Neonatal acute liver failure

Abstract

Neonatal acute liver failure (NALF) is a rare disease about which there is little published data; however, NALF is an extremely important condition as it is distinct from acute liver failure seen in older children and adults. First, unlike acute liver failure in older patients, NALF can be diagnosed in an infant with cirrhosis. This is due to the fetal‐neonatal continuum of liver disease, or the principle that neonatal liver failure may be the result of a liver disease that began in utero. Further differences exist in the mechanism of disease, diagnostic principles, and the common etiologies of NALF when compared with pediatric and adult acute liver failure. This review will address many of the distinguishing features of NALF and focus on the most common etiologies of NALF, including gestational alloimmune liver disease (GALD), the most common cause of NALF. Additionally, this review will provide insight into the pathogenesis, diagnosis, and treatment of this rare condition. Liver Transplantation 22 677‐685 2016 AASLD.

Neonatal acute liver failure: a diagnosis challenge

“…Diagnosis is confirmed once extrahepatic iron deposits are observed through magnetic resonance imaging and/or oral cavity salivary gland biopsy. [2][3][4] FAH treatment is aimed at the alloimmune etiology and consists in the combination of double volume exchange transfusion and highdose intravenous immunoglobulin to remove aggressive antibodies and block their action. This treatment should be implemented immediately if FAH is suspected.…”

“…Metabolic disorders are rarely the cause of NALF. [1][2][3] Galactosemia, type 1 tyrosinemia, and hereditary fructose intolerance -all inherited autosomal recessive disorders-may occur in the neonatal period together with jaundice or hypoglycemia, and may occasionally progress to NALF. Out of these three conditions, galactosemia may be more common in the first month of life, once milk feeding is introduced.…”

“…Although these conditions may exceptionally be the cause of NALF, test costs are minimal, considering that they are treatable diseases. 1,2,[9][10][11][12] Respiratory chain (RC) defects account for a group of mitochondrial diseases. These organelles are in charge of generating energy in the form of adenosine triphosphate (ATP), an element that is essential for effective cell functioning.…”

“…Supportive treatment may also include sodium bicarbonate infusion during acute metabolic acidosis events, transfusions in the case of anemia and thrombocytopenia, and exogenous enzymes for patients with pancreatic insufficiency. 1,2,[12][13][14] A mutation in the TRMU gene (a protein involved in mitochondrial transfer RNA [tRNA] modifications) has been associated with NALF, which is sometimes transient. Patients who survive the initial episode may recover and grow normally.…”

Insuficiencia hepática aguda neonatal: un desafío diagnóstico

“…El diagnóstico se confirma con la demostración de depósitos de hierro extrahepáticos, mediante resonancia magnética y/o biopsia de las glándulas salivares de la mucosa oral. [2][3][4] El tratamiento de la HFA está dirigido a la etiología aloinmune y consiste en la combinación de exanguinotransfusión de doble volumen con dosis elevadas de inmunoglobulina intravenosa para remover los anticuerpos ofensivos y bloquear su acción. Este tratamiento debe ser implementado rápidamente frente a la sospecha de esta patología.…”