tailieunhanh - Diseases of the Liver and Biliary System - part 7

Ở trẻ em, một thời gian prothrombin kéo dài (INR ), bilirubin toàn phần lớn hơn 100 mmol / l và đồng huyết thanh lớn hơn 12 mmol / l lúc chẩn đoán được thực hiện cùng nhau dự đoán một kết quả nghèo và có thể hữu ích trong việc xác định | Wilson s Disease 421 serum bilirubin asparate transaminase and prothrombin time are ominous signs 23 . In children a prolonged prothrombin time INR total bilirubin greater than 100 gmol l and a serum copper of greater than 12 gmol l at diagnosis taken together predict a poor outcome and may be helpful in identifying patients where liver transplantation should be considered 7 . Liver transplantation is life saving in such patients. Otherwise death is from liver failure bleeding oesophageal varices or intercurrent infections in those bedridden from neurological disability. Indian childhood cirrhosis See Chapter 26. Hereditary acaeruloplasminaemia See Chapter 23. References 1 Brewer GJ. Penicillamine should not be used as initial therapy in Wilson s disease. Mov. Disord. 1999 14 551. 2 Brewer GJ. Recognition diagnosis and management of Wilson s disease. Proc. Soc. Exp. Biol. Med. 2000 223 39. 3 Brewer GJ Dick RD Johnson V et al. Treatment of Wilson s disease with zinc XV. Long-term follow-up studies. J. Lab. Clin. Med. 1998 132 264. 4 Brewer GJ Johnson VD Dick RD et al. Treatment of Wilson s disease with zinc XVII. Treatment during pregnancy. Hepatology 2000 31 364. 5 Bull PC Cox DW. Wilson disease and Menkes disease new handles on heavy-metal transport. Trends Genet. 1994 10 246. 6 Dahlman T Hartvig P Lôholm M et al. Long-term treatment of Wilson s disease with triethylene tetramine dihydrochloride trientine . Q. J. Med. 1995 88 609. 7 Dhawan A D Silva P Taylor RM et al. Wilson s disease in children predictors of outcome. Thirty years experience at King s College Hospital. J. Hepatol. 2000 32 suppl. 2 136. 8 Eghtesad B Nezakatgoo N Geraci LC et al. Liver transplantation for Wilson s disease a single-centre experience. Liver Transplant Surg. 1999 5 467. 9 Faa G Nurchi V Demelia L et al. Uneven hepatic copper distribution in Wilson s disease. J. Hepatol. 1995 24 303. 10 Ferenci P. Wilson s disease. In Bacon BR Di Bisceglie AM eds. Liver Disease Diagnosis and .