Outcome and quality of life in patients with polycystic liver disease after liver or combined liver-kidney transplantation

Abstract

In advanced stages of polycystic liver disease, often associated with polycystic kidney disease, a curative therapy is liver or combined liver-kidney transplantation. However, little is known about long-term outcome and quality of life. Between 1990 and 2003, 36 patients (32 female, 4 male) with polycystic liver or combined liver-kidney disease underwent liver (n ϭ 21) or liver-kidney (n ϭ 15) transplantation at our center. Main indications for liver transplantation were cachexia, muscle atrophy, loss of weight, recurrent cyst infections, portal hypertension, and ascites. Apart from clinical parameters, 2 anonymous questionnaires (standard short form 36 and self-designed) addressing quality of life and social status were evaluated. Five patients (14 %) died due to sepsis or myocardial infarction with pneumonia, all within 61 days after transplantation. The follow-up time of the remaining 31 patients ranged from 5 to 156 months, with a mean of 62 months. Of the 23 (74%) answered the questionnaires, 91% of patients felt "much better" or "better," only 9% felt "worse" than before, and 52% of patients participated in sports regularly. Fatigue, physical fitness, loss of appetite, and vomiting improved significantly after transplantation. Physical attractiveness and interest in sex increased as well. Professional occupation did not change for 71% of patients. Family situation before and after transplantation changed in 1 case only. Finally, 78% of patients said they would opt for transplantation again, while 17% were undecided; 1 patient would not repeat transplantation. In conclusion, patients with advanced polycystic liver or polycystic liver-kidney disease have an excellent survival rate and an improved quality of life after liver or combined liver-kidney transplantation.

Repeated Minocycline Hydrochloride Injections for Symptomatic Polycystic Liver Disease

“…It reflects disease severity and is useful when choosing treatment options. Recently, several reports on PLD treatment have recommended aspiration sclerotherapy, cyst fenestration, and liver resection for Gigot type 1 or 2 PLD and liver transplantation for type 3 PLD; however, no standardized criteria for the selection of treatment exist [5,23,35,46,59,64,65,[73][74][75] . In our series, all patients had Gigot type 2 PLD.…”

Development and validation of a polycystic liver disease complaint-specific assessment (POLCA)

“…Studies thus far only evaluated HRQL in relation to cyst fenestration and LTx as an outcome in PCLD patients [23,24,34]. Although improvement was seen in some HRQL domains using the SF36 version1 in the two randomized placebo controlled trials, the extent to which SA ease complaints using non-disease specific questionnaires remained unclear [7,8].…”

“…Using search terms referring to the disease, its treatment forms and aspects of health-related quality of life, 15 articles were identified for data extraction [4,5,7,8,[17][18][19][20][21][22][23][24][25][26][27].…”

Surgical management of polycystic liver disease

“…Fatigue, physical fitness, anorexia, vomiting, physical attractiveness, and interest in sex improved significantly after transplantation. Overall, patients with advanced PCLD have an improved quality of life after liver or combined liver-kidney transplantation [66] .…”