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Successful living donor liver transplantation plus domino-auxiliary partial orthotopic liver transplantation for pediatric patients with metabolic disorders

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Abstract

Purpose

To investigate the efficacy of living donor liver transplantation (LDLT) plus domino-auxiliary partial orthotopic liver transplantation (D-APOLT) in pediatric patients with metabolic disorders.

Methods

From May 2017 to October 2018, two patients with ornithine aminotransferase deficiency (OTCD) and one patient with type I Crigler-Najjar syndrome (CNS1) received LDLT, their livers were prepared as donors for D-APOLT. Two patients with CNS1 received domino liver grafts from OTCD patients; one OTCD patient received a domino liver graft from a CNS1 patient.

Results

The mean follow-up was 26.6 months. The liver function and ammonia remained in the normal range at the end of the follow-up in all recipients. One D-APOLT patient experienced portal vein thrombosis 2 days after transplantation and required reoperation, this patient presented an imbalance of portal blood perfusion between the native and the domino liver at 8 months after liver transplant. The imbalance was improved by interventional radiology treatment. Two LDLT patients experienced early mild acute rejection.

Conclusions

The non-cirrhotic livers from pediatric patients with metabolic liver disease can be used as domino donor grafts for selected pediatric patients with different metabolic liver disease. D-APOLT achieves ideal recipient outcomes and provides a strategy to expand donor source for children.

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Abbreviations

ALT:

Alanine transaminase

AST:

Aspartate transaminase

APOLT:

Auxiliary partial orthotopic liver transplantation

CNS1:

Crigler-Najjar syndrome

CT:

Computed tomography

D-APOLT:

Domino-auxiliary partial orthotopic liver transplantation

HV:

Hepatic vein

LDLT:

Living donor liver transplantation

LLS:

Left lateral segment

LT:

Liver transplantation

MHV:

Middle hepatic vein

MLD:

Metabolic liver diseases

MRCP:

Magnetic resonance cholangiopancreatography

NCMLD:

Non-cirrhotic metabolic liver diseases

OTCD:

Ornithine aminotransferase deficiency

PLT:

Partial liver transplantation

PV:

Portal vein

RL:

Right lobe

TB:

Total bilirubin

References

  1. Reddy MS, Rajalingam R, Rela M (2017) Revisiting APOLT for metabolic liver disease: a new look at an old idea. Transplantation 101(2):260–266. https://doi.org/10.1097/tp.0000000000001472

    Article  Google Scholar 

  2. Fagiuoli S, Daina E, D'Antiga L, Colledan M, Remuzzi G (2013) Monogenic diseases that can be cured by liver transplantation. J Hepatol 59(3):595–612. https://doi.org/10.1016/j.jhep.2013.04.004

    Article  Google Scholar 

  3. Dong C, Gao W, Ma N, Sun C, Zheng WP, Wang K, Shen ZY (2017) Risks and treatment strategies for de novo hepatitis B virus infection from anti-HBc-positive donors in pediatric living donor liver transplantation. Pediatr Transplant. https://doi.org/10.1111/petr.12854

    Article  Google Scholar 

  4. Song Z, Ma N, Dong C, Sun C, Meng X, Zhang W, Wang K, Wu B, Li S, Qin H, Han C, Li H, Gao W, Shen Z (2019) Feasibility and safety of using low-body-weight donors in pediatric liver transplantation. J Pediatr Surg 54(11):2382–2386. https://doi.org/10.1016/j.jpedsurg.2019.04.023

    Article  Google Scholar 

  5. Shanmugam NP, Perumalla R, Gopinath R, Olithselvan A, Varghese J, Kapoor D, Rela M (2011) Auxiliary liver transplantation: a form of gene therapy in selective metabolic disorders. J Clin Exp Hepatol 1(2):118–120. https://doi.org/10.1016/s0973-6883(11)60132-1

    Article  Google Scholar 

  6. Read WL (2007) Reciprocal auxiliary partial liver transplant for inborn noncirrhotic metabolic diseases. Transplantation 83(10):1405–1406. https://doi.org/10.1097/01.tp.0000261730.50106.8d

    Article  Google Scholar 

  7. Rela M, Muiesan P, Vilca-Melendez H, Dhawan A, Baker A, Mieli-Vergani G, Heaton ND (1999) Auxiliary partial orthotopic liver transplantation for Crigler-Najjar syndrome type I. Ann Surg 229(4):565–569. https://doi.org/10.1097/00000658-199904000-00017

    Article  CAS  Google Scholar 

  8. Cunningham SC, Siew SM, Hallwirth CV, Bolitho C, Sasaki N, Garg G, Michael IP, Hetherington NA, Carpenter K, de Alencastro G, Nagy A, Alexander IE (2015) Modeling correction of severe urea cycle defects in the growing murine liver using a hybrid recombinant adeno-associated virus/piggyBac transposase gene delivery system. Hepatology 62(2):417–428. https://doi.org/10.1002/hep.27842

    Article  CAS  Google Scholar 

  9. Morioka D, Kasahara M, Takada Y, Shirouzu Y, Taira K, Sakamoto S, Uryuhara K, Egawa H, Shimada H, Tanaka K (2005) Current role of liver transplantation for the treatment of urea cycle disorders: a review of the worldwide English literature and 13 cases at Kyoto University. Liver Transpl 11(11):1332–1342. https://doi.org/10.1002/lt.20587

    Article  Google Scholar 

  10. Gao W, Song Z, Ma N, Dong C, Meng X, Sun C, Qin H, Han C, Yang Y, Zhang F, Zheng W, Shen Z (2020) Application of pediatric donors in split liver transplantation: Is there an age limit? Am J Transplant 20(3):817–824. https://doi.org/10.1111/ajt.15641

    Article  Google Scholar 

  11. Yamaoka Y, Ozawa K, Tanaka A, Mori K, Morimoto T, Shimahara Y, Zaima M, Tanaka K, Kumada K (1991) New devices for harvesting a hepatic graft from a living donor. Transplantation 52(1):157–160. https://doi.org/10.1097/00007890-199107000-00035

    Article  CAS  Google Scholar 

  12. Moini M, Mistry P, Schilsky ML (2010) Liver transplantation for inherited metabolic disorders of the liver. Curr Opin Organ Transplant 15(3):269–276. https://doi.org/10.1097/MOT.0b013e3283399dbd

    Article  Google Scholar 

  13. Roda KMO, Vincenzi R, Fonseca EA, Benavides M, Turine P, Afonso RC, Tonon T, Schwartz I, Miura IK, Pugliese R, Porta G, Chapchap P, Seda Neto J (2019) Domino liver transplant in maple syrup urine disease: technical details of cases in which the first surgery involved a living donor. Transplantation 103(3):536–543. https://doi.org/10.1097/tp.0000000000002300

    Article  Google Scholar 

  14. Ghabril M, Nguyen J, Kramer D, Genco T, Mai M, Rosser BG (2007) Presentation of an acquired urea cycle disorder post liver transplantation. Liver Transpl 13(12):1714–1716. https://doi.org/10.1002/lt.21291

    Article  Google Scholar 

  15. Govil S, Shanmugam NP, Reddy MS, Narasimhan G, Rela M (2015) A metabolic chimera: two defective genotypes make a normal phenotype. Liver Transpl 21(11):1453–1454. https://doi.org/10.1002/lt.24202

    Article  Google Scholar 

  16. Rela M, Bharathan A, Palaniappan K, Cherian PT, Reddy MS (2015) Portal flow modulation in auxiliary partial orthotopic liver transplantation. Pediatr Transplant 19(3):255–260. https://doi.org/10.1111/petr.12436

    Article  Google Scholar 

  17. Whitington PF, Emond JC, Heffron T, Thistlethwaite JR (1993) Orthotopic auxiliary liver transplantation for Crigler-Najjar syndrome type 1. Lancet 342(8874):779–780. https://doi.org/10.1016/0140-6736(93)91543-u

    Article  CAS  Google Scholar 

  18. Broering DC, Walter J, Bassas AF (2005) Overcoming the portal steal phenomenon in auxiliary partial orthotopic liver transplantation by modulation of the venous outflow of the native liver. Liver Transpl 11(9):1140–1143. https://doi.org/10.1002/lt.20535

    Article  Google Scholar 

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Funding

This study was funded by Tianjin Clinical Research Center for Organ Transplantation Project (15ZXLCSY00070).

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Correspondence to Wei Gao.

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All the authors of this manuscript have no conflicts of interest to disclose.

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All the procedures in studies involving human participants were in accordance with the institutional review board of Tianjin First Center Hospital (approval number: 2018N148KY) and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Informed consent was obtained from all individual participants included in the study.

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Dong, C., Song, Z., Meng, X. et al. Successful living donor liver transplantation plus domino-auxiliary partial orthotopic liver transplantation for pediatric patients with metabolic disorders. Pediatr Surg Int 36, 1443–1450 (2020). https://doi.org/10.1007/s00383-020-04756-3

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