Liver transplantation in Wilson disease: a single-center experience
Abstract Background Wilson disease is a complex genetic disorder due to copper accumulation, mainly in the liver and brain. It is associated with severe liver disease, which is effectively cured by liver transplantation (LT). This study aimed to analyze the outcome of Wilson disease after LT from a...
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BMC
2025-06-01
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| Series: | Orphanet Journal of Rare Diseases |
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| Online Access: | https://doi.org/10.1186/s13023-025-03827-9 |
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| author | Zahra Beyzaei Kiana Ghatei Alireza Shamsaeefar Kurosh Kazemi Saman Nikeghbalian Ali Bahador Masoud Dehghani Seyed-Ali Malekhosseini Bita Geramizadeh |
| author_facet | Zahra Beyzaei Kiana Ghatei Alireza Shamsaeefar Kurosh Kazemi Saman Nikeghbalian Ali Bahador Masoud Dehghani Seyed-Ali Malekhosseini Bita Geramizadeh |
| author_sort | Zahra Beyzaei |
| collection | DOAJ |
| description | Abstract Background Wilson disease is a complex genetic disorder due to copper accumulation, mainly in the liver and brain. It is associated with severe liver disease, which is effectively cured by liver transplantation (LT). This study aimed to analyze the outcome of Wilson disease after LT from a single center in Iran. Methods In this study, we analyzed data from Wilson patients and donors who received LT from March 2018 to December 2022 at Shiraz University of Medical Sciences, Shiraz. Long-term follow-up and post-LT outcomes for both deceased donor LT (DDLT) and living donor LT (LDLT) were measured. The Kaplan-Meier survival analysis was used to test the survival. Results 106 recipients with LT (LDLT, n = 22; DDLT, n = 84) were included (mean age of adult and pediatric: 33.1 and 10.8 years respectively; male: 58% and female 42%). The average serum ceruloplasmin and urinary copper levels improved in most patients, with values of 15.6 mg/dL and 32.3 µg per 24 h, respectively. For pediatric patients with Wilson’s disease, the survival rates at 6 months, 1 year, and 3 years were 97.0%, 96%, and 94.5%, and in adult patients achieved survival rates of 100%, 100%, and 75% at 6 months, 1 year, and 3 years, respectively. Conclusions LT is considered as a principal therapeutic option with good long-term results in Wilson patients, even in those presenting with hepatic failure. Neurologic manifestations have been improved post-LT; however, de novo neuropsychiatric symptoms begin in some cases after successful liver transplantation. |
| format | Article |
| id | doaj-art-25839a71a6df4bbb8870e93d45a7e87d |
| institution | DOAJ |
| issn | 1750-1172 |
| language | English |
| publishDate | 2025-06-01 |
| publisher | BMC |
| record_format | Article |
| series | Orphanet Journal of Rare Diseases |
| spelling | doaj-art-25839a71a6df4bbb8870e93d45a7e87d2025-08-20T02:39:48ZengBMCOrphanet Journal of Rare Diseases1750-11722025-06-012011910.1186/s13023-025-03827-9Liver transplantation in Wilson disease: a single-center experienceZahra Beyzaei0Kiana Ghatei1Alireza Shamsaeefar2Kurosh Kazemi3Saman Nikeghbalian4Ali Bahador5Masoud Dehghani6Seyed-Ali Malekhosseini7Bita Geramizadeh8Transplant Research Center, Shiraz University of Medical SciencesTransplant Research Center, Shiraz University of Medical SciencesDepartment of Hepatobiliary Surgery, Abu-Ali-Sina Hospital, Shiraz University of Medical SciencesDepartment of Hepatobiliary Surgery, Abu-Ali-Sina Hospital, Shiraz University of Medical SciencesDepartment of Hepatobiliary Surgery, Abu-Ali-Sina Hospital, Shiraz University of Medical SciencesDepartment of Hepatobiliary Surgery, Abu-Ali-Sina Hospital, Shiraz University of Medical SciencesDepartment of Hepatobiliary Surgery, Abu-Ali-Sina Hospital, Shiraz University of Medical SciencesDepartment of Hepatobiliary Surgery, Abu-Ali-Sina Hospital, Shiraz University of Medical SciencesTransplant Research Center, Shiraz University of Medical SciencesAbstract Background Wilson disease is a complex genetic disorder due to copper accumulation, mainly in the liver and brain. It is associated with severe liver disease, which is effectively cured by liver transplantation (LT). This study aimed to analyze the outcome of Wilson disease after LT from a single center in Iran. Methods In this study, we analyzed data from Wilson patients and donors who received LT from March 2018 to December 2022 at Shiraz University of Medical Sciences, Shiraz. Long-term follow-up and post-LT outcomes for both deceased donor LT (DDLT) and living donor LT (LDLT) were measured. The Kaplan-Meier survival analysis was used to test the survival. Results 106 recipients with LT (LDLT, n = 22; DDLT, n = 84) were included (mean age of adult and pediatric: 33.1 and 10.8 years respectively; male: 58% and female 42%). The average serum ceruloplasmin and urinary copper levels improved in most patients, with values of 15.6 mg/dL and 32.3 µg per 24 h, respectively. For pediatric patients with Wilson’s disease, the survival rates at 6 months, 1 year, and 3 years were 97.0%, 96%, and 94.5%, and in adult patients achieved survival rates of 100%, 100%, and 75% at 6 months, 1 year, and 3 years, respectively. Conclusions LT is considered as a principal therapeutic option with good long-term results in Wilson patients, even in those presenting with hepatic failure. Neurologic manifestations have been improved post-LT; however, de novo neuropsychiatric symptoms begin in some cases after successful liver transplantation.https://doi.org/10.1186/s13023-025-03827-9Wilson diseaseLiver transplantationCopperTransplant outcomesLiving donorsDeceased donors |
| spellingShingle | Zahra Beyzaei Kiana Ghatei Alireza Shamsaeefar Kurosh Kazemi Saman Nikeghbalian Ali Bahador Masoud Dehghani Seyed-Ali Malekhosseini Bita Geramizadeh Liver transplantation in Wilson disease: a single-center experience Orphanet Journal of Rare Diseases Wilson disease Liver transplantation Copper Transplant outcomes Living donors Deceased donors |
| title | Liver transplantation in Wilson disease: a single-center experience |
| title_full | Liver transplantation in Wilson disease: a single-center experience |
| title_fullStr | Liver transplantation in Wilson disease: a single-center experience |
| title_full_unstemmed | Liver transplantation in Wilson disease: a single-center experience |
| title_short | Liver transplantation in Wilson disease: a single-center experience |
| title_sort | liver transplantation in wilson disease a single center experience |
| topic | Wilson disease Liver transplantation Copper Transplant outcomes Living donors Deceased donors |
| url | https://doi.org/10.1186/s13023-025-03827-9 |
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