Proceedings of the European Society for Pediatric Gastroenterology, Hepatology and Nutrition Monothematic Conference, 2020

Abstract

Objectives:The Hepatology Committee of the European Society for Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) aims to educate pediatric gastroenterologists, members of ESPGHAN and professionals from other specialties promoting an exchange of clinical expertise in the field of pediatric hepatology.Methods:The 2020 single topic ESPGHAN monothematic 3-day conference on pediatric liver disease, was organized in Athens, Greece and was entitled “ Acute Liver Failure” (ALF). ALF is a devastating disease with high mortality and in a considerable fraction of patients, the cause remains unresolved. As knowledge in diagnosis and treatment of ALF in infants and children has increased in the past decades, the objective was to update physicians in the field with developments in medical therapy and indications for liver transplantation (LT) and to identify areas for future research in clinical and neurocognitive outcomes in ALF.Results:We recently reported the epidemiology, diagnosis, and initial intensive care management issues in separate manuscript. Herewith we report on the medical treatment, clinical lessons arising from pediatric studies, nutritional and renal replacement therapy (RRT), indications and contraindications for LT, neurocognitive outcomes, new techniques used as bridging to LT, and areas for future research. Oral presentations by experts in various fields are summarized highlighting key learning points.Conclusions:The current report summarizes the current insights in medical treatment of pediatric ALF and the directions for future research.

Acute Hepatitis of Unknown Etiology Among Young Children: Research Agenda by the ESPGHAN Hepatology Committee

“…The suggested investigations match the common work-up for infectious diseases of children with acute hepatitis and are summarized in Table 3. A complete differential diagnosis for acute hepatitis and acute liver failure is at the same time mandatory for all children (Table 4) (17,19). It is, indeed, important not to forget the noninfectious etiologies including autoimmune, metabolic, and drug-related causes of acute severe hepatitis which must be appropriately excluded.…”

“…At that time point, a specific definition of severe acute hepatitis was not provided, but it can be assumed that the common definition was applied being acute hepatitis the inflammation of the liver biochemically characterized by the steep rise of hepatic serum biomarkers (aspartate aminotransferase and alanine aminotransferase) in children. Since following discussion within the UK Health Security Agency with collaboration of the three pediatric liver services in the United Kingdom, a working case definition was agreed upon (see below) including the presence of high levels (>500 IU/L) of aminotransferases with or without acute liver failure (international normalized ratio ≥ 1.5 in encephalopathic patients or ≥ 2.0 in nonencephalopathic patients with no known evidence of chronic liver disease not improving on parenteral administration with vitamin K) (17,18).…”

Pediatric Acute Liver Failure

“…Recently, in close succession, the Italian [ 17 ], American [ 18 ], and European [ 19 , 20 ] societies for pediatric gastroenterology, hepatology, and nutrition have issued new recommendations regarding the etiology, clinical features, diagnostic work-up, medical management, and transplant indications for newborns, infants, and children with ALF. All recommendations used the 1999 PALFSG’s definition of PALF [ 10 , 11 , 12 ].…”

“…ESPGHAN [ 19 , 20 ] underscores that the knowledge gained from adult patients in the intensive care unit cannot necessarily be transferred to children. In fact, the diagnosis is strongly age-dependent, with newborns and infants being at higher risk of poor outcomes unless they receive prompt medical attention, proper imaging, appropriate critical care, and timely and judicious referral to an LT center.…”

“…Auxiliary LT and human hepatocyte transplantation could help restore the native liver in certain circumstances in expert centers. The health-related quality of life needs attention as it remains impaired in children after ALF in the first year after hospitalization [ 20 ]. Both NASPGHAN and ESPGHAN societies highlight the importance of an etiology-based therapies for some conditions with specific treatments when available ( Table 3 ).…”