Table 1 Blood biochemistry evolution. N-Carbamil glutamate (100 mg/kg through nasogastric tube) was administered at 2 h after PICU admission, and Sodium phenylacetate and sodium benzoate (Ammonul® 55 mL/m²) was administered after 8 h. A rapid increase in hyperammonemia may be observed, peaking at 7 h after admission to the PICU and a subsequent rapid decline to normalization between 14 and 19 h. The liver and Kidney function improved from 24 h of hospital admission. AST, aspartate aminotransferase; ALT, alanine aminotransferase; PT, Prothrombin Time; QI, Quick index; APTT (sec) activated partial thromboplastin time; CK, creatine phosphokinase; CRP, C-reactive protein

From: Management and long-term evolution of a patient with 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency