Marginal parental donors for pediatric living donor liver transplantation

Kasahara, Mureo; Sakamoto, Seisuke; Fukuda, Akira; Uchida, Hajime; Yi, Nam‐Joon; Schlegel, Andrea; Muiesan, Paolo; Xia, Qiang; Gao, Wei; Zhu, Zhi‐Jun; Rodríguez-Dávalos, Manuel I.; Rela, Mohamed

doi:10.1097/mot.0000000000000990

Cited by 9 publications

(1 citation statement)

References 40 publications

(46 reference statements)

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“…This poses the potential risk of the donor graft having inadequate metabolic function. Several diseases should not use heterozygous related living donors; heterozygous hypercholesterolemia (autosomal dominant; risk of hypercholesterolemia), heterozygous acute intermittent porphyria (autosomal recessive; risk of decrease in porphobilinogen deaminase activity), and asymptomatic parental donors with paucity of intrahepatic bile duct in AGS (autosomal recessive; risk of cholestasis and cholangitis) 95 …”

Section: Surgical Aspects Of Clktmentioning

confidence: 99%

Combined liver‐kidney transplantation in pediatric patients

Yi,

Kim,

Hong

et al. 2023

Pediatric Transplantation

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Combined liver‐kidney transplantation (CLKT) is a surgical procedure that involves transplanting both liver and kidney organs. There are two types of CLKT: simultaneous liver‐kidney transplantation (smLKT) and sequential LKT (sqLKT). CLKT accounts for a small percentage of liver transplantations (LTs), particularly in pediatric cases. Nevertheless, the procedure has demonstrated excellent outcomes, with high survival rates and lower rejection rates. The main indications for CLKT in pediatric patients differ somewhat from that in adults, in which end‐stage kidney disease after LT is the major indication. In children, congenital diseases are common reason for performing CLKT; the examples of such diseases include autosomal recessive polycystic kidney disease with congenital hepatic fibrosis which equally affects both organs, and primary hyperoxaluria type 1, a primary liver disease leading kidney failure. The decision between smLKT or sqLKT depends on the dominant organ failure, the specific pathophysiology, and available organ sources. However, there remain significant surgical and societal challenges surrounding CLKT. Innovations in pharmacology and genetic engineering have decreased the necessity for CLKT in early‐diagnosed cases without portal hypertension or kidney replacement therapy. Nonetheless, these advancements are not universally accessible. Therefore, decision‐making algorithms should be crafted, considering region‐specific organ allocation systems and prevailing medical environments.

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Section: Surgical Aspects Of Clktmentioning

confidence: 99%

Combined liver‐kidney transplantation in pediatric patients

Yi,

Kim,

Hong

et al. 2023

Pediatric Transplantation

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Improved survival of pediatric deceased donor liver transplantation recipients after introduction of the pediatric prioritization system: Analysis of data from a Japanese national survey

Takemura,

Shinoda,

Kasahara

et al. 2024

J Hepato Biliary Pancreat

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BackgroundIn Japan, there has never been a national analysis of pediatric deceased donor liver transplantation (pDDLT) based on donor and recipient factors. We constructed a Japanese nationwide database and assessed outcomes of pDDLT focusing on the pediatric prioritization system introduced in 2018.MethodsWe collected data on pDDLTs (<18 years) performed between 1999 and 2021 from the Japan Organ Transplant Network and Japanese Liver Transplantation Society, identified risk factors for graft survival and compared the characteristics and graft survival in pDDLTs conducted before and after the introduction of the pediatric prioritization system.ResultsOverall, 112 cases of pDDLT were included, with a 1‐year graft survival rate of 86.6%. Four poor prognostic factors were identified: recipient intensive care unit stay, model for end‐stage liver disease/pediatric end‐stage liver disease score, donor cause of death, and donor total bilirubin. After the introduction of the system, allografts from pediatric donors were more reliably allocated to pediatric recipients and the annual number of pDDLTs increased. The 1‐year graft survival rate improved significantly as did pDDLT conditions indicated by the risk factors.ConclusionsUnder the revised allocation system, opportunities for pDDLT increased, resulting in favorable recipient and donor conditions and improved survival.

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Outcomes of adult native liver survivors with biliary atresia: the current situation in Japan

Kawaguchi,

Terui,

Takenouchi

et al. 2023

Pediatr Surg Int

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Purpose: This study aimed to elucidate the di culties faced by adult native liver survivors with biliary atresia (BA) in Japan.Methods: A single-center, retrospective, observational study of 57 adult patients with BA was conducted.The clinical course of BA was compared between native liver survivors and non-survivors who reached adulthood. Indications and outcomes of liver transplantation (LT) among non-survivors were assessed.Results: A signi cantly larger portion of non-survivors (n=10) met the criteria for LT (p <0.001) and received treatment for portal hypertension after reaching 20 years of age (p <0.01) compared with the survivors. Causes of death included liver cirrhosis (n=8), graft failure of living donor liver transplantation (LDLT) (n=1), and hepatocarcinoma (n=1). Two of the non-survivors who died of liver cirrhosis had no indication for LT because of alcohol dependence and uncontrolled infection. An appropriate donor candidate could not be found for the ve patients who opted for LDLT. All six patients waitlisted for deceased donor liver transplantation (DDLT) died after a median waiting period of 17 months.Conclusion: Adult BA patients in Japan have limited options for LT, mainly owing to low donor candidate availability for LDLT and a low prevalence of DDLT.

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Marginal parental donors for pediatric living donor liver transplantation

Cited by 9 publications

References 40 publications

Combined liver‐kidney transplantation in pediatric patients

Combined liver‐kidney transplantation in pediatric patients

Improved survival of pediatric deceased donor liver transplantation recipients after introduction of the pediatric prioritization system: Analysis of data from a Japanese national survey

Outcomes of adult native liver survivors with biliary atresia: the current situation in Japan

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