title: Liver and kidney transplantation for polycystic liver and kidney-renal function and outcome.
abstract: BACKGROUND
  Polycystic liver disease (PLD) is a rare disorder frequently associated with polycystic kidney disease (PKD). Transplantation is a treatment option for these patients. Because of preservation of hepatic function in these patients, liver transplantation is not routinely utilized. We report a large series of PLD patients and their outcomes following liver and kidney transplantation.
  
  
  METHODS
  Fourteen patients underwent orthotopic liver transplantation (OLTx) for PLD between 1987 and 2003. Twelve patients had PKD combined with PLD. Nine patients received only liver transplantation. Five patients had combined liver and kidney transplantation. Thirteen patients (93%) survived for at least one year following liver transplantation. Two out of eight patients who received solitary liver transplantation later required kidney transplantation.
  
  
  RESULTS
  Pretransplant glomerular filtration rate (GFR) in patients with PKD was 75.8+/-25.4 ml/min/1.73 m. One year later, GFR was 37.2+/-8.3 ml/min/1.73 m. Kaplan-Meier analysis showed that one- and two-year graft survival for combined liver and kidney transplantation was 80% (n=5), whereas graft survival for solitary liver transplantation was 100% (n=9). Mean survival of patients who had combined liver and kidney transplantation was 46.7+/-54.2 months (n=5), whereas the mean survival for solitary liver transplant patients was 80.4+/-68.6 months (n=9) (P=0.36).
  
  
  CONCLUSIONS
  Transplantation is an excellent option for PLD with dramatic improvement in quality of life and acceptable morbidity. For combined liver and kidney transplantation one- and two-year patient survival rates were similar to combined transplantation for other indications. For patients with acceptable renal function at time of transplantation, solitary liver transplantation has an excellent outcome.
authors.affiliation: Baylor Regional Transplant Institute, Dallas, TX. 2 Dallas Transplant Institute, Dallas, TX, USA.
authors.forename: Takehisa
authors.initials: T
authors.lastname: Ueno
authors.forename: Yousri M
authors.initials: YM
authors.lastname: Barri
authors.forename: George J
authors.initials: GJ
authors.lastname: Netto
authors.forename: Adrian
authors.initials: A
authors.lastname: Martin
authors.forename: Nicholas
authors.initials: N
authors.lastname: Onaca
authors.forename: Edmund Q
authors.initials: EQ
authors.lastname: Sanchez
authors.forename: Srinath
authors.initials: S
authors.lastname: Chinnakotla
authors.forename: Henry B
authors.initials: HB
authors.lastname: Randall
authors.forename: Sherfield
authors.initials: S
authors.lastname: Dawson
authors.forename: Marlon F
authors.initials: MF
authors.lastname: Levy
authors.forename: Robert M
authors.initials: RM
authors.lastname: Goldstein
authors.forename: Goran B
authors.initials: GB
authors.lastname: Klintmalm
mesh_terms: D000328:Adult; D003560:Cysts / Q000601:surgery*; D005260:Female; D006084:Graft Rejection / Q000209:etiology; D006801:Humans; D007431:Intraoperative Complications / Q000209:etiology; D007668:Kidney / Q000503:physiopathology*; D016030:Kidney Transplantation / Q000009:adverse effects; D008107:Liver Diseases / Q000601:surgery*; D016031:Liver Transplantation / Q000009:adverse effects; D008297:Male; D008875:Middle Aged; D007690:Polycystic Kidney Diseases / Q000601:surgery*; D011183:Postoperative Complications / Q000209:etiology