Advertisement

CEN Case Reports

, Volume 7, Issue 2, pp 307–312 | Cite as

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

  • Taro Akihisa
  • Ayami Ino
  • Hiroto Egawa
  • Yoshihito Kotera
  • Shunichi Ariizumi
  • Akiko Oomori
  • Shingo Yamashita
  • Yusuke Yamamoto
  • Ken Tsuchiya
  • Masakazu Yamamoto
  • Kosaku Nitta
  • Toshio Mochizuki
Case Report
  • 50 Downloads

Abstract

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.

Keywords

Liver transplantation Hemodialysis ADPKD Polycystic kidney disease Polycystic liver disease Liver cyst infection 

Notes

Acknowledgements

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.

Ethical approval

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

Informed consent was obtained from the patient.

References

  1. 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. 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
  3. 3.
    Mochizuki T, Wu G, Hayashi T, Xenophontos SL, Veldhuisen B, Saris JJ, et al. PKD2, a gene for polycystic kidney disease that encodes an integral membrane protein. Science. 1996;272(5266):1339–42.CrossRefPubMedGoogle Scholar
  4. 4.
    Drenth JP, te Morsche RH, Smink R, Bonifacino JS, Jansen JB. Germline mutations in PRKCSH are associated with autosomal dominant polycystic liver disease. Nat Genet. 2003;33(3):345–7.  https://doi.org/10.1038/ng1104.CrossRefPubMedGoogle Scholar
  5. 5.
    Davila S, Furu L, Gharavi AG, Tian X, Onoe T, Qian Q, et al. Mutations in SEC63 cause autosomal dominant polycystic liver disease. Nat Genet. 2004;36(6):575–7.  https://doi.org/10.1038/ng1357.CrossRefPubMedGoogle Scholar
  6. 6.
    Gevers TJ, Drenth JP. Diagnosis and management of polycystic liver disease. Nat Rev Gastroenterol Hepatol. 2013;10(2):101–8.  https://doi.org/10.1038/nrgastro.2012.254.CrossRefPubMedGoogle Scholar
  7. 7.
    Gabow PA, Johnson AM, Kaehny WD, Manco-Johnson ML, Duley IT, Everson GT. Risk factors for the development of hepatic cysts in autosomal dominant polycystic kidney disease. Hepatology. 1990;11(6):1033–7.CrossRefPubMedGoogle Scholar
  8. 8.
    Chapman AB. Cystic disease in women: clinical characteristics and medical management. Adv Ren Replace Ther. 2003;10(1):24–30.  https://doi.org/10.1053/jarr.2003.50005.CrossRefPubMedGoogle Scholar
  9. 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
  10. 10.
    Bistritz L, Tamboli C, Bigam D, Bain VG. Polycystic liver disease: experience at a teaching hospital. Am J Gastroenterol. 2005;100(10):2212–7.  https://doi.org/10.1111/j.1572-0241.2005.50258.x.CrossRefPubMedGoogle Scholar
  11. 11.
    Gigot JF, Jadoul P, Que F, Van Beers BE, Etienne J, Horsmans Y, et al. Adult polycystic liver disease: is fenestration the most adequate operation for long-term management? Ann Surg. 1997;225(3):286–94.CrossRefPubMedPubMedCentralGoogle Scholar
  12. 12.
    Sallee M, Rafat C, Zahar JR, Paulmier B, Grunfeld JP, Knebelmann B, et al. Cyst infections in patients with autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol CJASN. 2009;4(7):1183–9.  https://doi.org/10.2215/CJN.01870309.CrossRefPubMedGoogle Scholar
  13. 13.
    Telenti A, Torres VE, Gross JB Jr, Van Scoy RE, Brown ML, Hattery RR. Hepatic cyst infection in autosomal dominant polycystic kidney disease. Mayo Clin Proc. 1990;65(7):933–42.CrossRefPubMedGoogle Scholar
  14. 14.
    Suwabe T, Ubara Y, Mise K, Ueno T, Sumida K, Yamanouchi M, et al. Suitability of patients with autosomal dominant polycystic kidney disease for renal transcatheter arterial embolization. J Am Soc Nephrol JASN. 2016;27(7):2177–87.  https://doi.org/10.1681/ASN.2015010067.CrossRefPubMedGoogle Scholar
  15. 15.
    Lantinga MA, Geudens A, Gevers TJ, Drenth JP. Systematic review: the management of hepatic cyst infection. Aliment Pharmacol Ther. 2015;41(3):253–61.  https://doi.org/10.1111/apt.13047.CrossRefPubMedGoogle Scholar
  16. 16.
    Kwok MK, Lewin KJ. Massive hepatomegaly in adult polycystic liver disease. Am J Surg Pathol. 1988;12(4):321–4.CrossRefPubMedGoogle Scholar
  17. 17.
    Lang H, von Woellwarth J, Oldhafer KJ, Behrend M, Schlitt HJ, Nashan B, et al. Liver transplantation in patients with polycystic liver disease. Transpl Proc. 1997;29(7):2832–3.CrossRefGoogle Scholar
  18. 18.
    Demirci G, Becker T, Nyibata M, Lueck R, Bektas H, Lehner F, et al. Results of combined and sequential liver-kidney transplantation. Liver Transpl. 2003;9(10):1067–78.  https://doi.org/10.1053/jlts.2003.50210.CrossRefPubMedGoogle Scholar
  19. 19.
    Chandok N, Uhanova J, Marotta P. Clinical outcomes of liver transplantation for polycystic liver disease: a single center experience. Ann Hepatol. 2010;9(3):278–81.PubMedGoogle Scholar
  20. 20.
    Starzl TE, Reyes J, Tzakis A, Mieles L, Todo S, Gordon R. Liver transplantation for polycystic liver disease. Arch Surg. 1990;125(5):575–7.CrossRefPubMedGoogle Scholar
  21. 21.
    Aussilhou B, Doufle G, Hubert C, Francoz C, Paugam C, Paradis V, et al. Extended liver resection for polycystic liver disease can challenge liver transplantation. Ann Surg. 2010;252(5):735–43.  https://doi.org/10.1097/SLA.0b013e3181fb8dc4.CrossRefPubMedGoogle Scholar
  22. 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
  23. 23.
    Temmerman F, Missiaen L, Bammens B, Laleman W, Cassiman D, Verslype C, et al. Systematic review: the pathophysiology and management of polycystic liver disease. Aliment Pharmacol Ther. 2011;34(7):702–13.  https://doi.org/10.1111/j.1365-2036.2011.04783.x.CrossRefPubMedGoogle Scholar
  24. 24.
    Kirchner GI, Rifai K, Cantz T, Nashan B, Terkamp C, Becker T, et al. Outcome and quality of life in patients with polycystic liver disease after liver or combined liver-kidney transplantation. Liver Transpl. 2006;12(8):1268–77.  https://doi.org/10.1002/lt.20780.CrossRefPubMedGoogle Scholar
  25. 25.
    Ogawa K, Fukunaga K, Takeuchi T, Kawagishi N, Ubara Y, Kudo M, et al. Current treatment status of polycystic liver disease in Japan. Hepatol Res. 2014;44(11):1110–8.  https://doi.org/10.1111/hepr.12286.CrossRefPubMedGoogle Scholar
  26. 26.
    Nair S, Verma S, Thuluvath PJ. Pretransplant renal function predicts survival in patients undergoing orthotopic liver transplantation. Hepatology. 2002;35(5):1179–85.  https://doi.org/10.1053/jhep.2002.33160.CrossRefPubMedGoogle Scholar
  27. 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
  28. 28.
    Krohn PS, Hillingso JG, Kirkegaard P. Liver transplantation in polycystic liver disease: a relevant treatment modality for adults? Scand J Gastroenterol. 2008;43(1):89–94.CrossRefPubMedGoogle Scholar
  29. 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

Copyright information

© Japanese Society of Nephrology 2018

Authors and Affiliations

  • Taro Akihisa
    • 1
  • Ayami Ino
    • 1
  • Hiroto Egawa
    • 2
  • Yoshihito Kotera
    • 2
  • Shunichi Ariizumi
    • 2
  • Akiko Oomori
    • 2
  • Shingo Yamashita
    • 2
  • Yusuke Yamamoto
    • 3
  • Ken Tsuchiya
    • 4
  • Masakazu Yamamoto
    • 2
  • Kosaku Nitta
    • 1
  • Toshio Mochizuki
    • 1
    • 5
  1. 1.Department of Medicine, Kidney CenterTokyo Women’s Medical UniversityTokyoJapan
  2. 2.Department of Surgery, Institute of GastroenterologyTokyo Women’s Medical UniversityTokyoJapan
  3. 3.Department of Plastic and Reconstructive SurgeryTokyo Women’s Medical UniversityTokyoJapan
  4. 4.Department of Blood Purification, Kidney CenterTokyo Women’s Medical UniversityTokyoJapan
  5. 5.Clinical Research Division for Polycystic Kidney Disease, Department of Medicine, Kidney CenterTokyo Women’s Medical UniversityTokyoJapan

Personalised recommendations