{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,8]],"date-time":"2026-07-08T17:29:50Z","timestamp":1783531790115,"version":"3.55.0"},"reference-count":25,"publisher":"MDPI AG","issue":"10","license":[{"start":{"date-parts":[[2020,9,29]],"date-time":"2020-09-29T00:00:00Z","timestamp":1601337600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Nutrients"],"abstract":"<jats:p>Methylmalonic acidemia (MMA) is caused by a deficiency of methyl-malonyl-CoA mutase. It is a multisystemic condition with poor clinical outcomes characterized by frequent metabolic decompensation with acidosis, hyperammonemia and encephalopathy. Restriction of intact protein and supplementation with amino acid-based formula play an important role in its management. Recently, liver transplant (LT) became a treatment option for MMA patients. However, there has been no current consensus on the post-operative nutrition management for MMA patients undergoing transplant, particularly during the initial phase of recovery period with catabolic stressors. We performed a retrospective analysis of clinical and nutritional management as well as biochemical profiles before and after LT in five patients with MMA. Through this study, we observed significant improvement of MMA-associated metabolites after LT. MMA patients were able to tolerate increased intact protein intake post-operatively. At least 1\u20131.5 g\/kg\/day of total protein during the acute phase after transplant may be tolerated without worsening of the metabolite levels. This information provides a guide in how to nutritionally manage MMA after LT.<\/jats:p>","DOI":"10.3390\/nu12102976","type":"journal-article","created":{"date-parts":[[2020,9,29]],"date-time":"2020-09-29T08:43:27Z","timestamp":1601369007000},"page":"2976","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Nutritional Management and Biochemical Outcomes during the Immediate Phase after Liver Transplant for Methylmalonic Acidemia"],"prefix":"10.3390","volume":"12","author":[{"given":"Casey","family":"Siegel","sequence":"first","affiliation":[{"name":"Department of Genetics and Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ronen","family":"Arnon","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA"},{"name":"Recanati\/Miller Transplantation Institute, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sander","family":"Florman","sequence":"additional","affiliation":[{"name":"Recanati\/Miller Transplantation Institute, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Bucuvalas","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA"},{"name":"Recanati\/Miller Transplantation Institute, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9446-8912","authenticated-orcid":false,"given":"Kimihiko","family":"Oishi","sequence":"additional","affiliation":[{"name":"Department of Genetics and Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA"},{"name":"Department of Pediatrics, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2020,9,29]]},"reference":[{"key":"ref_1","unstructured":"Adam, M.P., Ardinger, H.H., Pagon, R.A., Wallace, S.E., Bean, L.J.H., Stephens, K., and Amemiya, A. (1993). Isolated Methylmalonic Acidemia. GeneReviews(R), University of Washington."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"682","DOI":"10.1097\/MOP.0000000000000422","article-title":"Methylmalonic and propionic acidemias: Clinical management update","volume":"28","author":"Fraser","year":"2016","journal-title":"Curr. Opin. Pediatr."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"130","DOI":"10.1186\/s13023-014-0130-8","article-title":"Proposed guidelines for the diagnosis and management of methylmalonic and propionic acidemia","volume":"9","author":"Baumgartner","year":"2014","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"730","DOI":"10.1002\/jimd.12129","article-title":"Pathophysiology of propionic and methylmalonic acidemias. Part 1: Complications","volume":"42","author":"Haijes","year":"2019","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1059","DOI":"10.1007\/s10545-015-9840-x","article-title":"The phenotypic spectrum of organic acidurias and urea cycle disorders. Part 2: The evolving clinical phenotype","volume":"38","author":"Kolker","year":"2015","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"225","DOI":"10.1203\/PDR.0b013e3180a0325f","article-title":"Long-term outcome in methylmalonic acidurias is influenced by the underlying defect (mut0, mut-, cblA, cblB)","volume":"62","author":"Horster","year":"2007","journal-title":"Pediatr. Res."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"756","DOI":"10.1111\/petr.12741","article-title":"Liver transplantation for pediatric inherited metabolic disorders: Considerations for indications, complications, and perioperative management","volume":"20","author":"Oishi","year":"2016","journal-title":"Pediatr. Transplant."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"431","DOI":"10.1016\/j.ymgme.2019.11.001","article-title":"Liver transplantation in propionic and methylmalonic acidemia: A single center study with literature review","volume":"128","author":"Pillai","year":"2019","journal-title":"Mol. Genet. Metab."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"322","DOI":"10.1016\/j.ymgme.2006.04.003","article-title":"Liver transplantation is not curative for methylmalonic acidopathy caused by methylmalonyl-CoA mutase deficiency","volume":"88","author":"Kaplan","year":"2006","journal-title":"Mol. Genet. Metab."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"899","DOI":"10.1007\/s10545-014-9730-7","article-title":"A detailed analysis of methylmalonic acid kinetics during hemodialysis and after combined liver\/kidney transplantation in a patient with mut (0) methylmalonic acidemia","volume":"37","author":"Vernon","year":"2014","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"192","DOI":"10.1177\/0884533614521242","article-title":"Nutrition management after pediatric solid organ transplantation","volume":"29","author":"Asfaw","year":"2014","journal-title":"Nutr. Clin. Pract."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"706","DOI":"10.1177\/0148607117711387","article-title":"Guidelines for the Provision and Assessment of Nutrition Support Therapy in the Pediatric Critically Ill Patient: Society of Critical Care Medicine and American Society for Parenteral and Enteral Nutrition","volume":"41","author":"Mehta","year":"2017","journal-title":"JPEN J. Parenter. Enteral. Nutr."},{"key":"ref_13","first-page":"146","article-title":"Increased protein catabolism without hypermetabolism after human orthotopic liver transplantation","volume":"101","author":"Shanbhogue","year":"1987","journal-title":"Surgery"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"470","DOI":"10.21037\/hbsn.2019.03.04","article-title":"Perioperative characteristics and management of liver transplantation for isolated methylmalonic acidemia-the largest experience in China","volume":"8","author":"Jiang","year":"2019","journal-title":"Hepatobiliary Surg. Nutr."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"1866","DOI":"10.1007\/s12325-020-01305-1","article-title":"Post-transplantation Outcomes in Patients with PA or MMA: A Review of the Literature","volume":"37","author":"Yap","year":"2020","journal-title":"Adv. Ther."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"1260","DOI":"10.1002\/lt.25304","article-title":"Liver Transplantation for Propionic Acidemia and Methylmalonic Acidemia: Perioperative Management and Clinical Outcomes","volume":"24","author":"Critelli","year":"2018","journal-title":"Liver Transpl."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1455","DOI":"10.1016\/j.jpeds.2015.01.051","article-title":"Treatment of methylmalonic acidemia by liver or combined liver-kidney transplantation","volume":"166","author":"Niemi","year":"2015","journal-title":"J. Pediatr."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"1346","DOI":"10.1016\/j.jpeds.2015.03.026","article-title":"Liver or combined liver-kidney transplantation for patients with isolated methylmalonic acidemia: Who and when?","volume":"166","author":"Sloan","year":"2015","journal-title":"J. Pediatr."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1173","DOI":"10.1016\/j.jpeds.2015.08.022","article-title":"Liver transplantation in severe methylmalonic acidemia: The sooner, the better","volume":"167","author":"Spada","year":"2015","journal-title":"J. Pediatr."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"e252","DOI":"10.1542\/peds.2015-0175","article-title":"Early Liver Transplantation for Neonatal-Onset Methylmalonic Acidemia","volume":"136","author":"Spada","year":"2015","journal-title":"Pediatrics"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"1310","DOI":"10.1002\/lt.20894","article-title":"Nutrition for the liver transplant patient","volume":"12","author":"Sanchez","year":"2006","journal-title":"Liver Transpl."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"e13407","DOI":"10.1111\/petr.13407","article-title":"Kidney disease and organ transplantation in methylmalonic acidaemia","volume":"23","author":"Noone","year":"2019","journal-title":"Pediatr. Transplant."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1196\/annals.1320.004","article-title":"Carnitine deficiency disorders in children","volume":"1033","author":"Stanley","year":"2004","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"576","DOI":"10.1038\/nm.3145","article-title":"Intestinal microbiota metabolism of L-carnitine, a nutrient in red meat, promotes atherosclerosis","volume":"19","author":"Koeth","year":"2013","journal-title":"Nat. Med."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"20","DOI":"10.1016\/j.ymgme.2008.10.003","article-title":"Rare Diseases Clinical Research, N. Clinical research for rare disease: Opportunities, challenges, and solutions","volume":"96","author":"Griggs","year":"2009","journal-title":"Mol. Genet. Metab."}],"container-title":["Nutrients"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2072-6643\/12\/10\/2976\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T10:14:43Z","timestamp":1760177683000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2072-6643\/12\/10\/2976"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,9,29]]},"references-count":25,"journal-issue":{"issue":"10","published-online":{"date-parts":[[2020,10]]}},"alternative-id":["nu12102976"],"URL":"https:\/\/doi.org\/10.3390\/nu12102976","relation":{},"ISSN":["2072-6643"],"issn-type":[{"value":"2072-6643","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,9,29]]}}}