Neonatal liver failure: Not always a fatal outcome

A seven-day-old newborn presents with hypoglycemia, jaundice, slightly elevated liver function test results and a bleeding disorder, despite having received intramuscular vitamin K. The treating physician determines that the diagnosis is neonatal liver failure (NLF). The diagnosis presents unique clinical challenges. How can the physician overcome the false impression that this infant will have a fatal outcome without a liver transplant? By adding NLF to the monthly report form, the Canadian Paediatric Surveillance Program (CPSP) achieves this goal in several ways. It increases the awareness of participants by setting a clear case definition. It helps physicians to approach a difficult diagnosis by establishing a detailed clinical protocol that defines the different presentations – NLF may be classified as ‘acute pattern’ or ‘chronic pattern’. A newborn with acute pattern NLF has liver damage that was caused by an acute insult, such as infection with hepatitis B virus, hepatitis C virus, herpes simplex virus or enterovirus, or by neoplasia such as neuroblastoma or congenital leukemia. A newborn with chronic pattern NLF has already experienced significant liver damage because of metabolic diseases (such as tyrosinemia, galactosemia, fructosemia, perinatal hemochromatosis) or infection with parvovirus B19. Many cases of NLF are treatable in the neonatal period and some of the newborns will survive. Early recognition, aggressive neonatal intensive care and recognized, disease-specific treatments can be life-saving. For example, some cases of perinatal hemochromatosis, a chronic pattern NLF, have been treated successfully with a combination of antioxidants with desferroxamine, without liver transplantation. Your monthly CPSP report will help to advance knowledge about this rare but potentially treatable disease, leading to earlier identification and more specific diagnosis. Ultimately, the new knowledge will offer hope that an infant born with NLF will not always have a fatal outcome without a liver transplant.