UpToDate
Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate®

儿童急性肝衰竭:病因及评估

Author
Robert H Squires, Jr, MD, FAAP
Section Editor
Elizabeth B Rand, MD
Deputy Editor
Alison G Hoppin, MD
Translators
吴晓燕, 主治医师

引言

儿科急性肝衰竭(pediatric acute liver failure, PALF)是一种复杂的、进展迅速的临床综合征,是许多迥然不同的疾病(其中一些已知,另一些有待确定)的最终共同通路[1-3]。在美国,所有年龄组中急性肝衰竭(acute liver failure, ALF)的估计发病频率约为每年17例/100,000人,但是儿童的发病频率尚不清楚。在美国,PALF占每年进行的儿科肝移植中的10%-15%。

PALF是一种进展迅速的临床疾病。目前尚无足够有力的研究可以告知这一疾病的诊断流程、评估疾病严重程度和发展轨迹的标志物或指导关于肝脏移植(liver transplantation, LT)的决策。临床医生必须制定个体化的诊断方案和治疗策略。治疗需要包括肝脏疾病专科医生、重症医疗专科医生及肝脏移植外科医生在内的多学科小组。

当PALF儿童首次就诊时面对的紧迫临床问题包括:

患儿的疾病是可治疗的吗?

当患儿保留原有肝脏生存时,每日恶化或改善的风险是什么?

                               

Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: 2017-06 . | This topic last updated: 2017-06-20.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
References
Top
  1. Squires RH Jr, Shneider BL, Bucuvalas J, et al. Acute liver failure in children: the first 348 patients in the pediatric acute liver failure study group. J Pediatr 2006; 148:652.
  2. Squires RH Jr. Acute liver failure in children. Semin Liver Dis 2008; 28:153.
  3. Squires RH, Alonso EM. Acute liver failure in children. In: Liver Disease in Children, 4th ed, Suchy FJ, Sokol RJ, Balistreri WF (Eds), Cambridge University Press, New York 2012.
  4. Heubi JE, Barbacci MB, Zimmerman HJ. Therapeutic misadventures with acetaminophen: hepatoxicity after multiple doses in children. J Pediatr 1998; 132:22.
  5. Watkins PB, Seeff LB. Drug-induced liver injury: summary of a single topic clinical research conference. Hepatology 2006; 43:618.
  6. Rivera-Penera T, Gugig R, Davis J, et al. Outcome of acetaminophen overdose in pediatric patients and factors contributing to hepatotoxicity. J Pediatr 1997; 130:300.
  7. Leonis MA, Alonso EM, Im K, et al. Chronic acetaminophen exposure in pediatric acute liver failure. Pediatrics 2013; 131:e740.
  8. Abboud G, Kaplowitz N. Drug-induced liver injury. Drug Saf 2007; 30:277.
  9. Reuben A, Koch DG, Lee WM, Acute Liver Failure Study Group. Drug-induced acute liver failure: results of a U.S. multicenter, prospective study. Hepatology 2010; 52:2065.
  10. Stickel F, Patsenker E, Schuppan D. Herbal hepatotoxicity. J Hepatol 2005; 43:901.
  11. Narkewicz MR, Dell Olio D, Karpen SJ, et al. Pattern of diagnostic evaluation for the causes of pediatric acute liver failure: an opportunity for quality improvement. J Pediatr 2009; 155:801.
  12. Hennes EM, Zeniya M, Czaja AJ, et al. Simplified criteria for the diagnosis of autoimmune hepatitis. Hepatology 2008; 48:169.
  13. Mileti E, Rosenthal P, Peters MG. Validation and modification of simplified diagnostic criteria for autoimmune hepatitis in children. Clin Gastroenterol Hepatol 2012; 10:417.
  14. McClain K, Gehrz R, Grierson H, et al. Virus-associated histiocytic proliferations in children. Frequent association with Epstein-Barr virus and congenital or acquired immunodeficiencies. Am J Pediatr Hematol Oncol 1988; 10:196.
  15. Parizhskaya M, Reyes J, Jaffe R. Hemophagocytic syndrome presenting as acute hepatic failure in two infants: clinical overlap with neonatal hemochromatosis. Pediatr Dev Pathol 1999; 2:360.
  16. Henter JI, Horne A, Aricó M, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007; 48:124.
  17. Pan X, Kelly S, Melin-Aldana H, et al. Novel mechanism of fetal hepatocyte injury in congenital alloimmune hepatitis involves the terminal complement cascade. Hepatology 2010; 51:2061.
  18. Rand EB, Karpen SJ, Kelly S, et al. Treatment of neonatal hemochromatosis with exchange transfusion and intravenous immunoglobulin. J Pediatr 2009; 155:566.
  19. Helbling D, Buchaklian A, Wang J, et al. Reduced mitochondrial DNA content and heterozygous nuclear gene mutations in patients with acute liver failure. J Pediatr Gastroenterol Nutr 2013; 57:438.
  20. Sundaram SS, Alonso EM, Narkewicz MR, et al. Characterization and outcomes of young infants with acute liver failure. J Pediatr 2011; 159:813.
  21. Patterson M. Niemann-Pick disease type C. GeneReviews. Available at: http://www.ncbi.nlm.nih.gov/books/NBK1296/ (Accessed on April 18, 2012).
  22. Lee WS, Sokol RJ. Mitochondrial hepatopathies: advances in genetics, therapeutic approaches, and outcomes. J Pediatr 2013; 163:942.
  23. Shneider BL, Rinaldo P, Emre S, et al. Abnormal concentrations of esterified carnitine in bile: a feature of pediatric acute liver failure with poor prognosis. Hepatology 2005; 41:717.
  24. Korman JD, Volenberg I, Balko J, et al. Screening for Wilson disease in acute liver failure: a comparison of currently available diagnostic tests. Hepatology 2008; 48:1167.
  25. Moreira-Silva SF, Frauches DO, Almeida AL, et al. Acute liver failure in children: observations in Vitória, Espírito Santo State, Brazil. Rev Soc Bras Med Trop 2002; 35:483.
  26. Bartholomeusz A, Locarnini S. Hepatitis B virus mutants and fulminant hepatitis B: fitness plus phenotype. Hepatology 2001; 34:432.
  27. Farci P, Alter HJ, Shimoda A, et al. Hepatitis C virus-associated fulminant hepatic failure. N Engl J Med 1996; 335:631.
  28. Liang TJ, Jeffers L, Reddy RK, et al. Fulminant or subfulminant non-A, non-B viral hepatitis: the role of hepatitis C and E viruses. Gastroenterology 1993; 104:556.
  29. Poddar U, Thapa BR, Prasad A, Singh K. Changing spectrum of sporadic acute viral hepatitis in Indian children. J Trop Pediatr 2002; 48:210.
  30. Hamid SS, Jafri SM, Khan H, et al. Fulminant hepatic failure in pregnant women: acute fatty liver or acute viral hepatitis? J Hepatol 1996; 25:20.
  31. Khuroo MS, Teli MR, Skidmore S, et al. Incidence and severity of viral hepatitis in pregnancy. Am J Med 1981; 70:252.
  32. Verma A, Dhawan A, Zuckerman M, et al. Neonatal herpes simplex virus infection presenting as acute liver failure: prevalent role of herpes simplex virus type I. J Pediatr Gastroenterol Nutr 2006; 42:282.
  33. Härmä M, Höckerstedt K, Lautenschlager I. Human herpesvirus-6 and acute liver failure. Transplantation 2003; 76:536.
  34. Tung J, Hadzic N, Layton M, et al. Bone marrow failure in children with acute liver failure. J Pediatr Gastroenterol Nutr 2000; 31:557.
  35. Karetnyi YV, Beck PR, Markin RS, et al. Human parvovirus B19 infection in acute fulminant liver failure. Arch Virol 1999; 144:1713.
  36. Lee WM, Brown KE, Young NS, et al. Brief report: no evidence for parvovirus B19 or hepatitis E virus as a cause of acute liver failure. Dig Dis Sci 2006; 51:1712.
  37. Phillips MJ, Blendis LM, Poucell S, et al. Syncytial giant-cell hepatitis. Sporadic hepatitis with distinctive pathological features, a severe clinical course, and paramyxoviral features. N Engl J Med 1991; 324:455.
  38. Listernick R. Liver failure in a 2-day-old infant. Pediatr Ann 2004; 33:10.
  39. Lo JO, Harrison RA, Hunter AJ. Syphilitic hepatitis resulting in fulminant hepatic failure requiring liver transplantation. J Infect 2007; 54:e115.
  40. Stevens FM, McLoughlin RM. Is coeliac disease a potentially treatable cause of liver failure? Eur J Gastroenterol Hepatol 2005; 17:1015.
  41. Umemura T, Tanaka E, Ostapowicz G, et al. Investigation of SEN virus infection in patients with cryptogenic acute liver failure, hepatitis-associated aplastic anemia, or acute and chronic non-A-E hepatitis. J Infect Dis 2003; 188:1545.
  42. James LP, Alonso EM, Hynan LS, et al. Detection of acetaminophen protein adducts in children with acute liver failure of indeterminate cause. Pediatrics 2006; 118:e676.
  43. Rivera-Penera T, Moreno J, Skaff C, et al. Delayed encephalopathy in fulminant hepatic failure in the pediatric population and the role of liver transplantation. J Pediatr Gastroenterol Nutr 1997; 24:128.
  44. Lee WS, McKiernan P, Kelly DA. Etiology, outcome and prognostic indicators of childhood fulminant hepatic failure in the United kingdom. J Pediatr Gastroenterol Nutr 2005; 40:575.
  45. Stravitz RT, Lefkowitch JH, Fontana RJ, et al. Autoimmune acute liver failure: proposed clinical and histological criteria. Hepatology 2011; 53:517.