Abstract
Chronic liver disease (CLD) in children is more diverse compared to adults with respect to the etiology, progression and response to therapy. After history and clinical examination, the first step is to confirm the presence of CLD with basic blood investigations and ultrasonography. Markers of portal hypertension are splenomegaly, increased portal vein diameter, thrombocytopenia and presence of varices on endoscopy. The next step is to evaluate the etiology of CLD which will depend on the age of the child and needs targeted investigations as metabolic and inherited causes predominate in early childhood. CLD progression ought to be monitored regularly and several non-invasive markers are available but they have to be evaluated further in children. Since CLD progresses, complications have to be detected early not only to initiate appropriate treatment but also to prognosticate.
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References
Tsuchida T, Friedman SL. Mechanisms of hepatic stellate cell activation. Nat Rev Gastroenterol Hepatol. 2017;14:397–411.
European Association for the Study of the Liver. Electronic address: easloffice@easloffice.eu; European Association for the Study of the Liver. EASL Clinical Practice Guidelines for the management of patients with decompensated cirrhosis. J Hepatol. 2018;69:406–60.
Fawaz R, Baumann U, Ekong U, et al. Guideline for the evaluation of cholestatic jaundice in infants: Joint recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition. J Pediatr Gastroenterol Nutr. 2017;64:154–68.
Thapa BR, Walia A. Liver function tests and their interpretation. Indian J Pediatr. 2007;74:663–71.
Simko V. Alkaline phosphatases in biology and medicine. Dig Dis. 1991;9:189–93.
Tissières P, Chevret L, Debray D, Devictor D. Fulminant Wilson’s disease in children: Appraisal of a critical diagnosis. Pediatr Crit Care Med. 2003;4:338–43.
Alissa FT, Jaffe R, Shneider BL. Update on progressive familial intrahepatic cholestasis. J Pediatr Gastroenterol Nutr. 2008;46:241–52.
Fagundes ED, Ferreira AR, Roquete ML, et al. Clinical and laboratory predictors of esophageal varices in children and adolescents with portal hypertension syndrome. J Pediatr Gastroenterol Nutr. 2008;46:178–83.
Gatselis NK, Zachou K, Koukoulis GK, Dalekos GN. Autoimmune hepatitis, one disease with many faces: Etiopathogenetic, clinico-laboratory and histological characteristics. World J Gastroenterol. 2015;21:60–83.
Friedman SF, Martin P, Munoz JS. Laboratory evaluation of the patient with liver disease. Hepatology, A Textbook of Liver Disease. Philedelphia: Saunders Publication. 2003. p. 661–709.
Roberts LN, Bernal W. Incidence of bleeding and thrombosis in patients with liver disease. Semin Thromb Hemost. 2020;46:656–64.
O’Leary JG, Greenberg CS, Patton HM, Caldwell SH. AGA clinical practice update: Coagulation in cirrhosis. Gastroenterology. 2019;157:34-43.e1.
Kawada PS, Bruce A, Massicotte P, Bauman M, Yap J. Coagulopathy in children with liver disease. J Pediatr Gastroenterol Nutr. 2017;65:603–7.
Sardar A Sr, Parkash A, Merchant AA, Qamar B, Ayub F, Zehravi S. Etiology in children presented with chronic liver disease in a tertiary care hospital. Cureus. 2022;14:e25570.
Kamath BM, Baker A, Houwen R, Todorova L, Kerkar N. Systematic Review: The epidemiology, natural history, and burden of Alagille syndrome. J Pediatr Gastroenterol Nutr. 2018;67:148–56.
Chinsky JM, Singh R, Ficicioglu C, et al. Diagnosis and treatment of tyrosinemia type I: A US and Canadian consensus group review and recommendations. Genet Med. 2017;19:PMID:28771246.
Socha P, Janczyk W, Dhawan A, et al. Wilson’s disease in children: A position paper by the Hepatology Committee of the European Society for Paediatric Gastroenterology, Hepatology and Nutrition. J Pediatr Gastroenterol Nutr. 2018;66:334–44.
Hennes EM, Zeniya M, Czaja AJ, et al. International Autoimmune Hepatitis Group. Simplified criteria for the diagnosis of autoimmune hepatitis. Hepatology. 2008;48:169–76.
Mieli-Vergani G, Vergani D, Baumann U, Czubkowski P, Debray D, Dezsofi A. Diagnosis and management of pediatric autoimmune liver disease: ESPGHAN Hepatology Committee Position Statement. J Pediatr Gastroenterol Nutr. 2018;66:345–60.
Singh SK, Sen Sarma M, Yadav R, et al. Prognostic scoring systems and outcome of endovascular radiological intervention of chronic Budd-Chiari syndrome in children. Liver Int. 2018;38:1308–15.
Vos MB, Abrams SH, Barlow SE, Caprio S, Daniels SR, Kohli R. NASPGHAN clinical practice guideline for the diagnosis and treatment of nonalcoholic fatty liver disease in children: Recommendations from the Expert Committee on NAFLD (ECON) and the North American Society of Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN). J Pediatr Gastroenterol Nutr. 2017;64:319–34.
Lasagni A, Cadamuro M, Morana G, Fabris L, Strazzabosco M. Fibrocystic liver disease: Novel concepts and translational perspectives. Transl Gastroenterol Hepatol. 2021;6:26.
Gordon-Walker TT, Bove K, Veldtman G. Fontan-associated liver disease: A review. J Cardiol. 2019;74:223–32.
Maruyama H, Yokosuka O. Pathophysiology of portal hypertension and esophageal varices. Int J Hepatol. 2012;2012:895787.
de Franchis R. VI Faculty B. Expanding consensus in portal hypertension: Report of the Baveno VI Consensus Workshop: Stratifying risk and individualizing care for portal hypertension. J Hepatol. 2015;63:743–52.
Ghosh T, Banerjee M, Basu S, et al. Assessment of normal portal vein diameter in children. Trop Gastroenterol. 2014;35:79–84.
Grammatikopoulos T, McKiernan PJ, Dhawan A. Portal hypertension and its management in children. Arch Dis Child. 2018;103:186–91.
Poddar U, Shava U, Yachha SK, et al. β-Blocker therapy ameliorates hypersplenism due to portal hypertension in children. Hepatol Int. 2015;9:447–53.
Salvatore V, Borghi A, Peri E, et al. Relationship between hepatic haemodynamics assessed by Doppler ultrasound and liver stiffness. Dig Liver Dis. 2012;44:154–9.
Ravindranath A, Sen Sarma M, Yachha SK, et al. Outcome of portosystemic shunt surgery on pre-existing cholangiopathy in children with extrahepatic portal vein obstruction. J Hepatobiliary Pancreat Sci. 2020;27:141–8.
Ferah O, Akbulut A, Acik ME, et al. Scoring systems and postoperative outcomes in pediatric liver transplantation. Transplant Proc. 2019;51:2430–3.
Sutton H, Dhawan A, Grammatikopoulos T. Non-invasive markers of portal hypertension: Appraisal of adult experience and potential utilisation in children. J Pediatr Gastroenterol Nutr. 2018;66:559–69.
Chongsrisawat V, Vejapipat P, Siripon N, et al. Transient elastography for predicting esophageal/gastric varices in children with biliary atresia. BMC Gastroenterol. 2011;11:41.
Colecchia A, Di Biase AR, Scaioli E, et al. Non-invasive methods can predict oesophageal varices in patients with biliary atresia after a Kasai procedure. Dig Liver Dis. 2011;43:659–63.
Goldschmidt I, Brauch C, Poynard T, et al. Spleen stiffness measurement by transient elastography to diagnose portal hypertension in children. J Pediatr Gastroenterol Nutr. 2014;59:197–203.
Duche M, Ducot B, Tournay E, et al. Prognostic value of endoscopy in children with biliary atresia at risk for early development of varices and bleeding. Gastroenterology. 2010;139:1952–60.
Dhawan A, Taylor RM, Cheeseman P, De Silva P, Katsiyiannakis L, Mieli-Vergani G. Wilson's disease in children: 37-year experience and revised King's score for liver Wilson's disease in children: 37-year experience and revised King's score for liver transplantation. Liver Transpl. 2005;11:441-8.
Mannucci PM. Abnormal hemostasis tests and bleeding in chronic liver disease: Are they related? No. J Thromb Haemost. 2006;4:721–3.
Runyon BA, Montano AA, Akriviadis EA, Antillon MR, Irving MA, McHutchison JG. The serum-ascites albumin gradient is superior to the exudate-transudate concept in the differential diagnosis of ascites. Ann Intern Med. 1992;117:215–20.
Bolia R, Srivastava A, Marak R, Yachha SK, Poddar U. Prevalence and impact of bacterial infections in children with liver disease- A prospective study. J Clin Exp Hepatol. 2018;8:35–41.
Rimola A, Garcia-Tsao G, Navasa M, et al. Diagnosis, treatment and prophylaxis of spontaneous bacterial peritonitis. A consensus document. Int Ascites Club J Hepatol. 2000;32:142–53.
Dhiman RK, Kurmi R, Thumburu KK, et al. Diagnosis and prognostic significance of minimal hepatic encephalopathy in patients with cirrhosis of liver. Dig Dis Sci. 2010;55:2381–90.
Vilstrup H, Amodio P, Bajaj J, et al. Hepatic encephalopathy in chronic liver disease: 2014 Practice Guideline by the American Association for the Study of Liver Diseases and the European Association for the Study of the Liver. Hepatology. 2014;60:715–35.
Ginès P, Guevara M. Hyponatremia in cirrhosis: Pathogenesis, clinical significance, and management. Hepatology. 2008;48:1002–10.
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Ravindranath, A., Yachha, S.K. An Approach to Investigations of Chronic Liver Disease. Indian J Pediatr 91, 262–269 (2024). https://doi.org/10.1007/s12098-023-04751-1
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DOI: https://doi.org/10.1007/s12098-023-04751-1