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INTRODUCTION

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Liver transplantation is widely recognized as the standard of care for the management of irreversible acute liver failure and end-stage liver disease in children. Advances in diagnosis and management of liver disease in children and improvements in transplant surgical technique and perioperative care have contributed to improved graft and patient survival. At the same time, recognition of the importance of nutritional support, management of portal hypertension and ascites, and prompt management of infection have contributed to improved overall condition of children awaiting liver transplant. Changes in allocation policies and in transplant center listing practices have resulted in more active management of the patient’s candidacy and in broader geographic distribution of pediatric donor livers. Improved understanding of post-transplant immunosuppression, and earlier recognition of rejection, infection, and vascular and biliary complications have also contributed to improved outcomes. Advances in ultrasound and other radiologic imaging and the integrated involvement of interventional radiology into transplant care team have allowed for less invasive and often earlier diagnosis and treatment of certain post-transplant complications. This improved overall outcome has allowed for the expanded consideration of liver transplant in select clinical situations for quality-of-life indications, though the primary indication for liver transplant remains increased survival of end-stage liver disease.

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EPIDEMIOLOGY

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In 2014, approximately 400 children were active candidates waiting for a liver transplant on the United Network for Organ Sharing (UNOS) national waiting list. Six hundred fifty-five children were added to the waiting list in 2014, 9.1% of whom were listed for retransplantation. Four hundred seventy-eight children received a deceased donor liver transplant in 2014, representing approximately 72% of children removed from the waiting list. Another 51 children (7.7%) received a living donor (LD) liver transplant. Ten percent of candidates were removed from the list because of improved condition, 4.9% died on the list, and approximately 2.5% were considered too sick to transplant. Overall pretransplant mortality improved to 5.9 deaths per 100 waitlist-years (WL-years), but remained highest in children under 1 year of age (12.4 deaths per 100 WL-years). Though still high, this mortality rate is significantly lower than the rate of 40.95 recorded ten years earlier.

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Compared to 2004 to 2005, there were fewer children added to the waiting list, fewer retransplant registrations, and a decrease in pretransplant mortality rate. Candidates listed for liver-kidney transplant increased over this same time interval from 1.4% in 2004 to 3.1% in 2014. Similarly, liver-intestine candidates increased from 1.1% in 2004 to 10.1% in 2014. Despite these changes in waitlist composition, the overall number of liver transplants performed each year has remained fairly stable at approximately 530 per year. Although the overall pediatric transplant activity has been fairly constant, there is significant discrepancy between different age groups. Due to the clinical presentation of biliary atresia and of other congenital liver diseases in early infancy, children younger than 1 year of age have the highest transplant rates among those active on the waiting ...

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