A case of hypercitrullinemia is reported. A 52-year-old woman was admitted to our hospital because of eating and sleeping disturbailces due to chronic intractable pain.
When aminoacid-containing hyperalimentation fluid was administerd to the patient, she fell into deep coma. She returned to consciousness when the infusion was discontinued, however, subsequent aminoacid-free hyperalimentation induced hyperglycemia again followed by unconsciousness.
Although it has already been reported in the literature that coma is triggered by aminoacid infusions in patients with hypercitrullinemia, we obtained new evidence that aminoacid-free, glucose-rich solutions also cause unconsciousness.
In this report, we discuss the possible mechanism of plasma ammonia level elevation in response to the administration of amino acids and/or glucose, and concluded that a cautious nutrition therapy plan, with frequent measurement of blood pH, ammonia and glucose levels, is mandatory.