{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,13]],"date-time":"2026-08-13T10:45:11Z","timestamp":1786617911223,"version":"build-2736575974"},"reference-count":18,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2024,8,19]],"date-time":"2024-08-19T00:00:00Z","timestamp":1724025600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Transplantation"],"published-print":{"date-parts":[[2024,9]]},"abstract":"ABSTRACT<\/jats:title>Introduction<\/jats:title>Maple syrup urine disease (MSUD) is caused by the deficiency of branched\u2010chain keto acid dehydrogenase (BCKAD) and, it is well described that BCKAD contributed by an allograft following liver transplantation (LT) phenotypically normalizes this inborn error of metabolism (IEM). There is, however, a paucity of data especially with regards to the neurodevelopmental aspects and catch\u2010up growth profiles after LT in a resource\u2010challenged setting. We present our series of children under 6\u2009years of age who underwent LT for MSUD particularly focusing on their amino acid homeostasis, neurodevelopmental and somatic growth profiles.<\/jats:p><\/jats:sec>Methods<\/jats:title>Of 580 consecutive pediatric LT (PLT) performed between January 2011 and December 2022, all children who underwent LT for MSUD were included for analysis. Data accrued included peri\u2010LT details, pre\u2010 and post\u2010LT metabolic profile, neurodevelopmental assessment, somatic growth evaluation, and long\u2010term outcomes.<\/jats:p><\/jats:sec>Results<\/jats:title>Six children underwent LT for MSUD with a median age and weight at LT of 20.5 (IQR: 8\u201360) months and 10.1 (IQR: 6.7\u201315.8) kg, respectively. One explanted liver was used as a domino graft for Arginase deficiency. Median follow\u2010up period was 52.5 (IQR: 27\u201394) months. None had vascular or biliary complications. Following LT, all children were started on an unrestricted protein diet and had normalization of BCAA levels. Post\u2010LT height and weight improved by 1 SD but did not achieve the normal profile. None of the children had neuro\u2010deterioration and have achieved new milestones.<\/jats:p><\/jats:sec>Conclusion<\/jats:title>This is the first\u2010report presenting the growth aspects, amino acid and neurodevelopmental profiles of children who underwent LT for MSUD within the socio\u2010economic\u2010cultural idiosyncrasies and constraints prevalent in our part of the world.<\/jats:p><\/jats:sec>","DOI":"10.1111\/petr.14845","type":"journal-article","created":{"date-parts":[[2024,8,19]],"date-time":"2024-08-19T11:13:21Z","timestamp":1724066001000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Long\u2010Term Amino Acid Homeostasis, Neurodevelopmental and Growth Profiles Following Liver Transplantation in Maple Syrup Urine Disease"],"prefix":"10.1111","volume":"28","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3342-6326","authenticated-orcid":false,"given":"Anu\u00a0K.","family":"Vasudevan","sequence":"first","affiliation":[{"name":"The Institute of Liver Disease and Transplantation, Dr. Rela Institute & Medical Centre Bharath Institute of Higher Education & Research Chennai India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naresh","family":"Shanmugam","sequence":"additional","affiliation":[{"name":"The Institute of Liver Disease and Transplantation, Dr. Rela Institute & Medical Centre Bharath Institute of Higher Education & Research Chennai India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9528-8892","authenticated-orcid":false,"given":"Ashwin","family":"Rammohan","sequence":"additional","affiliation":[{"name":"The Institute of Liver Disease and Transplantation, Dr. Rela Institute & Medical Centre Bharath Institute of Higher Education & Research Chennai India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Umamaheswari","family":"Balakrishnan","sequence":"additional","affiliation":[{"name":"The Institute of Liver Disease and Transplantation, Dr. Rela Institute & Medical Centre Bharath Institute of Higher Education & Research Chennai India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2649-0058","authenticated-orcid":false,"given":"Jagadeesh","family":"Menon","sequence":"additional","affiliation":[{"name":"The Institute of Liver Disease and Transplantation, Dr. Rela Institute & Medical Centre Bharath Institute of Higher Education & Research Chennai India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohammed\u00a0Asrar\u00a0Ul\u00a0Huq","family":"Iqbal","sequence":"additional","affiliation":[{"name":"The Institute of Liver Disease and Transplantation, Dr. Rela Institute & Medical Centre Bharath Institute of Higher Education & Research Chennai India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gehan\u00a0Mohammed\u00a0Osman","family":"Mohammed","sequence":"additional","affiliation":[{"name":"The Institute of Liver Disease and Transplantation, Dr. Rela Institute & Medical Centre Bharath Institute of Higher Education & Research Chennai India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ilankumaran","family":"Kaliamoorthy","sequence":"additional","affiliation":[{"name":"The Institute of Liver Disease and Transplantation, Dr. Rela Institute & Medical Centre Bharath Institute of Higher Education & Research Chennai India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohamed","family":"Rela","sequence":"additional","affiliation":[{"name":"The Institute of Liver Disease and Transplantation, Dr. Rela Institute & Medical Centre Bharath Institute of Higher Education & Research Chennai India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,8,19]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2016.01.005"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0000000000000469"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-6143.2005.01209.x"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgmr.2018.08.006"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2020.01.006"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/PETR.14603"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.61806"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/s0022\u20103476(98)70523\u20102"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejmg.2015.08.002"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.4103\/2249-4863.130279"},{"key":"e_1_2_9_12_1","first-page":"638","article-title":"A Study on the Humoral and Complement Immune System of Patients With Organic Acidemia","volume":"14","author":"Najjarbashi F. A.","year":"2015","journal-title":"Iranian Journal of Allergy, Asthma, and Immunology"},{"key":"e_1_2_9_13_1","first-page":"442","article-title":"Analysis of Gene Mutations Among South Indian Patients With Maple Syrup Urine Disease: Identification of Four Novel Mutations","volume":"50","author":"Narayanan M. P.","year":"2013","journal-title":"Indian Journal of Biochemistry & Biophysics"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1080\/13854046.2013.816372"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1093\/JN\/135.6.1531S"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.3390\/IJNS8020026\/S1"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12098-018-2681-5"},{"key":"e_1_2_9_18_1","unstructured":"\u201cNew Born Screening in India: A Time to Implement National Policy \u201dThe Indian Practitioner accessed June 30 2024 https:\/\/theindianpractitioner.com\/new\u2010born\u2010screening\u2010in\u2010india\u2010a\u2010time\u2010to\u2010implement\u2010national\u2010policy\/."},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.3390\/IJNS6020024"}],"container-title":["Pediatric Transplantation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/petr.14845","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,8,27]],"date-time":"2024-08-27T00:41:39Z","timestamp":1724719299000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/petr.14845"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,8,19]]},"references-count":18,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2024,9]]}},"alternative-id":["10.1111\/petr.14845"],"URL":"https:\/\/doi.org\/10.1111\/petr.14845","archive":["Portico"],"relation":{},"ISSN":["1397-3142","1399-3046"],"issn-type":[{"value":"1397-3142","type":"print"},{"value":"1399-3046","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,8,19]]},"assertion":[{"value":"2024-02-28","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-08-05","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-08-19","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e14845"}}