{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T01:34:08Z","timestamp":1792373648152,"version":"4.4.0"},"reference-count":87,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2026,6,25]],"date-time":"2026-06-25T00:00:00Z","timestamp":1782345600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,6,25]],"date-time":"2026-06-25T00:00:00Z","timestamp":1782345600000},"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":[[2026,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Propionic acidemia (PA) is a rare inherited metabolic disorder associated with recurrent metabolic decompensations and chronic multisystemic complications. Liver transplantation (LT) may improve metabolic stability, but its long\u2010term impact on organ involvement remains debated. We retrospectively studied 20 patients with PA transplanted between 1993 and 2024 in three French reference centers. Clinical, biochemical, and organ\u2010specific data were collected before and after transplantation. Eighteen isolated LT and two combined liver\u2013kidney transplantations were performed. Median age at transplantation was 13.6\u2009years; median follow\u2010up was 4.5\u2009years. Indications included frequent metabolic decompensation (70%) and cardiomyopathy (35%). Four patients died perioperatively, three from cardiac causes; crude survival was 75%. Transplantation markedly improved metabolic stability and allowed significant dietary liberalization, with reduced need for enteral feeding. Cardiac involvement, present in 15 patients pre\u2010transplant, showed variable evolution: 10 improved initially, but three deteriorated later, and one required heart transplantation. New neurological manifestations occurred in eight patients, with acute episodes including CNI\u2010related encephalopathy. Psychiatric disorders progressed from 40% to 59% of patients, requiring treatment in seven. Renal impairment worsened post\u2010LT to 70%, with mean measured glomerular filtration rate declining from 72 to 60\u2009mL\/min\/1.73\u2009m\n                    <jats:sup>2<\/jats:sup>\n                    . LT improves metabolic and nutritional outcomes but does not consistently prevent chronic organ complications. In conclusion, early LT, before the onset of cardiac, neurologic, and psychiatric manifestations, should be considered on a case\u2010by\u2010case basis, as we could not demonstrate correlation between age at transplantation and organ involvement.\n                  <\/jats:p>","DOI":"10.1002\/jimd.70216","type":"journal-article","created":{"date-parts":[[2026,6,25]],"date-time":"2026-06-25T15:54:26Z","timestamp":1782402866000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Long Term Follow\u2010Up After Transplantation in Propionic Acidemia: A Retrospective French Pediatric and Adult Cohort Study"],"prefix":"10.1002","volume":"49","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5223-332X","authenticated-orcid":false,"given":"Tristan","family":"Mekdade","sequence":"first","affiliation":[{"name":"Reference Center of Inherited Metabolic Diseases, Necker Enfants Malades Hospital, APHP, Universit\u00e9 Paris 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