Book contents
- Liver Disease in Children
- Liver Disease in Children
- Copyright page
- Contents
- Contributors
- Preface
- Section I Pathophysiology of Pediatric Liver Disease
- Chapter 1 Liver Development
- Chapter 2 Functional Development of the Liver
- Chapter 3 Mechanisms of Bile Formation and the Pathogenesis of Cholestasis
- Chapter 4 Acute Liver Failure in Children
- Chapter 5 Cirrhosis and Chronic Liver Failure in Children
- Chapter 6 Portal Hypertension in Children
- Chapter 7 Laboratory Assessment of Liver Function and Injury in Children
- Section II Cholestatic Liver Disease
- Section III Hepatitis and Immune Disorders
- Section IV Metabolic Liver Disease
- Section V Other Considerations and Issues in Pediatric Hepatology
- Index
- References
Chapter 5 - Cirrhosis and Chronic Liver Failure in Children
from Section I - Pathophysiology of Pediatric Liver Disease
Published online by Cambridge University Press: 19 January 2021
Book contents
- Liver Disease in Children
- Liver Disease in Children
- Copyright page
- Contents
- Contributors
- Preface
- Section I Pathophysiology of Pediatric Liver Disease
- Chapter 1 Liver Development
- Chapter 2 Functional Development of the Liver
- Chapter 3 Mechanisms of Bile Formation and the Pathogenesis of Cholestasis
- Chapter 4 Acute Liver Failure in Children
- Chapter 5 Cirrhosis and Chronic Liver Failure in Children
- Chapter 6 Portal Hypertension in Children
- Chapter 7 Laboratory Assessment of Liver Function and Injury in Children
- Section II Cholestatic Liver Disease
- Section III Hepatitis and Immune Disorders
- Section IV Metabolic Liver Disease
- Section V Other Considerations and Issues in Pediatric Hepatology
- Index
- References
Summary
The word “cirrhosis” comes from the Greek kirrhos, meaning yellowish, tawny, and describes the gross pathology of the diseased liver. Since the late 1980s, however, clinicians have used the definition provided by the World Health Organization, which defines cirrhosis as a diffuse liver process where fibrosis has resulted in a conversion of the liver architecture into structurally abnormal nodules [1]. This distortion of liver architecture leads to compression of hepatic vascular and biliary structures, creating a further imbalance in the delivery of nutrients, oxygen, and metabolites. Even after the original insult has been controlled or stopped, the cirrhotic state persists. Although the causes of chronic liver disease encompass a wide spectrum of pathophysiological processes, cirrhosis is a common outcome [2].
- Type
- Chapter
- Information
- Liver Disease in Children , pp. 58 - 73Publisher: Cambridge University PressPrint publication year: 2021
References
Garcia-Tsao, G, Friedman, S, Iredale, J, Pinzani, M. Now there are many (stages) where before there was one: in search of a pathophysiological classification of cirrhosis. Hepatology 2010;51(4):1445–9.CrossRefGoogle ScholarPubMed
Pinzani, M, Rombouts, K. Liver fibrosis: from the bench to clinical targets. Dig Liver Dis. 2004;36(4):231–42.Google Scholar
Hernandez-Gea, V, Friedman, SL. Pathogenesis of liver fibrosis. Annu Rev Pathol 2011;6(1):425–56.CrossRefGoogle ScholarPubMed
Wells, RG. Cellular sources of extracellular matrix in hepatic fibrosis. Clin Liver Dis 2008;12(4):759–68.CrossRefGoogle ScholarPubMed
Wells, RG. The role of matrix stiffness in regulating cell behavior. Hepatology 2008;47(4):1394–400.Google Scholar
Yoon, YJ, Friedman, SL, Lee, YA. Antifibrotic therapies: where are we now? Semin Liver Dis 2016;36(1):87–98.CrossRefGoogle ScholarPubMed
Manka, P, Zeller, A, Syn, W-K. Fibrosis in chronic liver disease: an update on diagnostic and treatment modalities. Drugs 2019;79(9):903–27.Google Scholar
Riehle, KJ, Dan, YY, Campbell, JS, Fausto, N. New concepts in liver regeneration. J Gastroenterol Hepatol 2011;26 Suppl 1(2):203–12.Google Scholar
Satapathy, SK, Bernstein, D. Dermatologic disorders and the liver. Clin Liver Dis 2011;15(1):165–82.Google Scholar
Sussman, NL, Kochar, R, Fallon, MB. Pulmonary complications in cirrhosis. Curr Opin Organ Transplant 2011;16(3):281–8.CrossRefGoogle ScholarPubMed
Swanson, KL, Wiesner, RH, Krowka, MJ. Natural history of hepatopulmonary syndrome: impact of liver transplantation. Hepatology 2005;41(5):1122–9.Google Scholar
Ridaura-Sanz, C, Mejía-Hernández, C, López-Corella, E. Portopulmonary hypertension in children. A study in pediatric autopsies. Arch Med Res 2009;40(7):635–9.Google Scholar
Mukhtar, NA, Fix, OK. Portopulmonary hypertension. J Clin Gastroenterol 2011;45(8):703–10.CrossRefGoogle ScholarPubMed
Savale, L, O’Callaghan, DS, Magnier, R, Le Pavec, J, Hervé, P, Jaïs, X, et al. Current management approaches to portopulmonary hypertension. Int J Clin Pract Suppl 2011;65(169):11–18.Google Scholar
Laving, A, Khanna, A, Rubin, L, Ing, F, Dohil, R, Lavine, JE. Successful liver transplantation in a child with severe portopulmonary hypertension treated with epoprostenol. J Pediatr Gastroenterol Nutr 2005;41(4):466–8.Google Scholar
Maheshwari, A, Thuluvath, PJ. Endocrine diseases and the liver. Clin Liver Dis 2011;15(1):55–67.Google Scholar
Foerster, BR, Conklin, LS, Petrou, M, Barker, PB, Schwarz, KB. Minimal hepatic encephalopathy in children: evaluation with proton MR spectroscopy. Am J Neuroradiol 2009;30(8):1610–13.Google Scholar
Bonnel, AR, Bunchorntavakul, C, Reddy, KR. Immune dysfunction and infections in patients with cirrhosis. Clin Gastroenterol Hepatol 2011;9(9):727–38.Google Scholar
Utterson, EC, Shepherd, RW, Sokol, RJ, Bucuvalas, J, Magee, JC, McDiarmid, SV, et al. Biliary atresia: clinical profiles, risk factors, and outcomes of 755 patients listed for liver transplantation. J Pediatr 2005;147(2):180–5.Google Scholar
Wibaux, C, Legroux-Gerot, I, Dharancy, S, Boleslawski, E, Declerck, N, Canva, V, et al. Assessing bone status in patients awaiting liver transplantation. Joint Bone Spine 2011;78(4):387–91.Google Scholar
Schilsky, ML, Ala, A. Genetic testing for Wilson disease: availability and utility. Curr Gastroenterol Rep 2010;12(1):57–61.CrossRefGoogle ScholarPubMed
Ohwada, S, Kawate, S, Hamada, K, Yamada, T, Sunose, Y, Tsutsumi, H, et al. Perioperative real-time monitoring of indocyanine green clearance by pulse spectrophotometry predicts remnant liver functional reserve in resection of hepatocellular carcinoma. Br J Surg 2006;93(3):339–46.Google Scholar
Blüthner, E, Bednarsch, J, Pape, U-F, Karber, M, Maasberg, S, Gerlach, UA, et al. Advanced liver function assessment in patients with intestinal failure on long-term parenteral nutrition. Clin Nutr 2020;39(2):540–7.Google Scholar
Kamath, PS, Wiesner, RH, Malinchoc, M, Kremers, W, Therneau, TM, Kosberg, CL, et al. A model to predict survival in patients with end-stage liver disease. Hepatology 2001;33(2):464–70.Google Scholar
McDiarmid, SV, Anand, R, Lindblad, AS. Principal Investigators and Institutions of the Studies of Pediatric Liver Transplantation (SPLIT) Research Group. Development of a pediatric end-stage liver disease score to predict poor outcome in children awaiting liver transplantation. Transplantation 2002;74(2):173–81.CrossRefGoogle ScholarPubMed
Chang, C-CH, Bryce, CL, Shneider, BL, Yabes, JG, Ren, Y, Zenarosa, GL, et al. Accuracy of the pediatric end-stage liver disease score in estimating pretransplant mortality among pediatric liver transplant candidates. JAMA Pediatr 2018;172(11):1070–7.Google Scholar
Tripodi, A, Mannucci, PM. The coagulopathy of chronic liver disease. N Engl J Med 2011;365(2):147–56.CrossRefGoogle ScholarPubMed
Giefer, MJ, Murray, KF, Colletti, RB. Pathophysiology, diagnosis, and management of pediatric ascites. J Pediatr Gastroenterol Nutr 2011;52(5):503–13.Google Scholar
Bertino, F, Hawkins, CM, Shivaram, G, Gill, AE, Lungren, MP, Reposar, A, et al. Technical feasibility and clinical effectiveness of transjugular intrahepatic portosystemic shunt creation in pediatric and adolescent patients. J Vasc Interv Radiol 2019;30(2):178–86.Google Scholar
Ghannam, JS, Cline, MR, Hage, AN, Chick, JFB, Srinivasa, RN, Dasika, NL, et al. Technical success and outcomes in pediatric patients undergoing transjugular intrahepatic portosystemic shunt placement: a 20-year experience. Pediatr Radiol 2019;49(1):128–35.Google Scholar
Slowik, V, Monroe, EJ, Friedman, SD, Hsu, EK, Horslen, S. Pressure gradients, laboratory changes, and outcomes with transjugular intrahepatic portosystemic shunts in pediatric portal hypertension. Pediatric Transplantation 2019;23(3):e13387.Google Scholar
Koulaouzidis, A, Bhat, S, Saeed, AA. Spontaneous bacterial peritonitis. World J Gastroenterol 2009;15(9):1042–9.Google Scholar
Haghighat, M, Dehghani, SM, Alborzi, A, Imanieh, MH, Pourabbas, B, Kalani, M. Organisms causing spontaneous bacterial peritonitis in children with liver disease and ascites in Southern Iran. World J Gastroenterol 2006;12(36):5890–2.Google Scholar
Căruntu, FA, Benea, L. Spontaneous bacterial peritonitis: pathogenesis, diagnosis, treatment. J Gastrointestin Liver Dis 2006;15(1):51–6.Google Scholar
Vieira, SMG, Schwengber, FP, Melere, M, Ceza, MR, Souza, M, Kieling, CO. The first episode of spontaneous bacterial peritonitis is a threat event in children with end-stage liver disease. Eur J Gastroenterol Hepatol 2018;30(3):323–7.Google Scholar
Arroyo, V, Fernandez, J, Ginès, P. Pathogenesis and treatment of hepatorenal syndrome. Semin Liver Dis 2008;28(1):81–95.Google Scholar
Salerno, F, Gerbes, A, Ginès, P, Wong, F, Arroyo, V. Diagnosis, prevention and treatment of hepatorenal syndrome in cirrhosis. Gut 2007;56(9):1310–18.Google Scholar
Grangé, JD, Amiot, X. Nitric oxide and renal function in cirrhotic patients with ascites: from physiopathology to practice. Eur J Gastroenterol Hepatol 2004;16(6):567–70.Google Scholar
Rasaratnam, B, Kaye, D, Jennings, G, Dudley, F, Chin-Dusting, J. The effect of selective intestinal decontamination on the hyperdynamic circulatory state in cirrhosis. A randomized trial. Ann Intern Med 2003;139(3):186–93.CrossRefGoogle Scholar
Gonwa, TA, McBride, MA, Anderson, K, Mai, ML, Wadei, H, Ahsan, N. Continued influence of preoperative renal function on outcome of orthotopic liver transplant (OLTX) in the US: where will MELD lead us? Am J Transplant 2006;6(11):2651–9.Google Scholar
Capling, RK, Bastani, B. The clinical course of patients with type 1 hepatorenal syndrome maintained on hemodialysis. Ren Fail 2004;26(5):563–8.Google Scholar
Mas, A. Hepatic encephalopathy: from pathophysiology to treatment. Digestion 2006;73 Suppl 1(1):86–93.CrossRefGoogle ScholarPubMed
Stewart, CA, Smith, GE. Minimal hepatic encephalopathy. Nat Clin Pract Gastroenterol Hepatol 2007;4(12):677–85.CrossRefGoogle ScholarPubMed
Ross, BD, Jacobson, S, Villamil, F, Korula, J, Kreis, R, Ernst, T, et al. Subclinical hepatic encephalopathy: proton MR spectroscopic abnormalities. Radiology 1994;193(2):457–63.Google Scholar
Charlton, CP, Buchanan, E, Holden, CE, Preece, MA, Green, A, Booth, IW, et al. Intensive enteral feeding in advanced cirrhosis: reversal of malnutrition without precipitation of hepatic encephalopathy. Arch Dis Child 1992;67(5):603–7.Google Scholar
Morgan, MY, Blei, A, Grüngreiff, K, Jalan, R, Kircheis, G, Marchesini, G, et al. The treatment of hepatic encephalopathy. Metab Brain Dis 2007;22(3–4):389–405.CrossRefGoogle ScholarPubMed
Bass, NM, Mullen, KD, Sanyal, A, Poordad, F, Neff, G, Leevy, CB, et al. Rifaximin treatment in hepatic encephalopathy. N Engl J Med 2010;362(12):1071–81.Google Scholar