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Both conditions can lead to harmful levels of keto acids in biofluids\u2014phenylpyruvic acid (PPA) in PKU and branched\u2010chain \u03b1\u2010keto acids in MSUD. Monitoring urinary keto acids helps track dietary adherence and reduces the risk of metabolic crisis. However, current at\u2010home tests are qualitative and difficult to interpret, while existing metabolomic assays require expensive equipment and must be conducted in a lab. This study aimed to develop a simple, quantitative, rapid, at\u2010home assay for detecting multiple urinary keto acids associated with PKU and MSUD. A modified two\u2010step 2,4\u2010dinitrophenylhydrazine (DNPH)\u2013based multimetabolite assay was developed, where sodium hydroxide (NaOH) converts the yellow hydrazone precipitate to a stable amber solution, enabling quantification of multiple keto acids (700\u20137200\u2009\u03bcM) within 10\u2009min. The assay was validated using spiked urine samples and adapted into a prototype at\u2010home kit using caprolactam\u2010immobilized NaOH. Nuclear magnetic resonance (NMR)\u2013based metabolomics was used as a reference method to authenticate readings from a PKU patient. Correlation studies demonstrated strong linearity for MSUD (\n                    <jats:italic>R<\/jats:italic>\n                    <jats:sup>2<\/jats:sup>\n                    \u2009=\u20090.91\u20130.96)\u2010 and PKU (\n                    <jats:italic>R<\/jats:italic>\n                    <jats:sup>2<\/jats:sup>\n                    \u2009=\u20090.95\u20130.99)\u2010spiked samples. Quantification of keto acids in authentic PKU urine samples showed excellent agreement with the results of quantitative NMR\u2010based metabolomics assays (\n                    <jats:italic>R<\/jats:italic>\n                    <jats:sup>2<\/jats:sup>\n                    \u2009=\u20090.99). Low\u2010cost, at\u2010home colorimetric tests for urinary keto acids could enable screening, detection, and monitoring of PKU and MSUD in the 90% of the world without access to advanced metabolic clinics.\n                  <\/jats:p>","DOI":"10.1155\/jamc\/4116313","type":"journal-article","created":{"date-parts":[[2026,3,8]],"date-time":"2026-03-08T22:15:23Z","timestamp":1773008123000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["A Colorimetric Multimetabolite Assay for Quantitative Measurement of Keto Acids in Urine for At\u2010Home Monitoring of Metabolic Disorders"],"prefix":"10.1155","volume":"2026","author":[{"ORCID":"https:\/\/orcid.org\/0009-0000-3259-2993","authenticated-orcid":false,"given":"Dipanjan","family":"Bhattacharyya","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Abby","family":"Kropielnicki","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Brian L.","family":"Lee","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-3591-113X","authenticated-orcid":false,"given":"Yeganeh","family":"Khaniani","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5657-6661","authenticated-orcid":false,"given":"Marcia A.","family":"LeVatte","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3207-2434","authenticated-orcid":false,"given":"David S.","family":"Wishart","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2026,2,27]]},"reference":[{"key":"e_1_2_14_1_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0076-6879(00)24254-7"},{"key":"e_1_2_14_2_2","first-page":"1971","volume-title":"The Online Metabolic and Molecular Bases of Inherited Disease","author":"Chuang D. 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