{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T14:44:09Z","timestamp":1787064249360,"version":"build-2736575974"},"reference-count":28,"publisher":"Springer Science and Business Media LLC","issue":"8","license":[{"start":{"date-parts":[[2023,2,25]],"date-time":"2023-02-25T00:00:00Z","timestamp":1677283200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2023,2,25]],"date-time":"2023-02-25T00:00:00Z","timestamp":1677283200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Pediatr Nephrol"],"published-print":{"date-parts":[[2023,8]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>As modern medicine is advancing, younger, small, and more complex children are becoming multi-organ transplant candidates. This brings up new challenges in all aspects of their care.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We describe the first report of a small child receiving a simultaneous liver and kidney transplant and abdominal rectus sheath fascia transplant on the background of Williams syndrome and methylmalonic acidaemia. At the time of transplantation, the child was 3\u00a0years old, weighed 14.0\u00a0kg, had chronic kidney disease stage V, and had not yet started any other form of kidney replacement therapy.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>There were many anaesthetic, medical, metabolic, and surgical challenges to consider in this case. A long general anaesthetic time increased the risk of cardiac complications and metabolic decompensation. Additionally, the small size of the patient and the organ size mis-match meant that primary abdominal closure was not possible. The patient\u2019s recovery was further complicated by sepsis, transient CNI toxicity, and de novo DSAs.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Through a multidisciplinary approach between 9 specialties in 4 hospitals across England and Wales, and detailed pre-operative planning, a good outcome was achieved for this child. An hour by hour management protocol was drafted to facilitate transplant and included five domains: 1. management at the time of organ offer; 2. before the admission; 3. at admission and before theatre time; 4. intra-operative management; and 5. post-operative management in the first 24\u00a0h. Importantly, gaining a clear and in depth understanding of the metabolic state of the patient pre- and peri-operatively was crucial in avoiding metabolic decompensation. Furthermore, an abdominal rectus sheath fascia transplant was required to achieve abdominal closure, which to our knowledge, had never been done before for this indication. Using our experience of this complex case, as well as our experience in transplanting other children with MMA, and through a literature review, we propose a new perioperative management pathway for this complex cohort of transplant recipients.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s00467-023-05906-0","type":"journal-article","created":{"date-parts":[[2023,2,25]],"date-time":"2023-02-25T06:02:44Z","timestamp":1677304964000},"page":"2887-2896","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Transplantation in paediatric patients with MMA requires multidisciplinary approach for achievement of good clinical outcomes"],"prefix":"10.1007","volume":"38","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3891-904X","authenticated-orcid":false,"given":"Alicia","family":"Paessler","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miriam","family":"Cortes-Cerisuelo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wayel","family":"Jassem","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hector","family":"Vilca-Melendez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akash","family":"Deep","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vandana","family":"Jain","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew","family":"Pool","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephanie","family":"Grunewald","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicos","family":"Kessaris","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jelena","family":"Stojanovic","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2023,2,25]]},"reference":[{"key":"5906_CR1","doi-asserted-by":"publisher","first-page":"1071","DOI":"10.1007\/s00467-004-1572-3","volume":"19","author":"F H\u00f6rster","year":"2004","unstructured":"H\u00f6rster F, Hoffmann GF (2004) Pathophysiology, diagnosis, and treatment of methylmalonic aciduria\u2014recent advances and new challenges. Pediatr Nephrol 19:1071\u20131074","journal-title":"Pediatr Nephrol"},{"key":"5906_CR2","doi-asserted-by":"publisher","first-page":"943","DOI":"10.1111\/j.1399-3046.2006.00585.x","volume":"10","author":"M Kasahara","year":"2006","unstructured":"Kasahara M, Horikawa R, Tagawa M, Uemoto S, Yokoyama S, Shibata Y, Kawano T, Kuroda, T, Honna T, Tanaka K, Saeki M (2006) Current role of liver transplantation for methylmalonic acidemia: a review of the literature. Pediatr Transplant 10:943\u2013947","journal-title":"Pediatr Transplant"},{"key":"5906_CR3","doi-asserted-by":"crossref","unstructured":"Critelli K, McKiernan P, Vockley J, Mazariegos G, Squires RH, Soltys K, Squires JE (2018) Liver transplantation for propionic acidemia and methylmalonic acidemia: perioperative management and clinical outcomes. Liver Transpl 24:1260\u20131270","DOI":"10.1002\/lt.25304"},{"key":"5906_CR4","doi-asserted-by":"publisher","first-page":"1081","DOI":"10.1111\/petr.12804","volume":"20","author":"R Sakamoto","year":"2016","unstructured":"Sakamoto R, Nakamura K, Kido J, Matsumoto S, Mitsubuchi H, Inomata Y, Endo F (2016) Improvement in the prognosis and development of patients with methylmalonic acidemia after living donor liver transplant. Pediatr Transplant 20:1081\u20131086","journal-title":"Pediatr Transplant"},{"key":"5906_CR5","doi-asserted-by":"publisher","first-page":"e252","DOI":"10.1542\/peds.2015-0175","volume":"136","author":"M Spada","year":"2015","unstructured":"Spada M, Calvo PL, Brunati A, Peruzzi L, Dell\u2019Olio D, Romagnoli R, Porta F (2015) Early liver transplantation for neonatal-onset methylmalonic acidemia. Pediatrics 136:e252\u2013e256","journal-title":"Pediatrics"},{"key":"5906_CR6","doi-asserted-by":"publisher","first-page":"106","DOI":"10.1016\/j.ymgme.2013.05.001","volume":"110","author":"A Brassier","year":"2013","unstructured":"Brassier A, Boyer O, Valayannopoulos V, Ottolenghi C, Krug P, Cosson MA, Touati G, Arnoux J B, Barbier V, Bahi-Buisson N, Desguerre I, Charbit M, Benoist J F, Dupic L, Aigrain Y, Blanc T, Salomon R, Rabier D, Guest G, de Lonlay P, Niaudet P (2013) Renal transplantation in 4 patients with methylmalonic aciduria: a cell therapy for metabolic disease. Mol Genet Metab 110:106\u2013110","journal-title":"Mol Genet Metab"},{"key":"5906_CR7","doi-asserted-by":"publisher","first-page":"593","DOI":"10.1002\/jimd.12318","volume":"44","author":"F Molema","year":"2021","unstructured":"Molema F, Martinelli D, H\u00f6rster F, K\u00f6lker S, Tangeraas T, de Koning B, Dionisi-Vici C, Williams M (2021) Liver and\/or kidney transplantation in amino and organic acid-related inborn errors of metabolism: An overview on European data. J Inherit Metab Dis 44:593\u2013605","journal-title":"J Inherit Metab Dis"},{"key":"5906_CR8","doi-asserted-by":"publisher","first-page":"1455","DOI":"10.1016\/j.jpeds.2015.01.051","volume":"166","author":"AK Niemi","year":"2015","unstructured":"Niemi AK, Kim IK, Krueger CE, Cowan TM, Baugh N, Farrell R, Bonham CA, Concepcion W, Esquivel CO, Enns GM (2015) Treatment of methylmalonic acidemia by liver or combined liver-kidney transplantation. J Pediatr 166:1455\u20131461.e1","journal-title":"J Pediatr"},{"key":"5906_CR9","doi-asserted-by":"publisher","first-page":"234","DOI":"10.1002\/jimd.12174","volume":"43","author":"A Brassier","year":"2020","unstructured":"Brassier A, Krug P, Lacaille F, Pontoizeau C, Krid S, Sissaoui S, Servais A, Arnoux JB, Legendre C, Charbit M, Scemla A, Francoz C, Benoist JF, Schiff M, Mochel F, Touati G, Brou\u00e9 P, Capno A, Tardieu M, Querciagrossa S, Gr\u00e9vent D, Boyer O, Dupic L, Oualha M, Girard M, Aigrain Y, Debray D, Capito C, Ottolenghi C, Salomon R, Chardot C, de Lonlay P (2020) Long-term outcome of methylmalonic aciduria after kidney, liver, or combined liver-kidney transplantation: The French experience. J Inherit Metab Dis 43:234\u2013243","journal-title":"J Inherit Metab Dis"},{"key":"5906_CR10","doi-asserted-by":"publisher","first-page":"1346","DOI":"10.1016\/j.jpeds.2015.03.026","volume":"166","author":"JL Sloan","year":"2015","unstructured":"Sloan JL, Manoli I, Venditti CP (2015) Liver or combined liver-kidney transplantation for patients with isolated methylmalonic acidemia: who and when? J Pediatr 166:1346\u20131350","journal-title":"J Pediatr"},{"key":"5906_CR11","doi-asserted-by":"publisher","first-page":"265","DOI":"10.1016\/j.ymgme.2022.09.010","volume":"137","author":"L Dello Strologo","year":"2022","unstructured":"Dello Strologo L, Spada M, Vici CD, Atti MCD, Rheault M, Bjerre AK, Boyer O, Calvo PL, D'antiga L, Harshman LA, H\u00f6rster F, K\u00f6lker S, Jahnukainen T, Knops N, Krug P, Krupka K, Lee A, Levtchenko E, Marks SD, Stojanovic J, Martelli L, Mazariegos G, Montini G, Shenoy M, Sidhu S, Spada M, Tangeras T, Testa S, Vijay S, Wac K, Wennberg L, Concepcion W, Garbade S, T\u00f6nshoff B (2022) Renal outcome and plasma methylmalonic acid levels after isolated or combined liver or kidney transplantation in patients with methylmalonic acidemia: A multicenter analysis. Mol Genet Metab 137:265\u2013272","journal-title":"Mol Genet Metab"},{"key":"5906_CR12","doi-asserted-by":"publisher","first-page":"1866","DOI":"10.1007\/s12325-020-01305-1","volume":"37","author":"S Yap","year":"2020","unstructured":"Yap S, Vara R, Morais A (2020) Post-transplantation Outcomes in Patients with PA or MMA: A Review of the Literature. Adv Ther 37:1866\u20131896","journal-title":"Adv Ther"},{"key":"5906_CR13","doi-asserted-by":"publisher","first-page":"6","DOI":"10.1111\/petr.12196","volume":"18","author":"M Kasahara","year":"2014","unstructured":"Kasahara M, Sakamoto S, Horikawa R, Koji U, Mizuta K, Shinkai M, Takahito Y, Taguchi T, Inomata Y, Uemoto S, Tatsuo K, Kato S (2014) Living donor liver transplantation for pediatric patients with metabolic disorders: the Japanese multicenter registry. Pediatr Transplant 18:6\u201315","journal-title":"Pediatr Transplant"},{"key":"5906_CR14","doi-asserted-by":"publisher","first-page":"2754","DOI":"10.1111\/j.1600-6143.2005.01084.x","volume":"5","author":"D Morioka","year":"2005","unstructured":"Morioka D, Kasahara M, Takada Y, Corrales JP, Yoshizawa A, Sakamoto S, Taira K, Yoshitoshi EY, Egawa H, Shimada H, Tanaka K (2005) Living donor liver transplantation for pediatric patients with inheritable metabolic disorders. Am J Transplant 5:2754\u20132763","journal-title":"Am J Transplant"},{"key":"5906_CR15","doi-asserted-by":"publisher","first-page":"2782","DOI":"10.1111\/j.1600-6143.2007.01986.x","volume":"7","author":"D Morioka","year":"2007","unstructured":"Morioka D, Kasahara M, Horikawa R, Yokoyama S, Fukuda A, Nakagawa A (2007) Efficacy of living donor liver transplantation for patients with methylmalonic acidemia. Am J Transplant 7:2782\u20132787","journal-title":"Am J Transplant"},{"key":"5906_CR16","doi-asserted-by":"publisher","first-page":"483","DOI":"10.1111\/pan.13620","volume":"29","author":"MD Twite","year":"2019","unstructured":"Twite MD, Stenquist S, Ing RJ (2019) Williams syndrome. Pediatr Anaesth 29:483\u2013490","journal-title":"Pediatr Anaesth"},{"key":"5906_CR17","doi-asserted-by":"publisher","first-page":"130","DOI":"10.1186\/s13023-014-0130-8","volume":"9","author":"MR Baumgartner","year":"2014","unstructured":"Baumgartner MR, H\u00f6rster F, Dionisi-Vici C, Haliloglu G, Karall D, Chapman KA, Huemer M, Hochuli M, Assoun M, Ballhausen D, Burlina A, Fowler B, Gr\u00fcnert SC, Gr\u00fcnewald S, Honzik T, Merinero B, P\u00e9rez-Cerd\u00e1 C, Scholl-B\u00fcrgi S, Skovby F, Wijburg F, MacDonald A, Martinelli D, Sass JO, Valayannopoulos V, Chakrapani A (2014) Proposed guidelines for the diagnosis and management of methylmalonic and propionic acidemia. Orphanet J Rare Dis 9:130","journal-title":"Orphanet J Rare Dis"},{"issue":"S3","key":"5906_CR18","doi-asserted-by":"publisher","first-page":"S547","DOI":"10.1097\/01.tp.0000701476.97048.84","volume":"104","author":"P Vuong","year":"2020","unstructured":"Vuong P, Ebel N, Baker CV, Brubaker A, Than P, Enns G, Esquivel C (2020) Perioperative management and post-liver transplantation outcomes in methylmalonic acidemia, propionic acidemia and urea cycle disorders: our 20 year experience. Transplantation 104(S3):S547","journal-title":"Transplantation"},{"key":"5906_CR19","doi-asserted-by":"publisher","first-page":"44","DOI":"10.4103\/0971-9784.58834","volume":"13","author":"P Gupta","year":"2010","unstructured":"Gupta P, Tobias JD, Goyal S, Miller MD, Melendez E, Noviski N, De Moor MM, Mehta V (2010) Sudden cardiac death under anesthesia in pediatric patient with Williams syndrome: A case report and review of literature. Ann Card Anaesth 13:44\u201348","journal-title":"Ann Card Anaesth"},{"key":"5906_CR20","first-page":"876","volume":"52","author":"H Sato","year":"2003","unstructured":"Sato H, Okawa I, Matsukawa T, Sasuga M, Tamaki F, Kumazawa T (2003) Two occasions of anesthetic management for a patient with Williams syndrome. Masui 52:876\u2013878","journal-title":"Masui"},{"key":"5906_CR21","doi-asserted-by":"publisher","first-page":"1018","DOI":"10.4103\/ija.IJA_463_17","volume":"61","author":"N Shaikh","year":"2017","unstructured":"Shaikh N, Hashmi MG, Shah C, Dhansura T (2017) Anaesthetic considerations in a patient with methylmalonyl-coenzyme A mutase deficiency. Indian J Anaesth 61:1018\u20131020","journal-title":"Indian J Anaesth"},{"key":"5906_CR22","doi-asserted-by":"publisher","first-page":"245","DOI":"10.1007\/s00467-019-04362-z","volume":"36","author":"J Chandar","year":"2021","unstructured":"Chandar J, Chen L, Defreitas M, Ciancio G, Burke G 3rd (2021) Donor considerations in pediatric kidney transplantation. Pediatr Nephrol 36:245\u2013257","journal-title":"Pediatr Nephrol"},{"key":"5906_CR23","doi-asserted-by":"publisher","first-page":"e124351","DOI":"10.1172\/jci.insight.124351","volume":"3","author":"I Manoli","year":"2018","unstructured":"Manoli I, Sysol JR, Epping MW, Li L, Wang C, Sloan JL, Pass A, Gagn\u00e9 J, Ktena YP, Li L, Trivedi NS, Ouattara B, Zerfas PM, Hoffmann V, Abu-Asab M, Tsokos MG, Kleiner DE, Garone C, Cusmano-Ozog K, Enns GM, Vernon HJ, Andersson HC, Grunewald S, Elkahloun AG, Girard CL, Schnermann J, DiMauro S, Andres-Mateos E, Vandenberghe LH, Chandler RJ, Venditti CP (2018) FGF21 underlies a hormetic response to metabolic stress in methylmalonic acidemia. JCI Insight 3:e124351","journal-title":"JCI Insight"},{"key":"5906_CR24","doi-asserted-by":"publisher","first-page":"132","DOI":"10.1002\/jimd.12478","volume":"45","author":"N Longo","year":"2022","unstructured":"Longo N, Sass JO, Jurecka A, Vockley J (2022) Biomarkers for drug development in propionic and methylmalonic acidemias. J Inherit Metab Dis 45:132\u2013143","journal-title":"J Inherit Metab Dis"},{"key":"5906_CR25","doi-asserted-by":"publisher","first-page":"e13473","DOI":"10.1111\/petr.13473","volume":"23","author":"N Cassar","year":"2019","unstructured":"Cassar N, Cortes-Cerisuelo M, Bambridge C, Ali A, Heaton N, Vilca-Melendez H (2019) The difficult abdominal closure after paediatric intestinal transplantation: Use of abdominal rectus muscle fascia and literature review. Pediatr Transplant 23:e13473","journal-title":"Pediatr Transplant"},{"key":"5906_CR26","doi-asserted-by":"publisher","first-page":"711","DOI":"10.1097\/TP.0b013e3181b29f0c","volume":"88","author":"MT Perera","year":"2009","unstructured":"Perera MT, Silva MA, Sharif K, McKiernan PJ, Kelly DA, Lloyd C, Milford DV, Mayer DA, Mirza DF (2009) Improved outcomes of combined liver and kidney transplants in small children (<15 kg). Transplantation 88:711\u2013715","journal-title":"Transplantation"},{"key":"5906_CR27","doi-asserted-by":"publisher","first-page":"5312","DOI":"10.3748\/wjg.v24.i47.5312","volume":"24","author":"C Hackl","year":"2018","unstructured":"Hackl C, Schmidt KM, S\u00fcsal C, D\u00f6hler B, Zidek M, Schlitt HJ (2018) Split liver transplantation: Current developments. World J Gastroenterol 24:5312\u20135321","journal-title":"World J Gastroenterol"},{"key":"5906_CR28","doi-asserted-by":"publisher","first-page":"100634","DOI":"10.1016\/j.trre.2021.100634","volume":"35","author":"Y Janssen","year":"2021","unstructured":"Janssen Y, Van De Winkel N, Pirenne J, Ceulemans LJ, Miserez M (2021) Allotransplantation of donor rectus fascia for abdominal wall closure in transplant patients: A systematic review. Transplant Rev (Orlando) 35:100634","journal-title":"Transplant Rev (Orlando)"}],"container-title":["Pediatric Nephrology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00467-023-05906-0.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00467-023-05906-0\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00467-023-05906-0.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,1]],"date-time":"2023-08-01T03:13:10Z","timestamp":1690859590000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00467-023-05906-0"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,2,25]]},"references-count":28,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2023,8]]}},"alternative-id":["5906"],"URL":"https:\/\/doi.org\/10.1007\/s00467-023-05906-0","relation":{},"ISSN":["0931-041X","1432-198X"],"issn-type":[{"value":"0931-041X","type":"print"},{"value":"1432-198X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,2,25]]},"assertion":[{"value":"28 November 2022","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"6 February 2023","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"6 February 2023","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"25 February 2023","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The views expressed are those of the authors and not necessarily of the NHS, the individual hospital trusts or the Department of Health.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Disclaimer"}},{"value":"Patient and family consented for publication of their case and photographs. Formal ethical approval was not required.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval"}},{"value":"The authors declare no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}]}}