Introduction Acute liver failure (ALF) is defined as the acute onset of severe hepatitis with loss of hepatic function in patients with no known underlying liver disease. ALF can result from various hepatic insults including viral infections, drug-induced liver injury,…
Etiology of Pediatric Acute Liver Failure The Pediatric Acute Liver Failure (PALF) Study Group, a multicenter and multinational consortium, suggested the following definition: (1) no known evidence of chronic liver disease, (2) biochemical evidence of liver injury, and (3) hepatic-based…
Introduction Alagille syndrome (ALGS) is a rare multisystem disorder and one of the most frequent inherited causes of cholestatic liver disease in children. Traditionally, ALGS has been characterized by the presence of at least three of the following five principal…
Introduction Biliary atresia is a uniquely perinatal disease that 30 years ago would have been universally fatal. With the development of the palliative Kasai portoenterostomy (Morio Kasai, 1922–2008), along with advances in pediatric liver transplantation (first performed by Thomas Starzl,…
Introduction In recent years, the number of gene therapy (GT) trials targeting the liver have grown significantly, particularly when it comes to gene addition strategies (delivery of cDNA for a whole gene to the target organ/cells, which then exists in…
Introduction For numerous liver diseases of childhood, liver transplantation (LT) is a lifesaving procedure. However, it requires scarce organs, a highly experienced team to manage the surgical procedure, complications, and follow-up and lifelong immunosuppression for the recipient. Living donor organs…
Introduction Currently, the standard medical treatment (SMT) for pediatric liver failure incorporates optimal supportive care with hemodynamic and respiratory support, infection control, and avoidance of gastrointestinal bleeding, aiming to bridge patients to recovery or to liver transplantation (LT). LT is…
Introduction Over the past decades, survival rates after pediatric liver transplantation have increased from about 76% to over 90% more than 10 years after transplantation (Tx). Follow-up care of liver-transplanted children includes both monitoring of graft functioning and monitoring for…
Diagnosis of Complications: Role of Imaging Modalities Systematic Follow-up Ultrasonography with Doppler (US Doppler) is the best imaging modality for systematic follow-up of the liver allograft and for the detection and diagnosis of complications. Grayscale mode allows for the analysis…
Introduction Post-transplant lymphoproliferative diseases (PTLDs) are the most frequent malignant disorders after solid-organ transplantation in children. Although morphologically often indistinguishable from de novo lymphomatous diseases, per definition, every lymphoproliferation arising after transplantation is considered a PTLD. Because pathogenesis in the…