Hepatology and Transplant Hepatology: A Case Based Approach

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Share Give access Share full text access. Share full text access. Please review our Terms and Conditions of Use and check box below to share full-text version of article. Summary This chapter attempts to identify issues relating to the assessment of patients with chronic liver disease for orthotopic liver transplantation.

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Gastroenterology Update: A Case-Based Approach to Common GI Problems

Password Changed Successfully Your password has been changed. Returning user. Request Username Can't sign in? Moreover, most of the artificial liver assist devices help during hepatic encephalopathy but do not improve overall survival in ALF. A variety of such systems have been tested in non-randomized trials, but are not recommended outside clinical trials. The advent of successful liver transplantation has revolutionized management of children with MLD who fail to respond to conservative management. Galactosemia, HFI, tyrosinemia type 1 and urea cycle defects may not respond to medical therapy and dietary restrictions if diagnosed late, and in an emergency liver transplantation may prove to be life-saving.

MLD are the second most common indication for liver transplantation after biliary atresia [29]. UCD, alphaantitrypsin deficiency, cystic fibrosis, WD and tyrosinemia type 1 are the common MLD requiring liver transplantation in children. Post-transplant survival for children with MLD is comparable to those with other diseases with a better graft survival than those with other diseases [29].

A better outcome of liver transplantation in MLD could be attributed to the fact that many children with MLD underwent liver transplantation to correct an enzymatic defect, and did not have structural parenchymal liver disease. Liver transplantation has been successfully done in many cases of tyrosinemia, galactosemia, mitochondrio-pathies and UCD presenting as ALF [].

Liver transplantation is usually contraindicated in diseases with severe multisystemic involvement e. A rapid assessment of the severity of extrahepatic involvement in a child with mitochondrio-pathy and decompensating liver is mandatory, so as to take a decision about the usefulness of liver transplantation in such a case.

Suitability of heterozygous parents as donors is another important issue to be resolved. Although Wilson disease presenting with encephalo-pathy is invariably fatal and can be treated only by liver transplantation, the decision to list a child with this disorder without encephalopathy is very difficult [5,31]. However, doubts have been raised recently over the ability of this score to predict mortality without liver transplantation [33].

Survival is difficult to predict and continued investigations for predictors of outcome in Wilson disease are necessary. Hepatocyte transplantation is moderately successful for MLD presenting as ALF, as a bridge to liver transplantation [34]. Hepatocyte transplantation holds promise as an alternative to organ transplantation and numerous animal studies indicate that transplants of isolated liver cells can correct metabolic deficiencies of the liver.

Stem cell based technology is a new biotechnology approach to treat patients with MLD. Parents who have a child with MLD must undergo genetic couseling. The probability of the next sibling being affected from the disease should be explained, and prenatal testing and counseling should be offered where available. The parents must be explained about the nature of the illness and risk of occurrence in future pregnancies. Prenatal diagnosis of tyrosinemia is possible by analysis of succinylacetone in amniotic fluid supernatant and by assay of fumaryl acetoacetate hydrolase in cultured amniotic fluid cells or chorionic villus material [37].

Similarly, a GALT assay can be planned early for the next child of parents who already have a child suffering from galactosemia. Many of these conditions are potentially curable with dietary modifications or medications if recognized early.

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A high index of suspicion in presence of red flag symptoms and signs is need of the hour. A protocol-based approach will identify the etiology in most of the patients. Liver transplantation has markedly improved the outcome of MLD in children. Contributors : Both authors conceptualized the work, searched and reviewed the data. BBL prepared the first draft; SA: critically reviewed and revised the manuscript.

Both authors approved the final version. Acute liver failure in infants and young children in a specialized pediatric liver centre in India. Indian J Pediatr. Pediatric acute liver failure: Etiology, outcomes, and the role of serial pediatric end-stage liver disease scores. Pediatr Transplant. Acute liver failure in under two year-olds—are there markers of metabolic disease on admission? Ann Hepatol. Characterization and outcome of young infants with acute liver failure.

J Pediatr. Dhawan A. Etiology and prognosis of acute liver failure in children. Liver Transplant. Acute liver failure in children: The first patients in pediatric acute liver failure study group.

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Acute liver failure in infancy: A year experience of a pediatric liver transplantation center. Etiology and prognostic factors of acute liver failure in children. Indian Pediatr. Etiology, outcome and prognostic indicators of childhood fulminant hepatic failure in the United Kingdom. J Pediatr Gastroenterol Nutr. Neonatal liver failure- etiologies and management- state of the art.

Eur J Pediatr. Pattern of diagnostic evaluation for the causes of pediatric acute liver failure: an opportunity for quality improvement. Natural history and risk factors in fulminant hepatic failure. Arch Dis Child. Retrospective biochemical screening of fatty acid oxidation disorders in postmortem livers of cases of sudden death in the first year of life.

Clinical and molecular features of mitochondrial DNA depletion due to mutations in deoxyguanosine kinase. Hum Mutat. Screening for Wilson disease in acute liver failure: a comparison of currently available diagnostic tests. Eurpean Association for Study of Liver. J Hepatol. Lactulose vs polyethylene glycol —electrolyte solution for treatment of overt hepatic encephalopathy: The HELP randomized clinical trial. Non-absorbable disaccharides for hepatic encephalopathy: Systematic review of randomised trials.

Management of hepatic encephalopathy in the hospital. Mayo Clin Proc. Lactulose therapy in acute liver failure.

  1. Works of Mary Eleanor Wilkins Freeman.
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  3. Post-Liver Transplantation Management.
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  5. Hepatology and Transplant Hepatology: A Case Based Approach.
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  8. Sodium benzoate in the treatment of acute hepatic encephalopathy: A double-blind randomized trial. Treatment of inborn errors of urea synthesis: activation of alternative pathways of waste nitrogen synthesis and excretion. N Engl J Med. Comparison of Molecular Adsorbents Recirculating System MARS dialysis with combined plasma exchange and haemodialysis in children with acute liver failure.

    Nephrol Dial Transplant. Artificial and bioartificial support systems for liver failure. Cochrane Database Syst Rev. Molecular absorbent recirculating system therapy MARS in pediatric acute liver failure: A single center experience. Pediatr Nephrol. High-volume continuous venovenous hemofiltration as an effective therapy for acute management of inborn errors of meta-bolism in young children.

    Blood Purif. Continuous hemofiltration in the control of neonatal hyperammonemia: A year experience. Liver transplantation for pediatric metabolic diseases. Mol Genet Metab.

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    Liver transplantation in children with metabolic diseases: The studies of pediatric liver transplantation experience. Long-term outcome following pediatric liver transplantation for metabolic disorders. Factors that predict mortality in children with Wilson disease associated acute liver failure and comparison of Wilson disease specific prognostic indices. J Gastroenterol Hepatol. Current status of hepatocyte transplantation. Sokal EM. Treating inborn errors of liver metabolism with stem cells: Current clinical development.

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    J Inherit Metab Dis. Stem Cells. Recommendations for the management of tyrosinemia type 1.

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    Orphanet J Rare Dis. Pathophysiology In disorders such as galactosemia, tyrosinemia and Urea cycle defects, the pathogenesis of MLD can be attributed to a defect in the intermediary metabolic pathway leading to the accumulation of toxic metabolites formed in one of the preceding steps which leads to liver failure. Indian Pediatr ; 6 Older children.