290,291 Seventy percent of patients improve their clinical and laboratory findings within 2 years, and survival is preserved.354,355,357 Histological remission is achieved in only 20%, and most patients remain on therapy and at risk for drug-related side effects and/or disease progression.354,355,357 The development of hepatic encephalopathy, ascites, and/or variceal hemorrhage during therapy for treatment failure is an indication for liver transplantation.11,73 Protracted therapy that has improved the clinical, laboratory, and histological indices but not induced complete resolution constitutes an incomplete response (Table 8).282-285 Thirteen percent of

patients Pictilisib research buy fail to enter remission after 36 months of treatment, and they are classified as incomplete responders. In these instances, alternative

strategies must be considered. Long-term low dose corticosteroid therapy involves a gradual decrease in the prednisone dose by 2.5 mg per month until the lowest level (≤10 mg daily) is achieved, and the serum AST or ALT level remains stable.282-285,329 Long-term Galunisertib clinical trial azathioprine (2 mg/kg daily) can also be used to stabilize the serum AST and ALT levels in corticosteroid intolerant individuals who require continuous treatment.282-285,327 Drug toxicity justifies premature discontinuation or alteration of conventional therapy in 13% of patients (Table 8).277,282-285 In these instances, therapy with the tolerated agent (prednisone or azathioprine) can be maintained in adjusted dose to prevent

worsening in the clinical and laboratory features.282-285 The treatment endpoints for children are similar to those of adults. Almost all children demonstrate improvement in liver tests within the first 2-4 weeks of treatment with either prednisone or prednisone and azathioprine.35,36,279-281,283,305,358-361 Some 80%-90% achieve laboratory remission in 6-12 months. Cetuximab ic50 In most treatment protocols, high-dose prednisone (1-2 mg/kg daily) is administered for up to 2 weeks, at which time a gradual decrease in dose is undertaken to reach a maintenance level (usually 0.1-0.2 mg/kg daily or 5 mg daily) in 6-8 weeks.35,36,279-281,283,305,358-361 Clinical and laboratory parameters are usually sufficient to determine the adequacy of response. Flares in disease activity, as assessed by an increase in serum AST or ALT level, are treated with a temporary increase in corticosteroid dose. The goal of treatment in children is to have minimal or no serum AST or ALT abnormality on the lowest dose of medication possible.(35, 36, 279-281, 283, 305, 358-361) Long-term, low-dose therapy is anticipated and emotional, cosmetic, and growth-related side effects temper treatment in an individualized fashion.