Summary:Among the worldwide accepted indications for liver transplantation, inherited metabolic disorders play an increasing role. In some paediatric centres this indication runs second after extrahepatic biliary atresia.The aim of liver transplantation in inherited metabolic disorders is twofold: the first is to save a patient's life, the second is to accomplish phenotypic and functional cure of his disease. These aims may be achieved in disorders presenting with cirrhosis, hepatoma, life-threatening progression or failure of other organs with preserved liver function. The timing of liver transplantation has become easier with development of surgical techniques of reduced-size donor livers, These techniques enable the performance of liver transplantation with ABO blood group compatible organs of almost any size if indicated either by deterioration of liver function or impending complications such as hepatoma or life-threatening progression. In comparison with other indications such as extrahepatic biliary atresia, postnecrotic liver cirrhosis or acute liver failure, the results of transplantation in patients with inherited metabolic disorders seem to be better, reaching up to 78-95% actuarial 1-year survival rates. However, lifelong immunosuppressive therapy is necessary. This seems to be acceptable even in disorders with only partial liver function defects.Liver transplantation in inherited disorders of metabolism has a twofold aim. The first is to save the patient's life since the disorder may cause either acute or chronic liver failure, hepatoma or failure of other organs. The second aim is to cure the underlying metabolic defect. This aim may be achieved by phenotypic and functional cure; a genetic cure, however, is not possible (Esquivel et al., 1988). Nevertheless, phenotypic and functional cure enable a normal life style in children who otherwise would have died. In addition, from a scientific point of view, liver transplantation represents a new concept in clarifying the pathogenesis in hepatic-based metabolic disorders (Starzl, 1988a).When compared to conventional indications for liver transplantation, such as extrahepatic biliary atresia or postnecrotic cirrhosis, there are both similarities and major differences that can be recognized. As in extrahepatic biliary atresia, the most 604
Situs inversus is a rare anatomical abnormality that is often associated with multiple, complex malformations. In the past, patients with situs inversus were considered unsuitable candidates for transplantation or organ donation because associated visceral, and especially vascular, anomalies pose special technical difficulties. Recently, several cases of successful liver transplantation in recipients with situs inversus have been published using modified surgical techniques. This report reviews the literature and describes our own experience, including two liver graft recipients with complete and incomplete situs inversus, and one patient who underwent successful transplantation using a liver from a donor with situs inversus.
Situs inversus is a rare anatomical abnormality that is often associated with multiple, complex malformations. In the past, patients with situs inversus were considered unsuitable candidates for transplantation or organ donation because associated visceral, and especially vascular, anomalies pose special technical difficulties. Recently, several cases of successful liver transplantation in recipients with situs inversus have been published using modified surgical techniques. This report reviews the literature and describes our own experience, including two liver graft recipients with complete and incomplete situs inversus, and one patient who underwent successful transplantation using a liver from a donor with situs inversus.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.