/jats:italic>\n                    app is feasible, reliable, and provides rapid results comparable to FIA\u2010MS\/MS, representing a key step toward patient self\u2010testing.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>Trial Registration:<\/jats:bold>\n                    NCT05998109, NCT06940193, DRKS00031972.\n                  <\/jats:p>","DOI":"10.1002\/jimd.70187","type":"journal-article","created":{"date-parts":[[2026,4,16]],"date-time":"2026-04-16T14:44:39Z","timestamp":1776350679000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Real\u2010Life Application of a Point\u2010of\u2010Care Biosensor for Phenylalanine in Patients With Phenylketonuria"],"prefix":"10.1002","volume":"49","author":[{"given":"Corentin","family":"Gondrand","sequence":"first","affiliation":[{"name":"Department of Chemical Biology Max Planck Institute for Medical Research  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7766-4847","authenticated-orcid":false,"given":"Anna T.","family":"Reischl\u2010Hajiabadi","sequence":"additional","affiliation":[{"name":"Medical Faculty of Heidelberg, Department of Pediatrics, Division of Pediatric Neurology and Metabolic Medicine Heidelberg University  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Estelle","family":"Bonedeau","sequence":"additional","affiliation":[{"name":"Department of Chemical Biology Max Planck Institute for Medical Research  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicolas","family":"Wei\u00df","sequence":"additional","affiliation":[{"name":"Phellow Seven GmbH  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerd","family":"Schneider","sequence":"additional","affiliation":[{"name":"Phellow Seven GmbH  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maurice","family":"Fahn","sequence":"additional","affiliation":[{"name":"Phellow Seven GmbH  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frederic","family":"Myers","sequence":"additional","affiliation":[{"name":"Frederic Myers Consulting (JERY)  Reutlingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philipp Minel","family":"St\u00e4dle","sequence":"additional","affiliation":[{"name":"Frederic Myers Consulting (JERY)  Reutlingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathrin","family":"Jeltsch","sequence":"additional","affiliation":[{"name":"Medical Faculty of Heidelberg, Department of Pediatrics, Division of Pediatric Neurology and Metabolic Medicine Heidelberg University  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Georg F.","family":"Hoffmann","sequence":"additional","affiliation":[{"name":"Medical Faculty of Heidelberg, Department of Pediatrics, Division of Pediatric Neurology and Metabolic Medicine Heidelberg University  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00fcrgen G.","family":"Okun","sequence":"additional","affiliation":[{"name":"Medical Faculty of Heidelberg, Department of Pediatrics, Division of Pediatric Neurology and Metabolic Medicine Heidelberg University  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dorothea","family":"Haas","sequence":"additional","affiliation":[{"name":"Medical Faculty of Heidelberg, Department of Pediatrics, Division of Pediatric Neurology and Metabolic Medicine Heidelberg University  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7420-4757","authenticated-orcid":false,"given":"Sven F.","family":"Garbade","sequence":"additional","affiliation":[{"name":"Medical Faculty of Heidelberg, Department of Pediatrics, Division of Pediatric Neurology and Metabolic Medicine Heidelberg University  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kai","family":"Johnsson","sequence":"additional","affiliation":[{"name":"Department of Chemical Biology Max Planck Institute for Medical Research  Heidelberg Germany"},{"name":"\u00c9cole Polytechnique F\u00e9d\u00e9rale de Lausanne Institute of Chemical Sciences and Engineering, NCCR in Chemical Biology  Lausanne Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4349-7662","authenticated-orcid":false,"given":"Thomas","family":"Opladen","sequence":"additional","affiliation":[{"name":"Medical Faculty of Heidelberg, Department of Pediatrics, Division of Pediatric Neurology and Metabolic Medicine Heidelberg University  Heidelberg 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