Specific code 2014 ICD-9-CM Diagnosis Code 270.0
Disturbances of amino-acid transport
  • Billable Code
  • Short description: Amino-acid transport dis.
  • ICD-9-CM 270.0 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 270.0 is a billable medical code that can be used to specify a diagnosis on a reimbursement claim.
Disease Synonyms
  • 3-methylcrotonyl glycinuria
  • Amino acid transport disorder
  • Cystinosis
  • Cystinuria
  • Cystinuria type 1
  • Cystinuria, type 1
  • Disorder of amino acid transport
  • Fanconi syndrome
  • Glycinuria, 3 methylcrotonyl
  • Hartnup disease
  • Methylcrotonyl-CoA carboxylase deficiency
  • Neutral 1 amino acid transport defect
Clinical Information
  • An autosomal recessive hereditary disorder characterized by defective transportation of cystine across the lysosomal membranes and systemic deposition of cystine crystals in the body. It is associated with slight increase of the plasma cystine, cystinuria, aminoaciduria, glycosuria, polyuria, hypophosphatemia, rickets, and renal tubular dysfunction.
  • Constellation of clinical and laboratory manifestations produced by generalized proximal tubular insufficiency in the presence of normal, or nearly normal, glomerular filtration.
  • Disorder of amino acid transport characterized by the childhood (or rarely adult) onset of photosensitive dermatitis and intermittent neurologic symptoms; ataxia, personality changes, migraine headaches, and photophobia may occur periodically; results from impaired sodium-dependent transport of neutral amino acids across the brush border membrane of the small intestine and renal tubular epithelium.
  • Inherited abnormality of renal tubular transport of dibasic amino acids leading to massive urinary excretion of cystine, lysine, arginine, and ornithine.
  • Lysosomal storage disorders of unknown molecular defect, characterized by widespread deposition of cystine crystals in reticuloendothelial cells.
Applies To
  • Cystinosis
  • Cystinuria
  • Fanconi (-de Toni) (-Debré) syndrome
  • Glycinuria (renal)
  • Hartnup disease
Convert to ICD-10-CM: 270.0 converts approximately to:
  • 2014 ICD-10-CM E72.00 Disorders of amino-acid transport, unspecified
    Or:
  • 2014 ICD-10-CM E72.01 Cystinuria
    Or:
  • 2014 ICD-10-CM E72.04 Cystinosis
    Or:
  • 2014 ICD-10-CM E72.09 Other disorders of amino-acid transport
ICD-9-CM Volume 2 Index entries containing back-references to 270.0:
  • Abderhalden-Kaufmann-Lignac syndrome (cystinosis) 270.0
  • Beta-mercaptolactate-cysteine disulfiduria 270.0
  • Blue
    • diaper syndrome 270.0
  • Cystinosis (malignant) 270.0
  • Cystinuria 270.0
  • Deficiency, deficient
    • sulfite oxidase 270.0
  • de Toni-Fanconi syndrome (cystinosis) 270.0
  • Diathesis
    • cystine (familial) 270.0
  • Disease, diseased - see also Syndrome
    • cystine storage (with renal sclerosis) 270.0
    • H 270.0
    • Hart's (pellagra-cerebellar ataxia-renal aminoaciduria) 270.0
    • Hartnup (pellagra-cerebellar ataxia-renal aminoaciduria) 270.0
    • Lignac's (cystinosis) 270.0
  • 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
        • renal 270.0
      • xanthinuria 277.2
  • Disturbance - see also Disease
    • amino acid (metabolic) (see also Disorder, amino acid) 270.9
      • imidazole 270.5
      • maple syrup (urine) disease 270.3
      • transport 270.0
  • Disulfiduria, beta-mercaptolactate-cysteine 270.0
  • Dwarf, dwarfism 259.4
    • nephrotic-glycosuric, with hypophosphatemic rickets 270.0
  • Fanconi (-de Toni) (-Debré) syndrome (cystinosis) 270.0
  • Glycinuria (renal) (with ketosis) 270.0
  • Hart's disease or syndrome (pellagra-cerebellar ataxia-renal aminoaciduria) 270.0
  • Hartnup disease (pellagra-cerebellar ataxia-renal aminoaciduria) 270.0
  • H disease 270.0
  • Hyperaminoaciduria 270.9
    • cystine 270.0
    • glycine 270.0
    • renal (types I, II, III) 270.0
  • Lignac's disease (cystinosis) 270.0
  • Lignac (-de Toni) (-Fanconi) (-Debré) syndrome (cystinosis) 270.0
  • Lignac (-Fanconi) syndrome (cystinosis) 270.0
  • Pellagra-cerebellar-ataxia-renal aminoaciduria syndrome 270.0
  • Rickets (active) (acute) (adolescent) (adult) (chest wall) (congenital) (current) (infantile) (intestinal) 268.0
    • hypophosphatemic with nephrotic-glycosuric dwarfism 270.0
  • Sclerosis, sclerotic
    • renal 587
      • with
        • cystine storage disease 270.0
        • hypertension (see also Hypertension, kidney) 403.90
        • hypertensive heart disease (conditions classifiable to 402) (see also Hypertension, cardiorenal) 404.90
      • arteriolar (hyaline) (see also Hypertension, kidney) 403.90
        • hyperplastic (see also Hypertension, kidney) 403.90
  • Stone(s) - see also Calculus
    • cystine 270.0
  • Syndrome - see also Disease
    • de Toni-Fanconi (-Debre) (cystinosis) 270.0
    • Fanconi (-de Toni) (-Debré) (cystinosis) 270.0
    • Hart's (pellagra-cerebellar ataxia-renal aminoaciduria) 270.0
    • Lignac (-de Toni) (-Fanconi) (-Debré) (cystinosis) 270.0
    • pellagra-cerebellar ataxia-renal aminoaciduria 270.0
    • Toni-Fanconi (cystinosis) 270.0
  • Thesaurismosis
    • cystine 270.0
  • Toni-Fanconi syndrome (cystinosis) 270.0
ICD-9-CM codes are used in medical billing and coding to describe diseases, injuries, symptoms and conditions. ICD-9-CM 270.0 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.