Unlocking hope: domino liver transplantation for maple syrup syndrome, a single center experience work carried out at the King Fahad Specialist Hospital
BackgroundMaple Syrup Urine Disease (MSUD) is an autosomal recessive disorder caused by mutations in the BCKDH complex, leading to the accumulation of branched-chain amino acids. Severe cases of MSUD often require liver transplantation (LT) to restore metabolic stability and prevent neurological com...
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Frontiers Media S.A.
2025-05-01
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| Series: | Frontiers in Immunology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fimmu.2025.1579945/full |
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| author | Mohammed Al Qahtani Rawan Aloufi Ethar Gaw Hammam Momani Bodhisatwa Sengupta Iftikhar Khan Ibrahim Hassan Razan Bader Rehab Abdullah Mansour Tawfeeq Ahmed Zidan Ahmed Zidan |
| author_facet | Mohammed Al Qahtani Rawan Aloufi Ethar Gaw Hammam Momani Bodhisatwa Sengupta Iftikhar Khan Ibrahim Hassan Razan Bader Rehab Abdullah Mansour Tawfeeq Ahmed Zidan Ahmed Zidan |
| author_sort | Mohammed Al Qahtani |
| collection | DOAJ |
| description | BackgroundMaple Syrup Urine Disease (MSUD) is an autosomal recessive disorder caused by mutations in the BCKDH complex, leading to the accumulation of branched-chain amino acids. Severe cases of MSUD often require liver transplantation (LT) to restore metabolic stability and prevent neurological complications. Domino liver transplantation (DLT) using MSUD livers has emerged as an innovative approach to expand the donor pool, leveraging the fact that MSUD-affected livers can function normally in recipients without developing MSUD due to extrahepatic BCKDH activity.Methods &ResultsThis study retrospectively reviews the experience at King Fahad Specialist Hospital in Dammam, where seven patients with MSUD underwent LT, with their explanted livers subsequently transplanted into seven other recipients. The results demonstrate the feasibility and safety of this approach, with a 100% survival rate for MSUD patients at a median follow-up of 2.9 years. For the domino recipients, the 3-year graft and patient survival rate was 71.4%, with two graft-related fatalities.ConclusionThe study highlights the importance of careful recipient selection, optimal graft-to-recipient weight ratio, and the potential for hybrid dual graft transplantation in cases where graft volume is insufficient. The findings suggest that DLT using MSUD livers is a viable option, particularly in regions with limited deceased donor activity, and should be considered in mature liver transplant programs to address organ shortages. |
| format | Article |
| id | doaj-art-44c57a0a5511449eb04469ffb92d8ae9 |
| institution | OA Journals |
| issn | 1664-3224 |
| language | English |
| publishDate | 2025-05-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Immunology |
| spelling | doaj-art-44c57a0a5511449eb04469ffb92d8ae92025-08-20T01:55:41ZengFrontiers Media S.A.Frontiers in Immunology1664-32242025-05-011610.3389/fimmu.2025.15799451579945Unlocking hope: domino liver transplantation for maple syrup syndrome, a single center experience work carried out at the King Fahad Specialist HospitalMohammed Al Qahtani0Rawan Aloufi1Ethar Gaw2Hammam Momani3Bodhisatwa Sengupta4Iftikhar Khan5Ibrahim Hassan6Razan Bader7Rehab Abdullah8Mansour Tawfeeq9Ahmed Zidan10Ahmed Zidan11Organ Transplant Center of Excellence, King Fahad Specialist Hospital, Dammam, Saudi ArabiaOrgan Transplant Center of Excellence, King Fahad Specialist Hospital, Dammam, Saudi ArabiaOrgan Transplant Center of Excellence, King Fahad Specialist Hospital, Dammam, Saudi ArabiaOrgan Transplant Center of Excellence, King Fahad Specialist Hospital, Dammam, Saudi ArabiaOrgan Transplant Center of Excellence, King Fahad Specialist Hospital, Dammam, Saudi ArabiaOrgan Transplant Center of Excellence, King Fahad Specialist Hospital, Dammam, Saudi ArabiaOrgan Transplant Center of Excellence, King Fahad Specialist Hospital, Dammam, Saudi ArabiaOrgan Transplant Center of Excellence, King Fahad Specialist Hospital, Dammam, Saudi ArabiaOrgan Transplant Center of Excellence, King Fahad Specialist Hospital, Dammam, Saudi ArabiaOrgan Transplant Center of Excellence, King Fahad Specialist Hospital, Dammam, Saudi ArabiaOrgan Transplant Center of Excellence, King Fahad Specialist Hospital, Dammam, Saudi ArabiaSurgery Department, Assiut University, Assiut, EgyptBackgroundMaple Syrup Urine Disease (MSUD) is an autosomal recessive disorder caused by mutations in the BCKDH complex, leading to the accumulation of branched-chain amino acids. Severe cases of MSUD often require liver transplantation (LT) to restore metabolic stability and prevent neurological complications. Domino liver transplantation (DLT) using MSUD livers has emerged as an innovative approach to expand the donor pool, leveraging the fact that MSUD-affected livers can function normally in recipients without developing MSUD due to extrahepatic BCKDH activity.Methods &ResultsThis study retrospectively reviews the experience at King Fahad Specialist Hospital in Dammam, where seven patients with MSUD underwent LT, with their explanted livers subsequently transplanted into seven other recipients. The results demonstrate the feasibility and safety of this approach, with a 100% survival rate for MSUD patients at a median follow-up of 2.9 years. For the domino recipients, the 3-year graft and patient survival rate was 71.4%, with two graft-related fatalities.ConclusionThe study highlights the importance of careful recipient selection, optimal graft-to-recipient weight ratio, and the potential for hybrid dual graft transplantation in cases where graft volume is insufficient. The findings suggest that DLT using MSUD livers is a viable option, particularly in regions with limited deceased donor activity, and should be considered in mature liver transplant programs to address organ shortages.https://www.frontiersin.org/articles/10.3389/fimmu.2025.1579945/fulldomino liver transplantpediatric liver transplantliver transplantdomino living donor liver transplantliving donor liver transplant (LDLT) |
| spellingShingle | Mohammed Al Qahtani Rawan Aloufi Ethar Gaw Hammam Momani Bodhisatwa Sengupta Iftikhar Khan Ibrahim Hassan Razan Bader Rehab Abdullah Mansour Tawfeeq Ahmed Zidan Ahmed Zidan Unlocking hope: domino liver transplantation for maple syrup syndrome, a single center experience work carried out at the King Fahad Specialist Hospital Frontiers in Immunology domino liver transplant pediatric liver transplant liver transplant domino living donor liver transplant living donor liver transplant (LDLT) |
| title | Unlocking hope: domino liver transplantation for maple syrup syndrome, a single center experience work carried out at the King Fahad Specialist Hospital |
| title_full | Unlocking hope: domino liver transplantation for maple syrup syndrome, a single center experience work carried out at the King Fahad Specialist Hospital |
| title_fullStr | Unlocking hope: domino liver transplantation for maple syrup syndrome, a single center experience work carried out at the King Fahad Specialist Hospital |
| title_full_unstemmed | Unlocking hope: domino liver transplantation for maple syrup syndrome, a single center experience work carried out at the King Fahad Specialist Hospital |
| title_short | Unlocking hope: domino liver transplantation for maple syrup syndrome, a single center experience work carried out at the King Fahad Specialist Hospital |
| title_sort | unlocking hope domino liver transplantation for maple syrup syndrome a single center experience work carried out at the king fahad specialist hospital |
| topic | domino liver transplant pediatric liver transplant liver transplant domino living donor liver transplant living donor liver transplant (LDLT) |
| url | https://www.frontiersin.org/articles/10.3389/fimmu.2025.1579945/full |
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