Sequential analysis of variable markers for predicting outcomes in pediatric patients with acute liver failure

Uchida, Hajime; Sakamoto, Seisuke; Fukuda, Akira; Sasaki, Kengo; Shigeta, Takanobu; Nosaka, Shunsuke; Kubota, Masaya; Nakazawa, Atsuko; Nakagawa, Satoshi; Kasahara, Mureo

doi:10.1111/hepr.12859

Cited by 11 publications

(8 citation statements)

References 32 publications

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“…Diagnosis of MTDPS was confirmed by quantitative polymerase chain reaction and a relative copy number of mitochondrial DNA (mtDNA) to nuclear DNA of <35% was defined as mtDNA depletion 8,12 . The management of ALF and indications for LT are as we have reported previously, and a mitochondrial cocktail was used in some cases 13,14 . The mitochondrial cocktail contains the following: vitamin A, vitamin B1, 2, 3, 6, 7, 12, vitamin C, vitamin D, vitamin E, vitamin K, fursultiamine, levocarnitine, and ubiquinone carnitine.…”

Section: Methodsmentioning

confidence: 99%

Outcomes of liver transplantation for mitochondrial respiratory chain disorder in children

Uchida

Sakamoto

Shimizu

et al. 2021

Pediatric Transplantation

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Aim Mitochondrial respiratory chain disorder (MRCD) can cause acute liver failure (ALF), which may necessitate liver transplantation (LT). However, MRCD is often difficult to diagnose before LT and the indications of LT are controversial due to the likelihood of progressive neurological disease. The present study further characterized the patient population and described the outcomes. Methods Thirteen patients who underwent LT for MRCD from November 2005 to May 2020 were enrolled in this study. Results Six of 13 MRCD patients were diagnosed with a mitochondrial inner membrane protein 17‐related mitochondrial DNA depletion syndrome (MTDPS). Overall, nine survived with a median follow‐up of 1.8 years (IQR, 1.3–5.1 years); four died within 2 years. In the long‐term, seven survivors showed no progression of hypotonia after LT and attended a normal kindergarten or primary school. Neurological abnormalities were observed in two survivors, including vison loss related to Leber's hereditary optic neuropathy in one patient and psychomotor retardation related to Leigh syndrome in the other. Three non‐survivors after LT were diagnosed with MTDPS and died of severe pulmonary hypertension, which had developed at 8, 9, and 18 months after LT (n=1 each). The remaining patient died of postoperative respiratory infection with respiratory syncytial virus. Conclusion The long‐term results support the performance of LT in patients with MRCD, although a genetic diagnosis is preferable for determining the accurate indications for LT in these patients. Furthermore, care should be taken to avoid complications due to mitochondrial dysfunction during the long‐term follow‐up.

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Section: Methodsmentioning

confidence: 99%

Outcomes of liver transplantation for mitochondrial respiratory chain disorder in children

Uchida

Sakamoto

Shimizu

et al. 2021

Pediatric Transplantation

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“…Moreover, the Chinese Medical Association (CMA) reported the diagnostic and treatment guidelines for ACLF in 2012 . In Japan, however, the definition of ACLF has been unclear, and diagnostic criteria have not yet been established, despite the establishment of criteria for acute liver failure (ALF) in 2011 and the evaluation of the clinical features and outcomes of patients with ALF . Thus, the Intractable Hepato‐Biliary Disease Study Group of Japan, sponsored by the Japanese Ministry of Health, Labour and Wealth, undertook a multicenter pilot survey examining patients with chronic liver diseases manifesting acute decompensation to establish the Japanese diagnostic criteria for ACLF.…”

Section: Introductionmentioning

confidence: 99%

A multicenter pilot survey to clarify the clinical features of patients with acute‐on‐chronic liver failure in Japan

Nakayama

Uemura

Uchida

et al. 2018

Hepatology Research

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“…Consequently, 19 patients (28.8%) received DDLT and 38 (57.6%) underwent LDLT without the chance to have a graft from a deceased donor. Eight patients with acute liver failure on the DDLT waiting list did finally not require an LT because their condition improved with conventional apheresis treatment (withdrawal from waiting list) . One patient with graft failure after LDLT expired without having the opportunity to receive an LDLT.…”

Section: Resultsmentioning

confidence: 99%

Waiting list mortality for pediatric deceased donor liver transplantation in a Japanese living‐donor–dominant program

Kasahara

Katono

Schlegel

et al. 2019

Pediatric Transplantation

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Living donor liver transplantation (LDLT) has become a major life‐saving procedure for children with end‐stage liver disease in Japan, whereas deceased donor liver transplantation (DDLT) has achieved only limited success. The annual number of pediatric liver transplantations is approximately 100‐120, with a patient 20‐year survival rate of 81.0%. In 2005, the liver transplantation program at the National Center for Child Health and Development in Tokyo, Japan, was initiated, with an overall number of 560 pediatric patients with end‐stage liver disease to date. In July 2010, our center was qualified as a pediatric DDLT center; a total of 132 patients were listed for DDLT up until February 2019. The indications for DDLT included acute liver failure (n = 46, 34.8%), metabolic liver disease (n = 26, 19.7%), graft failure after LDLT (n = 17, 12.9%), biliary atresia (n = 16, 12.1%), and primary sclerosing cholangitis (n = 10, 7.6%). Overall, 25.8% of the patients on the waiting list received a DDLT and 52.3% were transplanted from a living donor. The 5‐year patient and graft survivals were 90.5% and 88.8%, respectively, with an overall waiting list mortality of 3.0%. LDLT provides a better survival compared with DDLT among the recipients on the DDLT waiting list. LDLT is nevertheless of great importance in Japan; however, it cannot save all pediatric recipients. As the mortality of children on the waiting list has not yet been reduced to zero, both LDLT and DDLT should be implemented in pediatric liver transplantation programs.

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Sequential analysis of variable markers for predicting outcomes in pediatric patients with acute liver failure

Abstract: We showed that a serial analysis of the JIHBDSG score might be useful for predicting outcomes of ALF in pediatric patients who fulfilled the criteria of LT indication in our center. However, further studies are needed to validate our results.

Cited by 11 publications

References 32 publications

Outcomes of liver transplantation for mitochondrial respiratory chain disorder in children

Outcomes of liver transplantation for mitochondrial respiratory chain disorder in children

A multicenter pilot survey to clarify the clinical features of patients with acute‐on‐chronic liver failure in Japan

Waiting list mortality for pediatric deceased donor liver transplantation in a Japanese living‐donor–dominant program

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