2008 ICD-9-CM Volume 1 Diagnosis Codes Home > Endocrine, Nutritional And Metabolic Diseases, And Immunity Disorders 240-279 > Other Metabolic Disorders And Immunity Disorders 270-279 > Disorders Of Amino-Acid Transport And Metabolism 270.* > 2008 ICD-9-CM Diagnosis 270.6 Disorders of urea cycle metabolism  On October 1, 2008 the 2009 ICD-9-CM codes came into effect. Therefore, this code may be out of date. The 2009 version of ICD-9-CM 270.6 can be accessed here. A group of diseases related to a deficiency of the enzyme ARGININOSUCCINATE SYNTHASE which causes an elevation of serum levels of CITRULLINE. In neonates, clinical manifestations include lethargy, hypotonia, and SEIZURES. Milder forms also occur. Childhood and adult forms may present with recurrent episodes of intermittent weakness, lethargy, ATAXIA, behavioral changes, and DYSARTHRIA. (From Menkes, Textbook of Child Neurology, 5th ed, p49) Metabolic disorder characterized by elevated level of ammonia in blood. 270.6 is a specific code that can be used to specify a diagnosis 270.6 contains 26 index entries View the ICD-9-CM Volume 1 270.* hierarchy ICD9Data's sister site ProcedureCodes.com launches with the 2008/09 CPT® codes - read more on the ICD9Data.com blog. 270.6 also known as: Argininosuccinic aciduria Citrullinemia Disorders of metabolism of ornithine, citrulline, argininosuccinic acid, arginine, and ammonia Hyperammonemia Hyperornithinemia Index entries containing 270.6: Acidemia 276.2 arginosuccinic 270.6 Aciduria 791.9 arginosuccinic 270.6 Arginosuccinicaciduria 270.6 Citrullinemia 270.6 Citrullinuria 270.6 Deficiency, deficient argininosuccinate synthetase or lyase 270.6 carbamyl phosphate synthetase 270.6 ornithine transcarbamylase 270.6 Disorder - see also Disease amino acid (metabolic) (see also Disturbance, metabolism, amino acid) 270.9 argininosuccinicaciduria 270.6 metabolism NEC 277.9 ammonia 270.6 arginine 270.6 argininosuccinic acid 270.6 citrulline 270.6 ornithine 270.6 urea cycle 270.6 Disturbance - see also Disease metabolism (acquired) (congenital) (see also Disorder, metabolism) 277.9 ammonia 270.6 arginine 270.6 argininosuccinic acid 270.6 citrulline 270.6 ornithine 270.6 urea cycle 270.6 Hyperaminoaciduria 270.9 arginine 270.6 citrulline 270.6 ornithine 270.6 Hyperammonemia (congenital) 270.6 Hyperornithinemia 270.6