Specific code 2015 ICD-9-CM Diagnosis Code 277.2
Other disorders of purine and pyrimidine metabolism
  • 2015
  • Billable Thru Sept 30/2015
  • Non-Billable On/After Oct 1/2015

  • ICD-9-CM 277.2 is a billable medical code that can be used to indicate a diagnosis on a reimbursement claim, however, 277.2 should only be used for claims with a date of service on or before September 30, 2015. For claims with a date of service on or after October 1, 2015, use an equivalent ICD-10-CM code (or codes).
Convert to ICD-10-CM: 277.2 converts approximately to:
  • 2015/16 ICD-10-CM E79.1 Lesch-Nyhan syndrome
    Or:
  • 2015/16 ICD-10-CM E79.8 Other disorders of purine and pyrimidine metabolism
Approximate Synonyms
  • Adenine phosphoribosyl transferase deficiency type I
  • Adenine phosphoribosyl transferase deficiency type II
  • Adenine phosphoribosyltransferase deficiency, Japanese type
  • Adenosine deaminase deficiency
  • Adenylosuccinate lyase deficiency
  • beta-Aminoisobutyricaciduria
  • Combined molybdoflavoprotein enzyme deficiency
  • Cytosine diphosphate choline phosphotransferase deficiency
  • Decreased uric acid level
  • Dihydropyrimidinase deficiency
  • Dihydrouracil dehydrogenase deficiency
  • Disorder of purine and pyrimidine metabolism
  • Disorder of purine metabolism
  • Disorder of pyrimidine metabolism
  • Hereditary orotic aciduria, type 2
  • Hereditary xanthinuria
  • Hooft's syndrome
  • Hyperuricuria
  • Hypouricemia
  • Inosine triphosphate pyrophosphohydrolase deficiency
  • Isolated xanthine oxidase deficiency
  • Lesch Nyhan syndrome
  • Lesch-Nyhan syndrome
  • Muscle AMP deaminase deficiency
  • Partial hypoxanthine-guanine phosphoribosyltransferase deficiency
  • Purine and pyrimidine metabolism disorder
  • Purine nucleoside phosphorylase deficiency
  • Purine-nucleoside phosphorylase deficiency
  • Ribose-phosphate pyrophosphokinase overactivity
  • Secondary orotic aciduria
  • Thiopurine methyltransferase deficiency
  • Uridine monophosphate hydrolase deficiency
  • Xanthinuria
Clinical Information
  • An inborn error of metabolism resulting from a deficiency of hypoxanthine-guanine phosphoribosyltransferase (ec 2.4.2.8) with increased conversion of glycine to uric acid with excessive purine synthesis and hyperuricemia. Patients are normal at birth but begin to show hypertonicity at about 4 months and irritability and other neurological symptoms become apparent during the second year of life, the child becoming gradually more aggressive and self-destructive, banging his head, biting the lower lip and, less commonly, the upper lip, cheeks, fingers, and hands, sometimes using the fingers to mutilate his ears and nose. Associated disorders include spastic cerebral palsy, choreoathetosis, renal uric acid calculi, gouty tophi, and uric acid nodules. Mental retardation is common but normal intelligence occurs in some cases
  • Rare x-linked disorder of purine metabolism, due to deficiency of hypoxanthine phosphoribosyltransferase; affected individuals are normal in the first year of life and then develop psychomotor retardation, extrapyramidal movement disorders, progressive spasticity, and seizures; self-destructive behaviors such as biting of fingers and lips are seen frequently; intellectual impairment may also occur but is typically not severe; elevation of uric acid in the serum leads to the development of renal calculi and gouty arthritis
277.2 Excludes
Applies To
  • Hypoxanthine-guanine-phosphoribosyltransferase deficiency [HG-PRT deficiency]
  • Lesch-Nyhan syndrome
  • Xanthinuria
ICD-9-CM Volume 2 Index entries containing back-references to 277.2:
  • Aciduria 791.9
    • beta-aminoisobutyric (BAIB) 277.2
  • Beta-aminoisobutyric aciduria 277.2
  • Deficiency, deficient
    • adenosine deaminase 277.2
    • HG-PRT 277.2
    • hypoxanthine-guanine phosphoribosyltransferase (HG-PRT) 277.2
    • PRT 277.2
    • purine nucleoside phosphorylase 277.2
  • Disorder - see also Disease
    • amino acid (metabolic) (see also Disturbance, metabolism, amino acid) 270.9
      • albinism 270.2
      • alkaptonuria 270.2
      • argininosuccinicaciduria 270.6
      • beta-amino-isobutyricaciduria 277.2
      • cystathioninuria 270.4
      • cystinosis 270.0
      • cystinuria 270.0
      • glycinuria 270.0
      • homocystinuria 270.4
      • imidazole 270.5
      • maple syrup (urine) disease 270.3
      • neonatal, transitory 775.89
      • oasthouse urine disease 270.2
      • ochronosis 270.2
      • phenylketonuria 270.1
      • phenylpyruvic oligophrenia 270.1
      • purine NEC 277.2
      • pyrimidine NEC 277.2
      • renal transport NEC 270.0
      • specified type NEC 270.8
      • transport NEC 270.0
      • xanthinuria 277.2
    • purine metabolism NEC 277.2
    • pyrimidine metabolism NEC 277.2
  • Lesch-Nyhan syndrome (hypoxanthine-guanine-phosphoribosyltransferase deficiency) 277.2
  • Stone(s) - see also Calculus
    • xanthine 277.2
  • Syndrome - see also Disease
    • Lesch-Nyhan (hypoxanthine-guanine-phosphroibosyltransferase deficiency) 277.2
  • Xanthine stones 277.2
  • Xanthinuria 277.2
ICD-9-CM codes are used in medical billing and coding to describe diseases, injuries, symptoms and conditions. ICD-9-CM 277.2 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 2015 ICD-9-CM Index or use the search engine at the top of this page to lookup any code.