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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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 . 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 .
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 .
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 . 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 .
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.
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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.