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Transplantation Reports
journal homepage:www.elsevier.com/locate/tprRight lobe liver transplantation for Wilson's disease from heterozygote
donor: Case report
Ümit Özçelik
a,⁎, Eryiğit Eren
b, Mehmet Tokaç
a, Ayhan Dinçkan
baDepartment of General Surgery, İstanbul Aydın University Training and Research Hospital, Beşyol mahallesi, Akasya sokak no: 4, Küçükçekmece, 34295, İstanbul, Turkey bDepartment of General Surgery, İstinye University Training and Research Hospital, Aşık Veysel Mah, Süleyman Demirel Cd. No:1, Esenyurt, 34517, İstanbul, Turkey
A B S T R A C T
Background: Use of liver grafts from heterozygote genetic carriers for Wilson's disease was considered safe. We present a 14 year-old male patient with Wilson's disease who was undergone right lobe liver transplantation from his heterozygote mother.
Case report: The patient was admitted to our hospital with decompensated liver cirrhosis due to Wilson's disease as a liver recipient candidate. His Child score was 11 (Child C) and MELD score was 28. His weight was 52 kg. Total liver volume of his 40-year-old mother was calculated 1488 mL in computed tomography. Right and left liver lobe of the donor were calculated as 1044/444 (70.16%/29.84%) mL. Preoperative ceruloplasmin level, 24-h urinary copper excretion, serum copper level and liver biopsy of the donor were all normal. Right lobe liver transplant was performed. Recipient and donor were discharged from the hospital uneventfully. The patient's preoperative ceruloplasmin level was 15.3 mg/dL and increased to 22 mg/dL after liver transplant. Also urinary copper decreased from 1939 to 68 μg/day and serum copper improved from 72 to 82 mg/dL after liver transplant. There were no statistically difference between preoperative and postoperative serum copper, ceruloplasmin and 24 h urinary copper levels of the donor.
Discussion: Equal outcomes of living related liver transplantation for Wilson's disease were reported for heterozygote and nonheterozygote donors previously. Usually left liver lobes were used for transplantation from heterozygous donors. The use of right liver lobe of the heterozygous donor has no negative impact on both the donor and recipient for Wilson's disease.
1. Background
Wilson's disease (WD) is an autosomal recessive disease with a prevalence of 1/30000-1/100000[1]. The frequency of heterozygous carriers is 1/90 [1]. It is a metabolic disorder of copper transport leading to progressive accumulation of copper in various organs caused by mutations in the ATP7B gene encoding a copper transporting P-type ATPase[2].
Liver transplantation (LT) is the treatment of choice for fulminant WD as well as patients with end-stage liver disease (ESLD) despite medical therapy. Living related liver transplantation (LDLT) has been used with increasing frequency due to deceased organ availability especially where cadaveric donation is unusual. Use of liver grafts from heterozygote genetic carriers for Wilson's disease was considered safe [1–5]. Mostly left lobes and left lateral segment grafts used for living related liver transplantation.
We present a 14-year-old male patient with Wilson's disease who was undergone right lobe liver transplantation from his heterozygote mother for Wilson genetic defect.
2. Case report
A 14-year-old male was admitted to our hospital with the diagnosis of decompensated liver cirrhosis due to Wilson's disease (compound mutation in the ATP7B gene; c.3207C>A [p.His1069Gln] inherited from the mother and c.4022G>A [pGlu1341Asp] inherited from the father) as a liver recipient candidate from his mother. His Child score was 11 (Child C) and MELD score was 28. His weight was 52 kg.
Total liver volume of his 40-year-old mother was calculated 1488 mL in computed tomography. Right liver lobe and left liver lobe of the donor were calculated as 1044/444 (70.16%/29.84%) mL. Preoperative ceruloplasmin level, 24 h urinary copper excretion, serum copper level and liver biopsy of the donor were all normal.
Right lobe liver transplant was performed from his mother. The donor graft weighed 950 g on back-table and the operation time was 480 min. Blood loss was 600 mL. Recipient and donor were discharged from the hospital uneventfully on postoperative 14th and 7th days. The patient's preoperative ceruloplasmin level was 15.3 mg/dL and increased to 22 mg/dL after liver transplant. Also urinary copper
https://doi.org/10.1016/j.tpr.2019.100023
Received 15 December 2018; Accepted 10 April 2019
⁎Corresponding author.
E-mail address:umit.ozcelik@iauh.com.tr(Ü. Özçelik).
Transplantation Reports 4 (2019) 100023
Available online 13 April 2019
2451-9596/ © 2019 Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/BY-NC-ND/4.0/).
decreased from 1939 to 68 μg/day and serum copper improved from 72 to 82 mg/dL after liver transplant. There were no statistically difference between preoperative and postoperative serum copper, ceruloplasmin and 24 h urinary copper levels of the donor.
3. Discussion
Wilson's disease was described by Kinnear Wilson in 1912 as an autosomal recessive disorder leading an excessive accumulation of copper in tissues, especially in the liver, brain, cornea and kidneys[6]. Metabolic disorders have become the second largest indication for liver transplantation after biliary atresia and WD is the most common me-tabolic disorder[5]. Fulminant WD and ESLD due to WD are the main indications for LT. As a result of scarcity of cadaveric donors LRLT became an alternative of rescue therapy for patients who need LT. LRLT outcomes are excellent with a patient survival of 90% at 5 years[4]. Equal outcomes of living related liver transplantation for Wilson's dis-ease were reported for heterozygote and nonheterozygote donors pre-viously[1–5]. But 10% of WD heterozygotes have low ceruloplasmin levels and these individuals are not suitable as donors[2,3].
Although WD patients were relatively older children with a high body weight usually left liver lobes were used for transplantation from heterozygous donors. This can lead to the graft weight/body weight ratios less than 1% and small-for-size livers. There were only small numbers of cases reported in the literature using a right lobe graft [1,6,7].
We did not face any complications for both donor and the recipient.
The use of right liver lobe of the heterozygous donor has no negative impact on both the donor and recipient for Wilson's disease. It can be a better option for older children to prevent small for size syndrome. Acknowledgments
The authors declare that they have no sources of funding for this study, and they have no conflicts of interest to declare.
References
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[3] K. Asonuma, Y. Inomata, M. Kasahara, S. Uemoto, H. Egawa, S. Fujita, et al., Living related liver transplantation from heterozygote genetic carriers to children with Wilson's disease, Pediatr. Transplant. 3 (Aug (3)) (1999) 201–205.
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[6] X.H. Wang, F. Cheng, F. Zhang, X.C. Li, J.M. Qian, L.B. Kong, et al., Copper meta-bolism after living related liver transplantation for Wilson's disease, World J. Gastroenterol. 9 (Dec (12)) (2003) 2836–2838.
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Ü. Özçelik, et al. Transplantation Reports 4 (2019) 100023
