An adult-type citrullinemia in HBV-carrier.

概要

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A 23-year-old man who is a carrier of hepatitis type B virus developed in October 1983 frequent episodes of unconsciousness, and admitted to our department in the following Novermber. High ammonia level in his serum and slight abnormality in his hepatic function were observed in laboratory data. EEG showed 2-3Hz triphasic wave and 5-6Hz theta wave even at clear consciousness. Laparoscopy for his liver and histology of his liver biopsy specimen showed the finding of chronic active hepatitis, but no cirrhosis. Abdominal angio-graphy revealed no abnormal shunt. Plasma aminogram showed that citrulline levels increased fifteen times more than that in normal. Hepatic enzymes concerning the urea cycle revealed a decreased levels of argininosuccinate synthetase with a titer of about one tenth of normal levels. Based on these results a diagnosis of citrullinemia was made. The restriction of his daily protein intake to 30g and oral administration of the specially-formulated milk (Meiji: 7925-A) for citrullinemia revealed a remarkable effect on his hyper-ammonemia.<BR>He was discharged in April 1984, and is in good condition attending to our out-patient clinic.<BR>The serial EEG study on this case was previously reported in "Rinsyo-noha" (27: 609-611, 1985). In the present case report, we made a report on details of the progress and the treatment.