Liver transplantation for children with biliary atresia in the pediatric end-stage liver disease era: the role of insurance status.

Socioeconomic status influences health outcomes, although its impact on liver transplantation (LT) in children with biliary atresia (BA) is unknown. We hypothesized that governmental insurance [public insurance (PU)], rather than private insurance (PR), would be associated with poorer outcomes for children with BA. Children with BA who underwent first isolated LT between January 2003 and June 2011 were identified from United Network for Organ Sharing Standard Transplant Analysis and Research files. We identified 757 patients with PR and 761 patients with PU. The race/ethnicity distribution was significantly different between the groups (65% white, 12% black, and 10% Hispanic in the PR group and 33% white, 26% black, and 29% Hispanic in the PU group, P < 0.01). Wait-list mortality was higher for the PU group versus the PR group [46/1654 (2.7%) versus 29/1895 (1.5%), P < 0.01]. PR patients were older than PU patients at transplant (2.4 ± 4.5 versus 1.5 ± 3.0 years, P < 0.01). The donor types differed between the groups: 165 children (21.8%) in the PR group received living donor grafts, whereas 79 children (10.4%) in the PU group did (P < 0.01). The 1- and 5-year posttransplant patient survival rates were greater for the PR group versus the PU group (98.0% versus 94.1% at 1 year, P < 0.01; 97.8% versus 92.2% at 5 years, P < 0.01). Cox proportional hazards models revealed that the insurance type (PU), the donor type (deceased), and life support were significant risk factors for death. A separate analysis of deceased donor LT revealed that the PU group still had significantly worse patient and graft survival. In conclusion, PU coverage is an independent risk factor for significantly increased wait-list and posttransplant mortality in children with BA. Further studies are needed to unearth the reasons for these important differences in outcomes.

Hepatopulmonary syndrome in children - is conventional liver transplantation always needed?

BACKGROUND: Hepatopulmonary syndrome (HPS) is the association of liver disease, hypoxemia, and intrapulmonary vascular dilatations. There are little data on the management of HPS in children other than conventional orthotopic liver transplantation (OLT). AIMS: To describe the patient characteristics, mode of diagnosis, treatment, and outcomes of children with HPS at our center. METHODS: Retrospective review of patients diagnosed with HPS between 1997 and 2007 after IRB approval. RESULTS: There were 10 patients, six females; median age at diagnosis of HPS was 12 yr. Six with cirrhosis underwent OLT and had subsequent resolution of HPS and are stable at last follow-up. Of the remaining four, two had cirrhosis. HPS resolved without conventional OLT in the following four patients: hepatitis C after antiviral treatment, biliary atresia with portal hypertension after transjugular intrahepatic portosystemic shunting, Abernethy syndrome after auxiliary partial OLT, and in a child with splenic vein thrombosis after splenectomy. CONCLUSIONS: Our series shows resolution of HPS in all patients and 100% survival after conventional OLT. Four children had resolution of HPS after surgical or medical treatments other than conventional OLT. Careful review of clinical status and underlying pathophysiology and anatomy at diagnosis of HPS should inform treatment decisions.

Liver transplantation for children with Wilson disease: comparison of outcomes between children and adults.

UNLABELLED: Liver transplantation (LT) is lifesaving for patients with Wilson disease (WD) presenting with fulminant hepatic failure (FHF) or chronic liver disease (CLD) unresponsive to treatment. AIM: To determine the outcome of LT in pediatric and adult patients with WD. METHODS: United Network for Organ Sharing data on LT from 1987 to 2008 were analyzed. Outcomes were compared for patients requiring LT for FHF and CLD after 2002. Multivariate logistic regression was used to determine risk factors for death and graft loss. RESULTS: Of 90,867 patients transplanted between 1987 and 2008, 170 children and 400 adults had WD. The one- and five-yr patient survival of children was 90.1% and 89% compared to 88.3% and 86% for adults, p = 0.53, 0.34. After 2002, 103 (41 children) were transplanted for FHF and 67 (10 children) for CLD. One- and five-yr patient survival was higher in children transplanted for CLD compared to FHF; 100%, 100% vs. 90%, 87.5% respectively, p = 0.30, 0.32. One- and five-yr patient survival was higher in adults transplanted for CLD compared to FHF; 94.7%, 90.1% vs. 90.3%, 89.7%, respectively, p = 0.36, 0.88. Encephalopathy, partial graft, and ventilator use were risk factors for death by logistic regression. CONCLUSION: LT is an excellent treatment option for patients with WD. Patients transplanted for CLD had higher patient survival rates than patients with FHF.

Liver and combined lung and liver transplantation for cystic fibrosis: analysis of the UNOS database.

A proportion of patients with CF develop cirrhosis and portal hypertension. LT and combined LLT are rarely performed in patients with CF. To determine the outcome of LT and LLT in patients with CF. Patients with CF who had LT or LLT between 10/1987 and 5/2008 were identified from UNOS database. A total of 182 children (<18 yr) and 48 adults underwent isolated LT for CF. Seven more children and eight adults with CF underwent combined LLT. One- and five-yr patient and graft survival were not significantly different in patients who underwent LT in comparison with patients who underwent LLT (patient survival: LT; 83.9%, 75.7%, LLT; 80%, 80%; graft survival: LT; 76.1%, 67.0%, LLT; 80.0%, 80.0%, respectively). The two major causes of death after LT were pulmonary disease (15 patients, 22.7%) and hemorrhage (12 patients, 18.2%). Bilirubin was identified as a risk factor for death, and previous liver transplant and prolonged cold ischemic time were identified as risk factors for graft loss in LT patients. LT is a viable option for children and young adults with CF and end-stage liver disease. Outcome of LLT patients with CF was comparable to the outcome of patients with CF who underwent isolated LT.

Farber's disease type IV presenting with cholestasis and neonatal liver failure: report of two cases.

We report 2 siblings diagnosed with Farber's disease type IV. Type IV is a subtype of Farber's disease that presents in the neonatal period and usually initially lacks the triad of symptoms, including painful and deformed joints, subcutaneous nodules, and hoarse cry, classically seen in the other subtypes. While it is well known that all neonates with type IV present with hepatomegaly, a previously unrecognized presentation is that of cholestatic jaundice and rapidly evolving liver failure. We describe 2 siblings who presented with jaundice in the neonatal period and discuss the clinical data and variation in pathologic findings that should be considered for the diagnosis.