sodium-benzoate and Liver-Failure--Acute

Acute liver failure may be caused by a variety of disorders including inborn errors of metabolism. In those cases, rapid metabolic investigations and adequate treatment may avoid the need for liver transplantation. We report two patients who presented with acute liver failure and were referred to our center for liver transplantation work-up. Urgent metabolic investigations revealed citrullinemia type I. Treatment for citrullinemia type I avoided the need for liver transplantation. Acute liver failure as a presentation of citrullinemia type I has not previously been reported in young children. Although acute liver failure has occasionally been described in other urea cycle disorders, these disorders may be underestimated as a cause. Timely diagnosis and treatment of these disorders may avoid liver transplantation and improve clinical outcome. Therefore, urea cycle disorders should be included in the differential diagnosis in young children presenting with acute liver failure.

Topics: Administration, Oral; Arginine; Biomarkers; Cells, Cultured; Citrullinemia; Diagnosis, Differential; Diet, Protein-Restricted; Drug Therapy, Combination; Female; Glucose; Humans; Infant; Infusions, Intravenous; Liver Failure, Acute; Liver Transplantation; Male; Predictive Value of Tests; Sodium Benzoate; Treatment Outcome; Unnecessary Procedures

Hepatic encephalopathy (HE) is a neuropsychiatric syndrome in patients with liver failure and/or a portal-systemic bypass. Since 2002 a new nomenclature of HE exists, that classifies HE in encephalopathy type A (associated with acute liver failure), type B (associated with portal-systemic bypass), and type C (associated with liver cirrhosis). HE type A is characterized by a rapid development to coma, cerebral edema, and a poor short-term prognosis. Therefore, these patients should be referred to a liver transplantation center. Standard treatment of HE consists of non absorbable disaccharides, non absorbable antibiotics, and a diet with an appropriate amount of proteins. In addition, the possibility of performing a liver transplantation should be evaluated. In patients with intractable HE other alternative treatments adjunct to standard treatment, like zinc, sodium benzoate, ornithine aspartate, branched chain amino acids, flumazenil, and bromocriptine should be considered.

Topics: Anti-Bacterial Agents; Antifungal Agents; Bromocriptine; Dipeptides; Dopamine Agonists; Electroencephalography; Flumazenil; GABA Modulators; Gastrointestinal Agents; Hepatic Encephalopathy; Humans; Lactulose; Liver Failure, Acute; Liver Transplantation; Neomycin; Nutritional Physiological Phenomena; Practice Guidelines as Topic; Prognosis; Sodium Benzoate; Terminology as Topic; Time Factors; Zinc