Liver Transplantation for Metabolic Liver Disease: Experience at a Living Donor Dominant Liver Transplantation Center

Kim, Jun Suk; Kim, Kyung Mo; Oh, Seak Hee; Kim, Hyun Jin; Cho, Jin Min; Yoo, Han‐Wook; Namgoong, Jung‐Man; Kim, Dae Yeon; Kim, Ki‐Hun; Hwang, Shin; Lee, Sung‐Gyu

doi:10.5223/pghn.2015.18.1.48

Cited by 17 publications

(24 citation statements)

References 24 publications

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“…Arnon et al (21) showed that survival rates of their patients with metabolic and non-metabolic diseases were 94.6% and 90.7% at one year respectively and 88.9% and 86.1% respectively at year five. The cumulative survival rates in pediatric patients with non-metabolic disease were 91.9%, 87.2%, and 85.8% at one, five, and 10 years, respectively (22). The survival rate was lower in both the metabolic and non-metabolic disease groups in our study, which was different from the study by Kayler et al (15).…”

Section: Discussioncontrasting

confidence: 92%

Pediatric Liver Transplantation Outcomes for Metabolic and Non-metabolic Diseases in Turkey: A Single Center Experience

Karakoyun¹,

Canda²,

Kizilcan³

et al. 2019

jpr

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Liver transplantation (LT) is performed for several indications in the pediatric population, including malignancy and acute hepatic failure among others. LT has become an important treatment alternative for metabolic diseases. In most pediatric transplant centers, metabolic liver disease is the second most common indication for LT after biliary atresia. Our studies aim is to compare the post-transplant outcomes of those patients with metabolic and other liver diseases in our transplant program. Materials and Methods: One hundred eighty-nine patients who underwent LT between 1997 and 2015 due to metabolic diseases and acute or chronic liver failure were included in the study. This study was performed retrospectively. Results: We enrolled 189 patients in our study. 54% (n=102) male and 46% (n=87) female patients were included in the study. The metabolic disease group included 56 patients and the non-metabolic disease group contained 133 patients. Progressive familial intrahepatic cholestasis is the most common disease among metabolic diseases resulting in LT and Wilson disease is the second most common. Post-transplant immunosuppression was similar for both groups. There was no difference in both groups regarding the onset of post-transplant complications for graft type or recipient age. Biliary and portal vein complications were most particularly defined in the group with non-metabolic diseases. There was no significant difference in survival between the two groups. Conclusion: LT is an important treatment option for acute hepatic failure and end-stage liver diseases. In addition, LT is an alternative treatment option for some metabolic diseases.

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Section: Discussioncontrasting

confidence: 92%

Pediatric Liver Transplantation Outcomes for Metabolic and Non-metabolic Diseases in Turkey: A Single Center Experience

Karakoyun¹,

Canda²,

Kizilcan³

et al. 2019

jpr

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“…In children with citrullinemia who have recovered from the initial presentation and have a subsequent period of stability, the indication and timing of LT need to be carefully considered within a multidisciplinary setting. Kim et al reported a mean age of LT in neonatal‐onset citrullinemia of 1.7 years (range, 0.8‐5.25), which is comparable to our group. Surgical and infectious complications post‐LT tend to be less frequent with increasing age of the child .…”

Section: Discussionsupporting

confidence: 89%

“…The use of heterozygote donors for some children with metabolic disorders is well documented . The overall experience is positive with adequate enzyme replacement despite using a carrier donor .…”

Section: Discussionmentioning

confidence: 99%

“…The use of heterozygote donors for some children with meta bolic disorders is well documented. 17 The overall experience is positive with adequate enzyme replacement despite using a carrier donor. 18 Following the surgery, both donor and recipient appear not to have In our study, we had a unique opportunity to study in detail the histopathologic features of the explanted native livers in children with citrullinemia.…”

Section: Discussionmentioning

confidence: 99%

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Liver transplantation for neonatal‐onset citrullinemia

Vara

Dhawan

Deheragoda

et al. 2018

Pediatric Transplantation

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Citrullinemia or ASS deficiency in its classical form presents in the neonatal period with poor feeding, hyperammonemia, encephalopathy, seizures, and if untreated can be fatal. Despite advances in medical therapy, neurocognitive outcomes remain suboptimal. LT has emerged as a potential management option. A retrospective single-center review identified 7 children with a median age of 1.1 years (range, 0.6-5.8) at referral. Five children presented clinically, and 2 were treated prospectively from birth due to positive family history. All patients received standard medical and dietary therapy prior to LT. The indications for LT were frequent metabolic decompensations in 4, elective in 2, and ALF in 1. The median age at LT was 2.4 years (range, 1.3-6.5). Five patients received 6 left lateral segment grafts, one a live unrelated donor left lateral segment as an APOLT graft, and one a cadaveric whole liver graft as APOLT. One child required retransplantation due to hepatic artery thrombosis. Graft and patient survival were 86% and 100%, respectively. Median follow-up is 3.1 years (range, 0.1-4.1), and the median age at follow-up is 5.5 years (range, 4.0-9.8). There have been no metabolic decompensations in 6 children, while 1 patient (with APOLT) developed asymptomatic hyperammonemia with no clinical or histological signs of liver injury, requiring additional medical therapy. Our medium-term experience following LT in citrullinemia is favorable, demonstrating a positive transformation of the clinical phenotype.

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“…Regardless of enzymatic testing, there were no observed mortality or morbidity related to recurrence of disease with the use of heterozygotes in all studied donors and recipients. This is consistent with other small cohorts utilizing heterozygote donors for metabolic liver disease . The common findings in all utilized heterozygote donors are the lack of symptoms related to the given disease and normal ammonia and amino acid levels, despite decreased enzymatic activity.…”

Section: Heterozygote Donorssupporting

confidence: 89%

Living donor liver transplantation for inborn errors of metabolism – An underutilized resource in the United States

Pham

Enns

Esquivel

2016

Pediatric Transplantation

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Inborn metabolic diseases of the liver can be life-threatening disorders that cause debilitating and permanent neurological damage. Symptoms may manifest as early as the neonatal period. Liver transplant replaces the enzymatically deficient liver, allowing for metabolism of toxic metabolites. LDLT for metabolic disorders is rarely performed in the United States as compared to countries such as Japan, where they report >2000 cases performed within the past two decades. Patient and graft survival is comparable to that of the United States, where most of the studies are based on deceased donors. No living donor complications were observed, suggesting that LDLT is as safe and effective as deceased donor transplants performed in the USA. Increased utilization of living donors in the USA will allow for early transplantation to prevent permanent neurological damage in those with severe disease. Pediatric transplant centers should consider utilizing living donors when feasible for children with metabolic disorders of the liver.

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Liver Transplantation for Metabolic Liver Disease: Experience at a Living Donor Dominant Liver Transplantation Center

Cited by 17 publications

References 24 publications

Pediatric Liver Transplantation Outcomes for Metabolic and Non-metabolic Diseases in Turkey: A Single Center Experience

Pediatric Liver Transplantation Outcomes for Metabolic and Non-metabolic Diseases in Turkey: A Single Center Experience

Liver transplantation for neonatal‐onset citrullinemia

Living donor liver transplantation for inborn errors of metabolism – An underutilized resource in the United States

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