ABSTRACT. Background: Mild liver dysfunction is common after prolonged use of parenteral nutrition (PN), but end-stage liver failure occurs only rarely. Few treatment options other than combined liver-intestine transplantation exist for patients with liver failure associated with PN use, however. Herein, we report the results of a cohort of patients undergoing isolated orthotopic liver transplantation (OLT) for PN-associated liver injury. Methods: A retrospective cohort study of 80 patients (73 pediatric patients and 7 adults) who have undergone isolated OLT for PN-associated liver injury as the primary indication for transplantation was performed. Results: At the time of OLT, the mean total serum bilirubin was 19.5 mg/dL and the mean serum albumin level was 2.9 mg/dL. Severe hepatic encephalopathy was seen in 5%, spontaneous bacterial peritonitis was seen in 6.3%, and respiratory failure requiring mechanical ventilation was seen in 14% of patients at the time of OLT. Overall 1- and 5-year survival rates were 72% and 52%, respectively, with infection being the most common cause of death after OLT. Retransplantation was required in 25% of patients, and the 5-year posttransplant patient survival rate only reached 35% in these cases. Conclusions: Patients with end-stage liver disease associated with PN administration often have very severe liver disease, multiple comorbidities, and poor prognosis by the time they are listed for OLT. Nonetheless, isolated OLT is associated with good long-term survival and should be considered for selected patients with combined intestine-liver failure. (Journal of Parenteral and Enteral Nutrition 30:526-529, 2006)

Parenteral nutrition (PN) has become an essential means of supporting patients who are be unable to sustain themselves on enterai feedings or intravenous (FV) carbohydrate solutions alone. Although lifesaving, PN is often associated with significant complications, including liver injury that can range from self-limited cholestasis to end-stage cirrhosis.1″5 Few good treatment options exist once cirrhosis has developed from long-term PN use. Perhaps the best option for those with PN-associated cirrhosis and continuing need for PN is a combined liver-intestine transplant, an option associated with 49%-55% long-term survival.6,7 Isolated orthotopic liver transplantation (OLT) exists as an option for selected patients, but in contrast to the combined liver-intestine procedure, the outcomes of OLT for PN-associated liver injury have only been described for single cases and single-institution series of no more than 10 patients.8,11 The current study was undertaken to estimate the long-term patient survival in pediatric and adult patients undergoing OLT for this rare indication.

MATERIALS AND METHODS

Patients enrolled in this study were identified through the United Network for Organ Sharing (UNOS) Organ Procurement and Transplant Network liver transplant database. This database contains patient and graft outcome data for >62,000 patients who underwent OLT in the United States between January 1988 and December 31, 2003. Patients with a primary diagnosis of PN-associated liver injury were selected for study. Patients who had received previous small intestine transplants or simultaneous multiorgan transplant were excluded.

Descriptive statistics for each cohort were calculated using all available pretransplant and peritransplant variables. Patient and allograft survival was assessed using Kaplan-Meier survival analysis, and survival rates of subgroups were compared with the log rank test. All statistical analyses were performed with SPSS version 11.0 (SPSS Corporation, Chicago, IL). A p value of

RESULTS

Patient Characteristics

Since 1988, a total of 80 patients have undergone isolated OLT, with PN-associated liver injury listed as the primary indication for transplantation. These patients were predominantly pediatric at the time of initial OLT. Specifically, 40 (50%) were

At the time of OLT, the mean total serum bilirubin was 19.5 mg/dL (range, 0.6-54.0 mg/dL), the mean serum creatinine was 0.58 mg/dL (range, 0.10-9.0 mg/dL), and the mean serum albumin was 2.9 mg/dL (range, 1.9-4.5 mg/dL). Eleven patients (14%) required mechanical ventilation at the time of OLT, and at least 1 had required dialysis in the week before OLT. Four patients (5.0%) had a history of spontaneous bacterial peritonitis, and 5 patients (6.3%) had a history of grade III or IV hepatic encephalopathy.

Twenty-three of the 73 pédiatrie recipients (32%) have been transplanted since the Pédiatrie End-Stage Liver Disease (PELD) model has been used to rank candidates awaiting OLT. Of these, 12 (52%) were listed as status 1 (high urgency, expected survival without transplantation of

Transplant and Allograft Characteristics

The median time from a candidate’s addition to the waiting list to the time of OLT was 35 days (range, 0-684 days). Of the 80 initial OLTs performed for PN-associated liver failure, whole-liver allografts obtained from cadaveric donors were used in 52 (65%), partial or reduced allografts from cadaveric donors were used in 9 (11%), and split-liver allografts from cadaveric donors were used in 7 (8.8%). Segmentai allografts from living donors were used in 10 (13%); 7 of these were obtained from the patient’s parents. The source of the remaining 2 allografts (3%) was unknown. The median length of posttransplant hospitalization was 38 days (range, 2-308 days).