A case of a maintenance hemodialysis patient with autosomal dominant polycystic kidney disease who underwent living donor liver transplantation alone due to refractory liver cyst infection
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Liver cysts are observed in 83% of cases of autosomal dominant polycystic kidney disease (ADPKD). Although not as prevalent as renal cyst infection, liver cyst infection is a serious complication that is sometimes difficult to treat. We report the case of a maintenance hemodialysis patient with ADPKD who received a living donor liver transplantation alone (LDLTA) due to refractory liver cyst infection. The patient was a 67-year-old Japanese man who developed fever and right-side abdominal pain, and liver cyst infection was suspected. Treatment with multiple antibiotics was ineffective. Many liver cysts were observed on magnetic resonance imaging scans and a cyst in liver segment S6, which produced the strongest signal variation, was drained. The fever subsided temporarily, but multiple infected liver cysts were observed on follow-up imaging examination; 4 months later, hepatectomy and LDLTA were performed. Although LDLTA due to refractory liver cyst infection in maintenance hemodialysis patients with ADPKD is risky and should be carefully considered, it may be the only effective treatment.
KeywordsLiver transplantation Hemodialysis ADPKD Polycystic kidney disease Polycystic liver disease Liver cyst infection
This report was supported in part by a Grant-in-Aid for Intractable Renal Diseases Research, Research on rare and intractable diseases; and Health and Labor Sciences Research Grants from the Ministry of Health, Labor and Welfare of Japan.
Compliance with ethical standards
Conflict of interest
Toshio Mochizuki and Ken Tsuchiya received travel fees and honoraria for lectures from Otsuka Pharmaceutical Co. Toshio Mochizuki belongs to an endowed university department sponsored by Otsuka Pharmaceutical Co, Chugai Pharmaceutical Co, Kyowa Hakko Kirin Co, MSD Co, and JMS Co.
All procedures performed in the patient study were in accordance with the 1964 Helsinki Declaration and its later amendments, or with comparable ethical standards.
Informed consent was obtained from the patient.
- 1.Bae KT, Zhu F, Chapman AB, Torres VE, Grantham JJ, Guay-Woodford LM, et al. Magnetic resonance imaging evaluation of hepatic cysts in early autosomal-dominant polycystic kidney disease: the Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease cohort. Clin J Am Soc Nephrol CJASN. 2006;1(1):64–9. https://doi.org/10.2215/CJN.00080605.CrossRefPubMedGoogle Scholar
- 2.The European Polycystic Kidney Disease Consortium. The polycystic kidney disease 1 gene encodes a 14 kb transcript and lies within a duplicated region on chromosome 16. Eur Polycystic Kidney Dis Consort Cell. 1994;77(6):881–94.Google Scholar
- 9.Hoevenaren IA, Wester R, Schrier RW, McFann K, Doctor RB, Drenth JP, et al. Polycystic liver: clinical characteristics of patients with isolated polycystic liver disease compared with patients with polycystic liver and autosomal dominant polycystic kidney disease. Liver Int. 2008;28(2):264–70. https://doi.org/10.1111/j.1478-3231.2007.01595.x.CrossRefPubMedGoogle Scholar
- 22.Cimsit B, Schilsky M, Moini M, Cartiera K, Arvelakis A, Kulkarni S, et al. Combined liver kidney transplantation: critical analysis of a single-center experience. Transpl Proc. 2011;43(3):901–4. https://doi.org/10.1016/j.transproceed.2011.02.033.CrossRefGoogle Scholar
- 27.Eguchi S, Furukawa H, Uemoto S, Umeshita K, Imamura H, Soyama A, et al. Outcomes of living donor liver transplantation alone for patients on maintenance renal replacement therapy in Japan: results of a Nationwide Survey. Transpl Direct. 2016;2(6):e74. https://doi.org/10.1097/TXD.0000000000000587.CrossRefGoogle Scholar
- 29.Taner B, Willingham DL, Hewitt WR, Grewal HP, Nguyen JH, Hughes CB. Polycystic liver disease and liver transplantation: single-institution experience. Transpl Proc. 2009;41(9):3769–71. https://doi.org/10.1016/j.transproceed.2009.05.043.CrossRefGoogle Scholar