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Transplantation in paediatric patients with MMA requires multidisciplinary approach for achievement of good clinical outcomes
BACKGROUND: 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. METHODS: We describe the first report of a small child receiving a simultaneous liver and kidney transpla...
Autores principales: | , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer Berlin Heidelberg
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10393894/ https://www.ncbi.nlm.nih.gov/pubmed/36840752 http://dx.doi.org/10.1007/s00467-023-05906-0 |
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author | Paessler, Alicia Cortes-Cerisuelo, Miriam Jassem, Wayel Vilca-Melendez, Hector Deep, Akash Jain, Vandana Pool, Andrew Grunewald, Stephanie Kessaris, Nicos Stojanovic, Jelena |
author_facet | Paessler, Alicia Cortes-Cerisuelo, Miriam Jassem, Wayel Vilca-Melendez, Hector Deep, Akash Jain, Vandana Pool, Andrew Grunewald, Stephanie Kessaris, Nicos Stojanovic, Jelena |
author_sort | Paessler, Alicia |
collection | PubMed |
description | BACKGROUND: 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. METHODS: 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 years old, weighed 14.0 kg, had chronic kidney disease stage V, and had not yet started any other form of kidney replacement therapy. RESULTS: 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’s recovery was further complicated by sepsis, transient CNI toxicity, and de novo DSAs. CONCLUSIONS: 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 h. 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. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s00467-023-05906-0. |
format | Online Article Text |
id | pubmed-10393894 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Springer Berlin Heidelberg |
record_format | MEDLINE/PubMed |
spelling | pubmed-103938942023-08-03 Transplantation in paediatric patients with MMA requires multidisciplinary approach for achievement of good clinical outcomes Paessler, Alicia Cortes-Cerisuelo, Miriam Jassem, Wayel Vilca-Melendez, Hector Deep, Akash Jain, Vandana Pool, Andrew Grunewald, Stephanie Kessaris, Nicos Stojanovic, Jelena Pediatr Nephrol Management Dilemma BACKGROUND: 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. METHODS: 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 years old, weighed 14.0 kg, had chronic kidney disease stage V, and had not yet started any other form of kidney replacement therapy. RESULTS: 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’s recovery was further complicated by sepsis, transient CNI toxicity, and de novo DSAs. CONCLUSIONS: 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 h. 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. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s00467-023-05906-0. Springer Berlin Heidelberg 2023-02-25 2023 /pmc/articles/PMC10393894/ /pubmed/36840752 http://dx.doi.org/10.1007/s00467-023-05906-0 Text en © The Author(s) 2023 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . |
spellingShingle | Management Dilemma Paessler, Alicia Cortes-Cerisuelo, Miriam Jassem, Wayel Vilca-Melendez, Hector Deep, Akash Jain, Vandana Pool, Andrew Grunewald, Stephanie Kessaris, Nicos Stojanovic, Jelena Transplantation in paediatric patients with MMA requires multidisciplinary approach for achievement of good clinical outcomes |
title | Transplantation in paediatric patients with MMA requires multidisciplinary approach for achievement of good clinical outcomes |
title_full | Transplantation in paediatric patients with MMA requires multidisciplinary approach for achievement of good clinical outcomes |
title_fullStr | Transplantation in paediatric patients with MMA requires multidisciplinary approach for achievement of good clinical outcomes |
title_full_unstemmed | Transplantation in paediatric patients with MMA requires multidisciplinary approach for achievement of good clinical outcomes |
title_short | Transplantation in paediatric patients with MMA requires multidisciplinary approach for achievement of good clinical outcomes |
title_sort | transplantation in paediatric patients with mma requires multidisciplinary approach for achievement of good clinical outcomes |
topic | Management Dilemma |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10393894/ https://www.ncbi.nlm.nih.gov/pubmed/36840752 http://dx.doi.org/10.1007/s00467-023-05906-0 |
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