Malnutrition is a treatable complication in children with end-stage liver disease (ESLD). Biliary atresia and other cholestatic disorders are the most frequent cause of ESLD in children. No single variable provides adequate information about nutrition status, yet effective nutrition support is the one intervention known to improve pre- and posttransplant outcomes. A proactive approach consisting of screening anthropometry interpreted using appropriate growth references, recognition of clinical manifestations associated with micronutrient deficiency, and timely aggressive nutrition support is of a paramount importance to maximize anabolism and optimize outcomes. This article presents the principles of nutrition assessment, intervention, and monitoring in children with ESLD.

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We conducted a cross-sectional study of patients who underwent pediatric liver transplant between 1988 to1992 to evaluate long-term health status. Survivors completed socio-demographic, medical and Health Related Quality of Life (HRQOL) surveys by mail including the SF 36v2, PedsQL™4.0 Generic Core Scale, PedsQL™ Cognitive Functioning Scale and PedsQL™3.0 Transplant Module. SF 36 scores were converted to SF6D-based utilities and risk factors for lower outcomes were assessed. Eighty-five of 171 patients had survived, Fifty-six were contacted with a response rate of 66%. Median age at LT was 0.86 yrs (IQR 0.58–3.0) and 64.3% had biliary atresia. Mean age at survey was 23.0±4.4 years. 62% attended college, 68% lived with parents and 80% of those over 23 were employed. Patient health utilities were lower than norms (0.75±0.12 versus 0.82±0.18, p<0.01) and correlated with unemployment (p<0.042), hospitalizations (p<0.005), and lower education level (p<0.016). Lower PedsQL™3.0 Transplant Module and PedsQL™ 4.0 Generic Core Scale scores correlated with unemployment (p=0.006, p=0.009) and hospitalizations (p=0.006, p=0.02). Pediatric transplant recipients who survive to adulthood have lower physical HRQOL, measureable transplant related disability and lower health utility. Transplantation is life saving however physical and psychological sequelae continue to affect health status up to two decades later.

The acquired or perinatal form of biliary atresia is a Th1 fibro-inflammatory disease affecting both the extrahepatic and intrahepatic bile ducts. Osteopontin (OPN) is a Th1 cytokine implicated in several fibro-inflammatory and autoimmune diseases. We examined the expression of OPN in acquired biliary atresia in comparison to normal liver and several pediatric cholestatic liver diseases. We also assessed OPN expression by cultured human bile duct epithelial cells. We found that liver OPN mRNA and protein expression were significantly increased in biliary atresia versus normal and other cholestatic diseases. OPN expression in biliary atresia was localized to epithelium of proliferating biliary structures (ductules and/or ducts) and bile plugs contained therein. No portal biliary OPN expression could be demonstrated in normal liver, syndromic biliary atresia, biliary obstruction not due to biliary atresia, and idiopathic neonatal hepatitis. OPN expression by human bile duct epithelial cells in culture was responsive to IL-2 and TNF-␣. Our results demonstrate an up-regulation of OPN expression by interlobular biliary epithelium in biliary atresia, which correlates with biliary proliferation and portal fibrosis. These findings suggest a role for OPN in the pathogenesis of biliary atresia. Biliary atresia is the most common liver disease of infancy that leads to cirrhosis, end-stage liver disease and the need for liver transplantation (1). A small proportion of cases are thought to be congenital in that they are associated with other developmental anomalies, whereas most cases are diagnosed at 4 -8 wk of age and appear to represent a disease acquired in perinatal or neonatal life. The etiology and pathogenesis of biliary atresia remain unknown. A current working unifying hypothesis that can explain the clinical and pathologic features of the acquired form of biliary atresia is that some injury to the bile ducts, likely a perinatal viral infection, leads to an autonomous immune response that results in progressive bile duct injury and fibrosis (2). This progressive, inflammatory cholangiopathy results in extrahepatic bile duct obstruction (1,3). The extrahepatic bile duct is partially or entirely obliterated by fibrosis in association with inflammation within the fibrous remnant. The intrahepatic biliary system also appears to be affected by the primary disease process. Portal areas are expanded with fibrosis and exhibit marked proliferation of biliary elements (ductules and/or ducts) (4 -8). These duct structures often contain bile plugs. Biliary proliferation may still be a feature of the disease even at end-stage, but in some cases all duct structures disappear.Previous studies have shown that the mononuclear inflammatory infiltrate in the portal tracts comprises macrophages, CD4ϩ T-cells and NK cells (9 -13). We have shown that the inflammation is typically Th1 in character, in that it consists mainly of macrophages, CD4ϩ T-cells and CD8 ϩ T-cells with production of IL-2, IFN-␥, . The inciting agent leading to thi...