{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T23:03:41Z","timestamp":1790550221763,"version":"4.1.0"},"reference-count":31,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"9","license":[{"start":{"date-parts":[[2018,9,1]],"date-time":"2018-09-01T00:00:00Z","timestamp":1535760000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"},{"start":{"date-parts":[[2018,9,24]],"date-time":"2018-09-24T00:00:00Z","timestamp":1537747200000},"content-version":"vor","delay-in-days":23,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Liver Transpl"],"published-print":{"date-parts":[[2018,9]]},"abstract":"<jats:title>Abstract<\/jats:title>\n          <jats:p>Propionic acidemia (PA) and methylmalonic acidemia (MMA) comprise the most common organic acidemias and account for profound morbidity in affected individuals. Although liver transplantation (LT) has emerged as a bulk enzyme\u2010replacement strategy to stabilize metabolically fragile patients, it is not a metabolic cure because patients remain at risk for disease\u2010related complications. We retrospectively studied LT and\/or liver\u2010kidney transplant in 9 patients with PA or MMA with additional focus on the optimization of metabolic control and management in the perioperative period. Metabolic crises were common before transplant. By implementing a strategy of carbohydrate minimization with gradual but early lipid and protein introduction, lactate levels significantly improved over the perioperative period (<jats:italic toggle=\"yes\">P <\/jats:italic>&lt; 0.001). Posttransplant metabolic improvement is demonstrated by improvements in serum glycine levels (for PA; <jats:italic toggle=\"yes\">P <\/jats:italic>&lt; 0.001 \u00d7 10<jats:sup>\u201314<\/jats:sup>), methylmalonic acid levels (for MMA; <jats:italic toggle=\"yes\">P <\/jats:italic>&lt; 0.001), and ammonia levels (for PA and MMA; <jats:italic toggle=\"yes\">P <\/jats:italic>&lt; 0.001). Dietary restriction remained after transplant. However, no further metabolic crises have occurred. Other disease\u2010specific comorbidities such as renal dysfunction and cardiomyopathy stabilized and improved. In conclusion, transplant can provide a strategy for altering the natural history of PA and MMA providing stability to a rare but metabolically brittle population. Nutritional management is critical to optimize patient outcomes.<\/jats:p>","DOI":"10.1002\/lt.25304","type":"journal-article","created":{"date-parts":[[2018,8,7]],"date-time":"2018-08-07T11:14:53Z","timestamp":1533640493000},"page":"1260-1270","source":"Crossref","is-referenced-by-count":81,"title":["Liver Transplantation for Propionic Acidemia and Methylmalonic Acidemia: Perioperative Management and Clinical Outcomes"],"prefix":"10.1097","volume":"24","author":[{"given":"Kristen","family":"Critelli","sequence":"first","affiliation":[{"name":"Division of Gastroenterology, Hepatology and Nutrition,Children\u2019s Hospital of Pittsburgh, University of Pittsburgh Medical Center,Pittsburgh,PA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick","family":"McKiernan","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, Hepatology and Nutrition,Children\u2019s Hospital of Pittsburgh, University of Pittsburgh Medical Center,Pittsburgh,PA"},{"name":"Center for Rare Disease Therapy,Children\u2019s Hospital of Pittsburgh, University of Pittsburgh Medical Center,Pittsburgh,PA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jerry","family":"Vockley","sequence":"additional","affiliation":[{"name":"Center for Rare Disease Therapy,Children\u2019s Hospital of Pittsburgh, University of Pittsburgh Medical Center,Pittsburgh,PA"},{"name":"Division of Medical Genetics,Children\u2019s Hospital of Pittsburgh, University of Pittsburgh Medical Center,Pittsburgh,PA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"George","family":"Mazariegos","sequence":"additional","affiliation":[{"name":"Center for Rare Disease Therapy,Children\u2019s Hospital of Pittsburgh, University of Pittsburgh Medical Center,Pittsburgh,PA"},{"name":"Thomas E. Starzl Transplantation Institute, Hillman Center for Pediatric Transplantation, Department of Transplant Surgery,Children\u2019s Hospital of Pittsburgh, University of Pittsburgh Medical Center,Pittsburgh,PA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert H.","family":"Squires","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, Hepatology and Nutrition,Children\u2019s Hospital of Pittsburgh, University of Pittsburgh Medical Center,Pittsburgh,PA"},{"name":"Center for Rare Disease Therapy,Children\u2019s Hospital of Pittsburgh, University of Pittsburgh Medical Center,Pittsburgh,PA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kyle","family":"Soltys","sequence":"additional","affiliation":[{"name":"Center for Rare Disease Therapy,Children\u2019s Hospital of Pittsburgh, University of Pittsburgh Medical Center,Pittsburgh,PA"},{"name":"Thomas E. Starzl Transplantation Institute, Hillman Center for Pediatric Transplantation, Department of Transplant Surgery,Children\u2019s Hospital of Pittsburgh, University of Pittsburgh Medical Center,Pittsburgh,PA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James E.","family":"Squires","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, Hepatology and Nutrition,Children\u2019s Hospital of Pittsburgh, University of Pittsburgh Medical Center,Pittsburgh,PA"},{"name":"Center for Rare Disease Therapy,Children\u2019s Hospital of Pittsburgh, University of Pittsburgh Medical Center,Pittsburgh,PA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2018,9,24]]},"reference":[{"key":"lt25304-bib-0001-20240115","first-page":"106","article-title":"Incidence of maple syrup urine disease, propionic acidemia, and mathylmalonic aciduria from newborn screening 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