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Advances in Liver Failure and Management

  • Moreshwar Desai
  • Ayse Akcan-ArikanEmail author
Chapter

Abstract

Acute liver failure (ALF) and acute-on-chronic liver failure (ACLF) in children are rare but catastrophic clinical conditions with a high mortality and morbidity. Massive hepatocyte necrosis leads to profound impairment in synthetic, excretory, and detoxifying ability of the liver and leads to coagulopathy, jaundice, and encephalopathy. Circulating inflammatory mediators and toxins lead to multi-organ failure and death. As there are yet no treatment options for reversing or halting hepatocellular necrosis, current therapies focus more on supporting the failing organs, while the liver either spontaneously regenerates or child undergoes a liver transplant. Continuous renal replacement therapy (CRRT) forms one of such essential pillars of support implemented to successfully manage a critically ill child with ALF or ACLF. CRRT is either used alone or in combination of other blood purification modalities to attenuate the deleterious effects of a failing liver in the host. This chapter discusses the pathophysiology of liver failure, indications, benefits, and challenges of CRRT in pediatric liver failure.

References

  1. 1.
    Arroyo V, Jalan R. Acute-on-chronic liver failure: definition, diagnosis, and clinical characteristics. Semin Liver Dis. 2016;36(2):109–16.CrossRefGoogle Scholar
  2. 2.
    Sarin SK, Choudhury A. Acute-on-chronic liver failure: terminology, mechanisms and management. Nat Rev Gastroenterol Hepatol. 2016;13(3):131–49.CrossRefGoogle Scholar
  3. 3.
    Squires RH. End-stage liver disease in children. Curr Treat Options Gastroenterol. 2001;4(5):409–21.CrossRefGoogle Scholar
  4. 4.
    Squires RH Jr, Shneider BL, Bucuvalas J, Alonso E, Sokol RJ, Narkewicz MR, et al. Acute liver failure in children: the first 348 patients in the pediatric acute liver failure study group. J Pediatr. 2006;148(5):652–8.CrossRefGoogle Scholar
  5. 5.
    Miloh T, Desai MS. Chapter 419: acute liver failure. In: Rudolph’s pediatrics. 23rd ed. New York: McGraw Hill; 2018.Google Scholar
  6. 6.
    Bernal W, Wendon J. Acute liver failure. N Engl J Med. 2013;369(26):2525–34.CrossRefGoogle Scholar
  7. 7.
    Bacchetta J, Mekahli D, Rivet C, Demede D, Leclerc AL. Pediatric combined liver-kidney transplantation: a 2015 update. Curr Opin Organ Transplant. 2015;20(5):543–9.CrossRefGoogle Scholar
  8. 8.
    Cheng XS, Tan JC, Kim WR. Management of renal failure in end-stage liver disease: a critical appraisal. Liver Transpl. 2016;22(12):1710–9.CrossRefGoogle Scholar
  9. 9.
    Maiwall R, Sarin SK, Moreau R. Acute kidney injury in acute on chronic liver failure. Hepatol Int. 2016;10(2):245–57.CrossRefGoogle Scholar
  10. 10.
    Wong F. Acute kidney injury in liver cirrhosis: new definition and application. Clin Mol Hepatol. 2016;22(4):415–22.CrossRefGoogle Scholar
  11. 11.
    Bucsics T, Krones E. Renal dysfunction in cirrhosis: acute kidney injury and the hepatorenal syndrome. Gastroenterol Rep (Oxf). 2017;5(2):127–37.CrossRefGoogle Scholar
  12. 12.
    Davenport A. Continuous renal replacement therapy for liver disease. Hemodial Int. 2003;7(4):348–52.CrossRefGoogle Scholar
  13. 13.
    Deep A, Saxena R, Jose B. Acute kidney injury in children with chronic liver disease. Pediatr Nephrol. 2018.  https://doi.org/10.1007/s00467-018-3893-7.CrossRefGoogle Scholar
  14. 14.
    Krag A, Bendtsen F, Henriksen JH, Moller S. Low cardiac output predicts development of hepatorenal syndrome and survival in patients with cirrhosis and ascites. Gut. 2010;59(1):105–10.CrossRefGoogle Scholar
  15. 15.
    Krag A, Bendtsen F, Burroughs AK, Moller S. The cardiorenal link in advanced cirrhosis. Med Hypotheses. 2012;79(1):53–5.CrossRefGoogle Scholar
  16. 16.
    Durand F, Graupera I, Gines P, Olson JC, Nadim MK. Pathogenesis of hepatorenal syndrome: implications for therapy. Am J Kidney Dis. 2016;67(2):318–28.CrossRefGoogle Scholar
  17. 17.
    Francoz C, Nadim MK, Durand F. Kidney biomarkers in cirrhosis. J Hepatol. 2016;65(4):809–24.CrossRefGoogle Scholar
  18. 18.
    Schaefer B, Ujszaszi A, Schaefer S, Heckert KH, Schaefer F, Schmitt CP. Safety and efficacy of tandem hemodialysis and plasma exchange in children. Clin J Am Soc Nephrol. 2014;9(9):1563–70.CrossRefGoogle Scholar
  19. 19.
    Singer AL, Olthoff KM, Kim H, Rand E, Zamir G, Shaked A. Role of plasmapheresis in the management of acute hepatic failure in children. Ann Surg. 2001;234(3):418–24.CrossRefGoogle Scholar
  20. 20.
    Deep A, Stewart CE, Dhawan A, Douiri A. Effect of continuous renal replacement therapy on outcome in pediatric acute liver failure. Crit Care Med. 2016;44(10):1910–9.CrossRefGoogle Scholar
  21. 21.
    Durand F, Olson JC, Nadim MK. Renal dysfunction and cirrhosis. Curr Opin Crit Care. 2017;23(6):457–62.CrossRefGoogle Scholar
  22. 22.
    Karvellas CJ, Durand F, Nadim MK. Acute kidney injury in cirrhosis. Crit Care Clin. 2015;31(4):737–50.CrossRefGoogle Scholar
  23. 23.
    Hadjihambi A, Arias N, Sheikh M, Jalan R. Hepatic encephalopathy: a critical current review. Hepatol Int. 2018;12(Suppl 1):135–47.CrossRefGoogle Scholar
  24. 24.
    Romero-Gomez M, Montagnese S, Jalan R. Hepatic encephalopathy in patients with acute decompensation of cirrhosis and acute-on-chronic liver failure. J Hepatol. 2015;62(2):437–47.CrossRefGoogle Scholar
  25. 25.
    Polson J, Lee WM. AASLD position paper: the management of acute liver failure. Hepatology. 2005;41(5):1179–97.CrossRefGoogle Scholar
  26. 26.
    Cardoso FS, Gottfried M, Tujios S, Olson JC, Karvellas CJ. Continuous renal replacement therapy is associated with reduced serum ammonia levels and mortality in acute liver failure. Hepatology. 2017;67(2):711–20.CrossRefGoogle Scholar
  27. 27.
    Chevret L, Durand P, Lambert J, Essouri S, Balu L, Devictor D, et al. High-volume hemofiltration in children with acute liver failure*. Pediatr Crit Care Med. 2014;15(7):e300–5.CrossRefGoogle Scholar
  28. 28.
    Kim JM, Jo YY, Na SW, Kim SI, Choi YS, Kim NO, et al. The predictors for continuous renal replacement therapy in liver transplant recipients. Transplant Proc. 2014;46(1):184–91.CrossRefGoogle Scholar
  29. 29.
    Alam S, Lal BB. Metabolic liver diseases presenting as acute liver failure in children. Indian Pediatr. 2016;53(8):695–701.CrossRefGoogle Scholar
  30. 30.
    Gupta S, Fenves AZ, Hootkins R. The role of RRT in hyperammonemic patients. Clin J Am Soc Nephrol. 2016;11(10):1872–8.CrossRefGoogle Scholar
  31. 31.
    Banares R, Nevens F, Larsen FS, Jalan R, Albillos A, Dollinger M, et al. Extracorporeal albumin dialysis with the molecular adsorbent recirculating system in acute-on-chronic liver failure: the RELIEF trial. Hepatology. 2013;57(3):1153–62.CrossRefGoogle Scholar
  32. 32.
    Saliba F, Samuel D. Artificial liver support: a real step forward. Minerva Med. 2015;106(1):35–43.PubMedGoogle Scholar
  33. 33.
    Saliba F, Camus C, Durand F, Mathurin P, Letierce A, Delafosse B, et al. Albumin dialysis with a noncell artificial liver support device in patients with acute liver failure: a randomized, controlled trial. Ann Intern Med. 2013;159(8):522–31.CrossRefGoogle Scholar
  34. 34.
    Saliba F. The molecular adsorbent recirculating system (MARS) in the intensive care unit: a rescue therapy for patients with hepatic failure. Crit Care. 2006;10(1):118.CrossRefGoogle Scholar
  35. 35.
    Schaefer B, Schaefer F, Engelmann G, Meyburg J, Heckert KH, Zorn M, et al. Comparison of molecular adsorbents recirculating system (MARS) dialysis with combined plasma exchange and haemodialysis in children with acute liver failure. Nephrol Dial Transplant. 2011;26(11):3633–9.CrossRefGoogle Scholar
  36. 36.
    Schaefer B, Schmitt CP. The role of molecular adsorbent recirculating system dialysis for extracorporeal liver support in children. Pediatr Nephrol. 2013;28(9):1763–9.CrossRefGoogle Scholar
  37. 37.
    Lexmond WS, Van Dael CM, Scheenstra R, Goorhuis JF, Sieders E, Verkade HJ, et al. Experience with molecular adsorbent recirculating system treatment in 20 children listed for high-urgency liver transplantation. Liver Transpl. 2015;21(3):369–80.CrossRefGoogle Scholar
  38. 38.
    Akcan AA, Srivaths P, Himes RW, Tufan PN, Lam F, Nguyen T, et al. Hybrid extracorporeal therapies as a bridge to pediatric liver transplantation. Pediatr Crit Care Med. 2018;19(7):e342–9.Google Scholar
  39. 39.
    Habib M, Roberts LN, Patel RK, Wendon J, Bernal W, Arya R. Evidence of rebalanced coagulation in acute liver injury and acute liver failure as measured by thrombin generation. Liver Int. 2014;34(5):672–8.CrossRefGoogle Scholar
  40. 40.
    Goonasekera CD, Wang J, Bunchman TE, Deep A. Factors affecting circuit life during continuous renal replacement therapy in children with liver failure. Ther Apher Dial. 2015;19(1):16–22.CrossRefGoogle Scholar
  41. 41.
    Chadha V, Garg U, Warady BA, Alon US. Citrate clearance in children receiving continuous venovenous renal replacement therapy. Pediatr Nephrol. 2002;17(10):819–24.CrossRefGoogle Scholar
  42. 42.
    Elhanan N, Skippen P, Nuthall G, Krahn G, Seear M. Citrate anticoagulation in pediatric continuous venovenous hemofiltration. Pediatr Nephrol. 2004;19(2):208–12.CrossRefGoogle Scholar
  43. 43.
    Brophy PD, Somers MJ, Baum MA, Symons JM, McAfee N, Fortenberry JD, et al. Multi-centre evaluation of anticoagulation in patients receiving continuous renal replacement therapy (CRRT). Nephrol Dial Transplant. 2005;20(7):1416–21.CrossRefGoogle Scholar
  44. 44.
    Rodriguez K, Srivaths PR, Tal L, Watson MN, Riley AA, Himes RW, et al. Regional citrate anticoagulation for continuous renal replacement therapy in pediatric patients with liver failure. PLoS One. 2017;12(8):e0182134.CrossRefGoogle Scholar
  45. 45.
    Santiago MJ, Lopez-Herce J, Urbano J, Solana MJ, Del CJ, Ballestero Y, et al. Complications of continuous renal replacement therapy in critically ill children: a prospective observational evaluation study. Crit Care. 2009;13(6):R184.CrossRefGoogle Scholar
  46. 46.
    Thorat A, Jeng LB. Management of renal dysfunction in patients with liver cirrhosis: role of pretransplantation hemodialysis and outcomes after liver transplantation. Semin Vasc Surg. 2016;29(4):227–35.CrossRefGoogle Scholar

Copyright information

© Springer Nature Singapore Pte Ltd. 2019

Authors and Affiliations

  1. 1.Department of Pediatrics, Section of Pediatric Critical CareBaylor College of Medicine, Texas Children’s HospitalHoustonUSA
  2. 2.Liver Intensive Care UnitTexas Children’s HospitalHoustonUSA
  3. 3.Department of Pediatrics, Sections of Critical Care and NephrologyBaylor College of Medicine, Texas Children’s HospitalHoustonUSA
  4. 4.Extracorporeal Liver SupportTexas Children’s HospitalHoustonUSA
  5. 5.Critical Care NephrologyTexas Children’s HospitalHoustonUSA

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