Specific code 2014 ICD-9-CM Diagnosis Code 270.6
Disorders of urea cycle metabolism
  • Billable Code
  • Short description: Dis urea cycle metabol.
  • ICD-9-CM 270.6 will be replaced by an equivalent ICD-10-CM code (or codes) when the United States transitions from ICD-9-CM to ICD-10-CM on October 1, 2015.
  • ICD-9-CM 270.6 is a billable medical code that can be used to specify a diagnosis on a reimbursement claim.
Disease Synonyms
  • Arginase deficiency
  • Argininaemia
  • Argininemia
  • Argininosuccinate lyase deficiency
  • Arginosuccinate lyase deficiency
  • Carbamoyl phosphate synthetase deficiency
  • Citrullinemia
  • Congenital hyperammonemia, type I
  • Deficiency, arginosuccinate lyase
  • Deficiency, carbamyl phosphate synthetase
  • Deficiency, N acetylglutamate synthetase
  • Deficiency, ornithine transcarbamylase
  • Disorder of ornithine metabolism
  • Disorder of the urea cycle metabolism
  • Disorder of urea cycle metabolism
  • Hyperammonemia
  • Hyperammonemia, type III
  • Hyperornithinemia hyperammonemia homocitrullinemia
  • Hyperornithinemia hyperammonemia homocitrullinemia syndrome
  • Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome
  • N-acetylglutamate synthetase deficiency
  • Ornithine carbamoyltransferase deficiency
  • Ornithine metabolism disorder
  • Ornithine transcarbamylase deficiency
  • Urea cycle metabolism disorder
Clinical Information
  • A genetic inborn error of metabolism characterized by the deficiency of one of the enzymes necessary for the urea cycle. It results in accumulation of ammonia in the body.
  • Autosomal recessive aminoacidopathy characterized by marked elevation in plasma and urine levels of citrulline, with hyperammonemia and sometimes secondary oroticaciduria; clinical findings include mental retardation and neurologic abnormalities.
  • Excess of ornithine in the plasma, such as occurs in the genetic disorders gyrate atrophy of choroid and retina and hyperornithinemia-hyperamonemia-homocitrullinuria syndrome.
  • Inherited errors in the metabolic reactions occurring in the liver that convert ammonia to urea, resulting from inborn genetic mutations.
  • Rare congenital metabolism disorders of the urea cycle. The disorders are due to mutations that result in complete (neonatal onset) or partial (childhood or adult onset) inactivity of an enzyme, involved in the urea cycle. Neonatal onset results in clinical features that include irritability, vomiting, lethargy, seizures, neonatal hypotonia; respiratory alkalosis; hyperammonemia; coma, and death. Survivors of the neonatal onset and childhood/adult onset disorders share common risks for encephalopathies, metabolic, inborn; and respiratory alkalosis due to hyperammonemia.
Applies To
  • Argininosuccinic aciduria
  • Citrullinemia
  • Disorders of metabolism of ornithine, citrulline, argininosuccinic acid, arginine, and ammonia
  • Hyperammonemia
  • Hyperornithinemia
Convert to ICD-10-CM: 270.6 converts approximately to:
  • 2014 ICD-10-CM E72.20 Disorder of urea cycle metabolism, unspecified
    Or:
  • 2014 ICD-10-CM E72.22 Arginosuccinic aciduria
    Or:
  • 2014 ICD-10-CM E72.23 Citrullinemia
    Or:
  • 2014 ICD-10-CM E72.29 Other disorders of urea cycle metabolism
ICD-9-CM Volume 2 Index entries containing back-references to 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
  • Disturbance - see also Disease
  • Hyperaminoaciduria 270.9
    • arginine 270.6
    • citrulline 270.6
    • ornithine 270.6
  • Hyperammonemia (congenital) 270.6
  • Hyperornithinemia 270.6
ICD-9-CM codes are used in medical billing and coding to describe diseases, injuries, symptoms and conditions. ICD-9-CM 270.6 is one of thousands of ICD-9-CM codes used in healthcare. Although ICD-9-CM and CPT codes are largely numeric, they differ in that CPT codes describe medical procedures and services. Can't find a code? Start at the root of ICD-9-CM, check the 2014 ICD-9-CM Index or use the search engine at the top of this page to lookup any code.