TYPE-II CITRULLINEMIA ASSOCIATED WITH NEUTROPENIA

A 37-year-old Japanese man was admitted with delirium and hyperammonem ia, He was diagnosed as having type II citrullinemia because of an ele vated citrulline level on amino acid analysis and very low hepatic arg ininosuccinate synthetase activity. He also showed a low neutrophil co unt and a low serum level of granulocyte colony-stimulating factor, Re duced production of this cytokine and/or impairment of its feedback re gulation by the neutrophil count may have played a role in the neutrop enia of this patient.