2020 Volume 63 Issue 3 Pages 132-138
A 27-year-old woman felt physical fatigue and loss of appetite 7 days after delivering her child. She was emergently hospitalized for disturbance of consciousness due to diabetic ketoacidosis and hyperammonemia 25 days after delivery. Although her metabolic acidosis and hyperglycemia were rapidly corrected with intravenous insulin infusion, the disturbance of consciousness and hyperammonemia did not improve. She had no remarkable medical history or family history of urea cycle disorders (UCDs), but a plasma amino acid analysis showed low serum citrulline levels, indicating UCD. Without additional treatment for hyperammonemia, her consciousness level improved with normalization of her serum ammonia and citrulline levels after oral zinc supplementation for the zinc deficiency (49 μg/dL). This clinical course strongly suggested that hyperammonemia was caused by the temporary decrease in ornithine transcarbamylase activity due to zinc deficiency. It is necessary to consider the influence of zinc deficiency as a cause of acute hyperammonemia if malnutrition due to a long-term poor appetite is suspected.
