What Is Different Between Pediatric and Adult Liver Transplantation?





Introduction


Clinical liver transplantation stems from the pioneering work of Professor Thomas Starlz in Denver, Colorado, where he attempted the first human liver transplantation on March 1, 1963. The recipient was a 3-year-old child with biliary atresia, who unfortunately died in the operating room with overwhelming intraabdominal bleeding. A few other attempts followed shortly after, but, in view of dismal results, the transplantation program was stopped and surgeons concentrated on the best strategies to solve the problems that they were encountering. Four years later, on July 23, 1967, the first successful liver transplantation was performed on a 19-month-old girl with liver cancer, who survived over a year. Pediatric liver transplantation, therefore, is positioned at the very birth of the hepatic transplantation surgical specialty, which now has become the standard treatment worldwide for a large range of serious or end-stage liver diseases in adults and children.


At the present time, the need for adult liver transplantation outnumbers greatly the need for pediatric liver transplantation. In the United Kingdom, the ratio between adult and pediatric liver transplants is around 9:1, and data from the National Health Service Blood and Transplant Annual Liver Transplant Report 2018 3 showed that out of 6830 deceased donor first liver-only transplantations done between 2008 and 2018, 6500 (90%) were performed in adult and 669 (10%) in pediatric patients. Importantly, the process of liver transplantation differs between adult and pediatric recipients; in this chapter, we will highlight the most important differences.


Pediatric Hepatology


At the onset of the development of pediatrics as a specialty, pediatricians were taught to treat the child as a whole entity, and specialties and subspecialties were not encouraged or considered useful. Pediatric gastroenterology started to separate from adult gastroenterology around the 1960s in recognition of the fact that children’s hepatic and gastrointestinal clinical problems had a peculiar nature and complexity. The discovery of metabolic liver diseases, bile acid synthesis defects, cryptogenic neonatal hepatitis syndromes, and the development of the Kasai portoenterostomy procedure for the treatment of biliary atresia, a condition up until then considered untreatable, were all factors that contributed to the foundation of the subspecialty of pediatric hepatology in the 1970s. The first two centers recognized for the diagnosis and management of pediatric liver disease were in Paris, led by Professor Daniel Alagille, and in London, led by Professor Alex Mowat. In the early 1980s, at King’s College Hospital, Mowat introduced the first supraregional tertiary referral center for pediatric hepatology. This rapidly became a world-class clinical and research unit, attracting fellows from all over the world and nurturing the development of similar units worldwide.


Also, in the early 1980s, the advent of new immunosuppression strategies upgraded liver transplantation from an experimental procedure to a therapeutic option. At that time, pediatric liver transplantation was only performed by Starzl in Pittsburgh. Pediatric liver transplantations started in the United Kingdom in 1983 initiated by the collaboration between Calne’s Transplant Unit in Cambridge and Williams’ and Mowat’s Liver Unit at King’s College Hospital in London. This collaboration laid the basis for the standardization of the procedure, indications, and care of pediatric patients.


Indications for liver transplantation in adults and children differ ( Table 2.1 ), and pediatric multidisciplinary teams should be involved throughout the child’s care, from the initial diagnosis and management to the preparation to transplantation when necessary.



Table 2.1

Common Pediatric and Adult Indications for Liver Transplantation

(Modified from Hackl C, Schlitt HJ, Melter M, et al. Current developments in pediatric liver transplantation. World J Hepatol. 2015;7(11):1509-1520.)




































Cause Pediatric Adult
Cholestatic disorders


  • Extrahepatic biliary atresia



  • Alagille syndrome



  • Progressive familial



  • intrahepatic cholestasis



  • Sclerosing cholangitis



  • Caroli syndrome



  • Intestinal failure-associated liver disease




  • Primary biliary cirrhosis



  • Primary sclerosing cholangitis



  • Secondary sclerosing cholangitis



  • Caroli syndrome

Metabolic liver disease, with cirrhosis


  • Alpha-1 antitrypsin deficiency



  • Wilson disease



  • Tyrosinemia



  • Neonatal hemochromatosis



  • Cystic fibrosis



  • Glycogenosis type IV



  • Primary bile acid synthesis defects




  • Alpha-1 antitrypsin deficiency



  • Hereditary hemochromatosis



  • Wilson disease



  • Cystic fibrosis



  • Glycogenosis type I

Metabolic liver disease, without cirrhosis


  • Hyperoxaluria



  • Crigler-Najjar syndrome



  • Urea cycle disorders



  • Familial hypercholesterolemia type IIA



  • Glycogenosis type IA



  • Hemophilia types A and B



  • Protein C deficiency



  • Wolman disease



  • Organic acidemias




  • Hyperoxaluria



  • Familial amyloidosis

Chronic hepatitis


  • Hepatitis non-ABC



  • Autoimmune hepatitis



  • Cryptogenic neonatal hepatitis




  • Viral hepatitis (A-E)



  • Alcoholic liver disease



  • Nonalcoholic steatohepatitis



  • Autoimmune hepatitis

Acute liver failure


  • Undetermined cause



  • Hepatitis non-ABC



  • Drug toxicity



  • Wilson disease



  • Autoimmune hepatitis




  • Paracetamol overdose



  • Drug- or toxin-induced hepatotoxicity



  • Viral and non-ABC hepatitis



  • Autoimmune hepatitis



  • Wilson disease



  • Acute alcoholic hepatitis



  • Acute fatty liver of pregnancy

Liver tumors


  • Hepatoblastoma



  • Hepatocellular carcinoma



  • Infantile Hemangioendothelioma




  • Hepatocellular carcinoma



  • Hemangioendothelioma



  • Multifocal adenomas

Other causes


  • Budd-Chiari syndrome



  • Cryptogenic liver cirrhosis



  • Infantile copper overload




  • Budd-Chiari syndrome



  • Cryptogenic liver cirrhosis



  • Polycystic liver disease



  • Hereditary hemorrhagic telangiectasia

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Feb 23, 2021 | Posted by in HEPATOPANCREATOBILIARY | Comments Off on What Is Different Between Pediatric and Adult Liver Transplantation?
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