[240] There is uncertainty whether ASC in children and PSC in adults have similar biological underpinnings or outcomes. In lieu of the phrase “overlap” syndrome, which suggests two separate conditions occurring in the same patient, the International Autoimmune Hepatitis Group posits that patients should be categorized by the predominant condition (e.g., AIH, PSC) and those with “overlapping” features should not be considered to be unique diagnosis.[249] Langerhans cell histiocytosis, primary and secondary immunodeficiencies, and cystic fibrosis have histological findings similar to PSC. LT is the only therapeutic option for endstage liver disease resulting from PSC.[250] Immunoglobulin

G4-associated cholangitis (IAC) is a newly recognized multisystem condition with intra- and extrahepatic NVP-BKM120 in vivo biliary strictures that is often, but not always, associated with autoimmune pancreatitis.[251, 252] Strictures disappear with corticosteroid therapy. Evidence of

IAC in children is currently limited to case reports with a similar clinical and biochemical presentation and response to corticosteroids that is seen in adults.[253] Patients with PSC are at higher risk of developing inflammatory bowel disease (IBD) than the general population, with ulcerative colitis and Crohn’s disease diagnosed see more before LT in 46% and 3.3% of children, respectively, and IBD diagnosed after LT in another 9.8%.[254] Cholangiocarcinoma in children is rare and not all cases are associated with PSC.[255] Risks associated with cholangiocarcinoma in PSC are not well defined in children,[250] but HCC may be more prevalent among children with Crohn’s disease and PSC.[255] PSC accounts for 3.5% of pediatric patients listed

for LT,[256] and 2.6% pediatric medchemexpress transplants.[254] LT is effective therapy for endstage liver disease due from PSC.[257] Patient and graft survival rates are comparable to those of age-matched children who undergo transplantation for other indications.[254] Post-LT complications include intrahepatic biliary strictures, cholangitis, and disease recurrence in the graft.[254] Patients with IBD and PSC have a higher recurrence rate post-LT compared to those with PSC alone. Five-year survival following LT for PSC is >80%.[258, 259] 55. Surveillance for inflammatory bowel disease with a full colonoscopy with biopsy both before and after LT in patients with features of primary sclerosing cholangitis is recommended. (2-B) 56. LT evaluation should be considered for patients with decompensated liver disease, recurrent cholangitis, unmanageable bile duct strictures, and/or concerns for the risk of cholangiocarcinoma. (2-B) Progressive familial intrahepatic cholestasis (PFIC) refers to a group of autosomal recessive cholestatic conditions. The nomenclature for these conditions is evolving as the underlying genetic defects and affected proteins are identified.