864 Standardised Mortality Ratio in Autoimmune Hepatitis (Aih) (original) (raw)

2008, Journal of Hepatology

POSTERS immunoglobulin G (IgG) subclasses, and standard autoantibodies were recorded and, when unavailable, determined on archived blood samples. Results: Thirty patients (14 male) with CP were identified. Median age at presentation was 9 years range, 0.4−15. Presentation symptoms included abdominal pain (30), food intolerance (29) and obstructive jaundice (10). Three patients had long common choledocho-pancreatic channel, 1 double outlet pancreatic duct, 8 common bile or pancreatic duct dilatation and/or stricture, 2 pancreatic and 1 gallstones, 4 inflammatory pseudotumour, 1 post-chemotherapy biliary stricture (medulloblastoma), 1 systemic lupus erythematosus, 1 portal vein thrombosis and 8 cryptogenic pancreatitis. Ten patients (33%; 4 male) had raised age-adjusted IgG4 levels, with normal total IgG levels. Median IgG4 was 1.73 g/L range, 0.065−3.3. Autoantibodies were negative in all except in 1 (SMA 1:40). At presentation, median serum amylase was 195 IU/L range, 27-638; normal <100 IU/L and triglycerides 1.7 mmol/L range, 0.9−2.1; normal 0.5−2 mmol/L. All tested patients were negative for common cystic fibrosis mutations. 3/6 patients tested for hereditary pancreatitis had PRSS1 mutation in the cationic trypsinogen gene. Irregularity of the pancreatic duct was documented in 7 children CT = 2, MRCP = 5. ERCP showed a stricture affecting the pancreatic duct in 3 patients, 2 of whom required stent insertion. Subsequently, 2 of them underwent a pancreatico-enterostomy (Puestow procedure). One girl was treated with prednisolone (60 mg) and azathioprine (2 mg/kg/d) and normalized IgG4 within 2 months, but developed diabetes mellitus. In the remaining 9 patients pancreatitic symptoms improved after endoscopic or surgical treatment median follow up: 12 months (range, 4−84). Conclusion: One-third of our patients with CP fulfilled clinical criteria for AIP. Some of them possess PRSS1 mutation and conventional endoscopico-surgical treatment appears to be superior.