{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,27]],"date-time":"2026-08-27T19:20:25Z","timestamp":1787858425566,"version":"build-2784847793"},"reference-count":39,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2025,12,4]],"date-time":"2025-12-04T00:00:00Z","timestamp":1764806400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2025,12,4]],"date-time":"2025-12-04T00:00:00Z","timestamp":1764806400000},"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":[[2026,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Methylmalonic acidemia (MMA) and propionic acidemia (PA) are inherited metabolic disorders affecting valine and isoleucine catabolism. Long\u2010term therapy mainly involves dietary protein restriction. An amino acid mixture (AAM, medical food) free of the precursor amino acids is frequently used, especially when protein intake does not reach World Health Organization (WHO) recommendations. However, its clinical impact on disease control and patient outcomes remains unclear. Our study aimed to retrospectively review the dietary prescriptions in a cohort of vitamin B12\u2010unresponsive MMA and PA patients and to analyze their impact on clinical and laboratory parameters. Clinical data, anthropometric measurements and dietary prescriptions were collected from the patients' medical and dietary files. We included 71 patients (38\u2009MMA and 33 PA). Fifty\u2010nine percent of the patients' dietary prescriptions did not reach the safe WHO\u2010recommended daily total protein intake. Among these, 28% included AAM supplementation versus 62% in the group of patients that met the WHO recommendations (\n                    <jats:italic>p<\/jats:italic>\n                    \u2009&lt;\u20090.001). AAM was associated with a decrease in mean plasma concentrations of isoleucine and valine. These plasma amino acid concentrations were corrected by isoleucine and valine supplementation; however, leucine\/isoleucine and leucine\/valine ratios remained elevated in comparison to patients without AAM. Nutritional and clinical scores were worsened by AAM supplementation. We found that MMA\/PA patients receiving AAM tend to have altered plasma amino acid concentrations, raising concerns about potential long\u2010term deleterious consequences of AAM. We recommend prioritizing natural protein intake over AAM when possible, and if not, to carefully monitor and moderately supplement valine and isoleucine to prevent deficiencies.\n                  <\/jats:p>","DOI":"10.1002\/jimd.70114","type":"journal-article","created":{"date-parts":[[2025,12,5]],"date-time":"2025-12-05T01:14:52Z","timestamp":1764897292000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Nutritional Management in Severe Methylmalonic and Propionic Acidemias: How Much Medical Food Is Too Much?"],"prefix":"10.1002","volume":"49","author":[{"given":"Diane","family":"Margoses","sequence":"first","affiliation":[{"name":"Reference Centre for Inborn Errors of Metabolism Necker\u2010Enfants Malades Hospital, APHP, Fili\u00e8re G2m, MetabERN  Paris 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Centre for Inborn Errors of Metabolism Necker\u2010Enfants Malades Hospital, APHP, Fili\u00e8re G2m, MetabERN  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandrine","family":"Dubois","sequence":"additional","affiliation":[{"name":"Reference Centre for Inborn Errors of Metabolism Necker\u2010Enfants Malades Hospital, APHP, Fili\u00e8re G2m, MetabERN  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B\u00e9n\u00e9dicte","family":"Samba","sequence":"additional","affiliation":[{"name":"Reference Centre for Inborn Errors of Metabolism Necker\u2010Enfants Malades Hospital, APHP, Fili\u00e8re G2m, MetabERN  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aude","family":"Servais","sequence":"additional","affiliation":[{"name":"Reference Centre for Inborn Errors of Metabolism Necker\u2010Enfants Malades Hospital, APHP, Fili\u00e8re G2m, MetabERN  Paris France"},{"name":"Department of Adult Nephrology Necker\u2010Enfants Malades Hospital, APHP, Fili\u00e8re G2m, MetabERN  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Myriam","family":"Dao","sequence":"additional","affiliation":[{"name":"Reference Centre for Inborn Errors of Metabolism Necker\u2010Enfants Malades Hospital, APHP, Fili\u00e8re G2m, MetabERN  Paris France"},{"name":"Department of Adult Nephrology Necker\u2010Enfants Malades Hospital, APHP, Fili\u00e8re G2m, MetabERN  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edouard","family":"Le Guillou","sequence":"additional","affiliation":[{"name":"Biochemistry Laboratory Necker\u2010Enfants Malades Hospital, APHP  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Am\u00e9lie","family":"de Colnet","sequence":"additional","affiliation":[{"name":"Biochemistry Laboratory Necker\u2010Enfants Malades Hospital, APHP  Paris 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