[7] Recurrent cholangitis with or without associated decompensation of liver function can be an indication for LT in

BA.[145] At least 80% of patients with BA are transplanted by 20 years of age, with the majority transplanted under 4 years of age.[7, 126] Technical variant grafts (i.e., living related, split) are frequently utilized in smaller children with comparable results.[140] In the U.S., the overall 10-year actuarial graft and patient survival for liver transplant in BA is 73% and 86%, respectively.[146] 32. Hepatoportoenterostomy (HPE) is the preferred initial management for biliary atresia (1-B), but liver transplant evaluation should be considered in infants with evidence of decompensated liver disease prior to HPE. (2-B) 33. Aggressive nutritional ABT-263 purchase support prior to LT is needed to improve outcomes in cholestatic children with BA. (1-B) 34. BA patients post-HPE Selleck R428 should be promptly referred for LT evaluation if the total bilirubin is greater than 6 mg/dL beyond 3 months from HPE (1-B); liver transplant evaluation should be considered in

BA patients whose total bilirubin remains between 2-6 mg/dL (1-B), and for those with lesser bilirubin values who have unmanageable consequences of biliary cirrhosis or portal hypertension. (2-B) 35. High-dose corticosteroid therapy initiated within 72 hours of HPE is not recommended. (1-B) Alagille syndrome (AGS) is an autosomal dominant, multisystem disorder which may affect the liver, heart, eyes, and skeleton, kidneys, and cerebro-vascular or peripheral vascular systems with recognizable facial features including triangular facies, hypertelorism, prominent forehead, and pointed chin.[147-150] Liver involvement ranges from minimal liver

test abnormalities to biliary cirrhosis. Infants and children with AGS and significant cholestasis may experience well-compensated liver disease with absent or minimal evidence of clinical liver disease later in life.[151] An estimated 20% to 30% of patients with AGS will require LT.[152-155] Impaired synthetic function, uncontrolled portal hypertension, and chronic encephalopathy are uncommon in AGS. Complications of profound cholestasis, intractable pruritus, failure to thrive, severe hypercholesterolemia, and MCE osteodystrophy have prompted consideration for LT.[62, 122, 153, 155-157] However, partial internal biliary diversion,[158] partial external biliary diversion,[159] and ileal exclusion[160] have improved pruritus, xanthoma burden, and quality of life in some patients. Hypercholesterolemia associated with AGS is predominantly due to elevations in lipoprotein X which may, in fact, protect against atherosclerosis.[161] Reduced somatic growth parameters are recognized components of AGS. While cholestasis is resolved by LT, growth parameters may not be completely reversed by LT.