FIGURE 1.

Clinical presentation and investigation. A) Table; hospitalization summary including age at presentation, clinical status and intervention during each admission. ALF, acute liver failure; INR, International normalized ratio; MRI, magnetic resonance imaging; NICU Neonatal Intensive Care Unit; PICU, Pediatric Intensive Care Unit; pRBC, packed red blood cells. B) Evolution of liver profile and methionine levels since the neonatal period; This panel shows the variation in liver enzyme (alanine aminotransferase shown in U/L), bilirubin total/direct (μmol/L), international normalized ratio and plasma methionine level (μmol/L) since the first presentation during the neonatal period. Since birth, he has had five episodes of acute liver injury. After the patient was diagnosed with ADK deficiency and started on a low‐methionine diet (at age 8‐9 months), no episode of acute liver injury was observed, and his liver profile and methionine level normalize