[359, 360] LT is curative, and typically occurs in the setting of multivisceral transplantation following an abdominal catastrophe due to mesenteric vein thrombosis or renal vein thrombosis.[361] 83. Medical management of Factor VII and Protein C deficiency is preferred; LT should be considered only for those who experience complications or failure of management. (2-B) Budd-Chiari syndrome (BCS) is the result of hepatic venous outflow tract obstruction at any level from any mechanism, exclusive of cardiac disease. An underlying risk factor for thrombosis is identified in up to 87% of adult BCS cases.[362, 363] Whether this

prevalence is similar in children with BCS is not known, as evaluation for underlying prothrombotic conditions has not been routinely GSI-IX ic50 investigated in this age group. Prognostic scoring systems have not been systematically evaluated in children.[363, 364] Transjugular intrahepatic portosystemic shunts (TIPS) should be considered in the majority of patients not responsive Selleck Autophagy Compound Library to medical therapy and have been successfully used in children.[365, 366] Well-selected patients with acute liver failure or advanced chronic disease from BCS can benefit from LT with good long-term posttransplant survival. LT is generally thought to be contraindicated for BCS that occurs due to paroxysmal nocturnal hemoglobinuria, as recurrence of

intravascular thrombosis in the graft can be expected; however, scheduled treatments with

the anti-complement antibody eculizumab, before and after LT, resulted in stable graft function without radiographic recurrence of thrombosis 1.5 years following LT.[367] Most patients transplanted for BCS remain on some form of prolonged anticoagulation.[368-371] 84. Patients with progressive endstage liver disease from BCS benefit from LT, where others with less severe disease may benefit from alternative therapy. (2-B) Noncirrhotic portal hypertension (NCPH) is often classified based on the level of the vascular obstruction into suprahepatic, intrahepatic, or prehepatic, and is the result of an obliterative vasculopathy resulting from a variety of insults such as infections, drugs or toxins, immune disorders, or thrombophilic states. Patients of all age groups will typically check details present with gastrointestinal hemorrhage and splenomegaly, and less commonly with hepatic synthetic failure.[372] Endoscopic control of variceal hemorrhage, TIPS, or surgical shunts are usually available as options in the management of patients with NCPH.[373] The meso-Rex bypass is an excellent option if the child has an accessible intrahepatic left portal vein.[373] Increasingly, hepatopulmonary syndrome (HPS) is recognized as a complication of NCPH.[374, 375] If HPS is present prior to initiating a management strategy for portal hypertension associated with NCPH, the clinical features of HPS may worsen HPS, or if not present, may develop.

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