Metabolic disorders E70-E88

Metabolic disorders E70-E88

Type 1 Excludes
Type 1 Excludes Help
A type 1 excludes note is a pure excludes. It means "not coded here". A type 1 excludes note indicates that the code excluded should never be used at the same time as E70-E88. A type 1 excludes note is for used for when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
  • androgen insensitivity syndrome (
    ICD-10-CM Diagnosis Code E34.5

    Androgen insensitivity syndrome

      2016 2017 2018 2019 2020 2021 Non-Billable/Non-Specific Code
  • congenital adrenal hyperplasia (
    ICD-10-CM Diagnosis Code E25.0

    Congenital adrenogenital disorders associated with enzyme deficiency

      2016 2017 2018 2019 2020 2021 Billable/Specific Code
    Applicable To
    • Congenital adrenal hyperplasia
    • 21-Hydroxylase deficiency
    • Salt-losing congenital adrenal hyperplasia
  • Ehlers-Danlos syndromes (
    ICD-10-CM Diagnosis Code Q79.6

    Ehlers-Danlos syndromes

      2016 2017 2018 2019 2020 - Converted to Parent Code 2021 Non-Billable/Non-Specific Code
  • hemolytic anemias attributable to enzyme disorders (
    ICD-10-CM Diagnosis Code D55
    • D55 Anemia due to enzyme disorders
      • D55.0 Anemia due to glucose-6-phosphate dehydrogena...
      • D55.1 Anemia due to other disorders of glutathione ...
      • D55.2 Anemia due to disorders of glycolytic enzymes...
      • D55.3 Anemia due to disorders of nucleotide metabol...
      • D55.8 Other anemias due to enzyme disorders
      • D55.9 Anemia due to enzyme disorder, unspecified
  • Marfan's syndrome (
    ICD-10-CM Diagnosis Code Q87.4

    Marfan's syndrome

      2016 2017 2018 2019 2020 2021 Non-Billable/Non-Specific Code
  • 5-alpha-reductase deficiency (
    ICD-10-CM Diagnosis Code E29.1

    Testicular hypofunction

      2016 2017 2018 2019 2020 2021 Billable/Specific Code Male Dx
    Applicable To
    • Defective biosynthesis of testicular androgen NOS
    • 5-delta-Reductase deficiency (with male pseudohermaphroditism)
    • Testicular hypogonadism NOS
    Type 1 Excludes
    • postprocedural testicular hypofunction (E89.5)
    Use Additional
    • code for adverse effect, if applicable, to identify drug (T36-T50 with fifth or sixth character 5)
ICD-10-CM Diagnosis Codes E70-*
  • E70 Disorders of aromatic amino-acid metabolism
    • E70.0 Classical phenylketonuria
    • E70.1 Other hyperphenylalaninemias
    • E70.2 Disorders of tyrosine metabolism
      • E70.20 Disorder of tyrosine metabolism, unspecified
      • E70.21 Tyrosinemia
      • E70.29 Other disorders of tyrosine metabolism
    • E70.3 Albinism
      • E70.30 …… unspecified
      • E70.31 Ocular albinism
      • E70.32 Oculocutaneous albinism
        • E70.320 Tyrosinase negative oculocutaneous albinism
        • E70.321 Tyrosinase positive oculocutaneous albinism
        • E70.328 Other oculocutaneous albinism
        • E70.329 …… unspecified
      • E70.33 Albinism with hematologic abnormality
        • E70.330 Chediak-Higashi syndrome
        • E70.331 Hermansky-Pudlak syndrome
        • E70.338 Other albinism with hematologic abnormality
        • E70.339 Albinism with hematologic abnormality, unspec...
      • E70.39 Other specified albinism
    • E70.4 Disorders of histidine metabolism
      • E70.40 Disorders of histidine metabolism, unspecifie...
      • E70.41 Histidinemia
      • E70.49 Other disorders of histidine metabolism
    • E70.5 Disorders of tryptophan metabolism
    • E70.8 Other disorders of aromatic amino-acid metabo...
      • E70.81 Aromatic L-amino acid decarboxylase deficienc...
      • E70.89 Other disorders of aromatic amino-acid metabo...
    • E70.9 Disorder of aromatic amino-acid metabolism, u...
ICD-10-CM Diagnosis Codes E71-*
  • E71 Disorders of branched-chain amino-acid metabo...
    • E71.0 Maple-syrup-urine disease
    • E71.1 Other disorders of branched-chain amino-acid ...
      • E71.11 Branched-chain organic acidurias
        • E71.110 Isovaleric acidemia
        • E71.111 3-methylglutaconic aciduria
        • E71.118 Other branched-chain organic acidurias
      • E71.12 Disorders of propionate metabolism
        • E71.120 Methylmalonic acidemia
        • E71.121 Propionic acidemia
        • E71.128 Other disorders of propionate metabolism
      • E71.19 Other disorders of branched-chain amino-acid ...
    • E71.2 Disorder of branched-chain amino-acid metabol...
    • E71.3 Disorders of fatty-acid metabolism
      • E71.30 Disorder of fatty-acid metabolism, unspecifie...
      • E71.31 Disorders of fatty-acid oxidation
        • E71.310 Long chain/very long chain acyl CoA dehydroge...
        • E71.311 Medium chain acyl CoA dehydrogenase deficienc...
        • E71.312 Short chain acyl CoA dehydrogenase deficiency...
        • E71.313 Glutaric aciduria type II
        • E71.314 Muscle carnitine palmitoyltransferase deficie...
        • E71.318 Other disorders of fatty-acid oxidation
      • E71.32 Disorders of ketone metabolism
      • E71.39 Other disorders of fatty-acid metabolism
    • E71.4 Disorders of carnitine metabolism
      • E71.40 Disorder of carnitine metabolism, unspecified...
      • E71.41 Primary carnitine deficiency
      • E71.42 Carnitine deficiency due to inborn errors of ...
      • E71.43 Iatrogenic carnitine deficiency
      • E71.44 Other secondary carnitine deficiency
        • E71.440 Ruvalcaba-Myhre-Smith syndrome
        • E71.448 Other secondary carnitine deficiency
    • E71.5 Peroxisomal disorders
      • E71.50 Peroxisomal disorder, unspecified
      • E71.51 Disorders of peroxisome biogenesis
        • E71.510 Zellweger syndrome
        • E71.511 Neonatal adrenoleukodystrophy
        • E71.518 Other disorders of peroxisome biogenesis
      • E71.52 X-linked adrenoleukodystrophy
        • E71.520 Childhood cerebral X-linked adrenoleukodystro...
        • E71.521 Adolescent X-linked adrenoleukodystrophy
        • E71.522 Adrenomyeloneuropathy
        • E71.528 Other X-linked adrenoleukodystrophy
        • E71.529 X-linked adrenoleukodystrophy, unspecified ty...
      • E71.53 Other group 2 peroxisomal disorders
      • E71.54 Other peroxisomal disorders
        • E71.540 Rhizomelic chondrodysplasia punctata
        • E71.541 Zellweger-like syndrome
        • E71.542 Other group 3 peroxisomal disorders
        • E71.548 Other peroxisomal disorders
ICD-10-CM Diagnosis Codes E72-*
  • E72 Other disorders of amino-acid metabolism
    • E72.0 Disorders of amino-acid transport
      • E72.00 Disorders of amino-acid transport, unspecifie...
      • E72.01 Cystinuria
      • E72.02 Hartnup's disease
      • E72.03 Lowe's syndrome
      • E72.04 Cystinosis
      • E72.09 Other disorders of amino-acid transport
    • E72.1 Disorders of sulfur-bearing amino-acid metabo...
      • E72.10 Disorders of sulfur-bearing amino-acid metabo...
      • E72.11 Homocystinuria
      • E72.12 Methylenetetrahydrofolate reductase deficienc...
      • E72.19 Other disorders of sulfur-bearing amino-acid ...
    • E72.2 Disorders of urea cycle metabolism
      • E72.20 Disorder of urea cycle metabolism, unspecifie...
      • E72.21 Argininemia
      • E72.22 Arginosuccinic aciduria
      • E72.23 Citrullinemia
      • E72.29 Other disorders of urea cycle metabolism
    • E72.3 Disorders of lysine and hydroxylysine metabol...
    • E72.4 Disorders of ornithine metabolism
    • E72.5 Disorders of glycine metabolism
      • E72.50 Disorder of glycine metabolism, unspecified
      • E72.51 Non-ketotic hyperglycinemia
      • E72.52 Trimethylaminuria
      • E72.53 Primary hyperoxaluria
      • E72.59 Other disorders of glycine metabolism
    • E72.8 Other specified disorders of amino-acid metab...
      • E72.81 Disorders of gamma aminobutyric acid metaboli...
      • E72.89 Other specified disorders of amino-acid metab...
    • E72.9 Disorder of amino-acid metabolism, unspecifie...
ICD-10-CM Diagnosis Codes E73-*
  • E73 Lactose intolerance
    • E73.0 Congenital lactase deficiency
    • E73.1 Secondary lactase deficiency
    • E73.8 Other lactose intolerance
    • E73.9 Lactose intolerance, unspecified
ICD-10-CM Diagnosis Codes E74-*
  • E74 Other disorders of carbohydrate metabolism
    • E74.0 Glycogen storage disease
    • E74.1 Disorders of fructose metabolism
      • E74.10 Disorder of fructose metabolism, unspecified
      • E74.11 Essential fructosuria
      • E74.12 Hereditary fructose intolerance
      • E74.19 Other disorders of fructose metabolism
    • E74.2 Disorders of galactose metabolism
      • E74.20 Disorders of galactose metabolism, unspecifie...
      • E74.21 Galactosemia
      • E74.29 Other disorders of galactose metabolism
    • E74.3 Other disorders of intestinal carbohydrate ab...
      • E74.31 Sucrase-isomaltase deficiency
      • E74.39 Other disorders of intestinal carbohydrate ab...
    • E74.4 Disorders of pyruvate metabolism and gluconeo...
    • E74.8 Other specified disorders of carbohydrate met...
      • E74.81 Disorders of glucose transport, not elsewhere...
        • E74.810 Glucose transporter protein type 1 deficiency...
        • E74.818 Other disorders of glucose transport
        • E74.819 Disorders of glucose transport, unspecified
      • E74.89 Other specified disorders of carbohydrate met...
    • E74.9 Disorder of carbohydrate metabolism, unspecif...
ICD-10-CM Diagnosis Codes E75-*
  • E75 Disorders of sphingolipid metabolism and othe...
ICD-10-CM Diagnosis Codes E76-*
  • E76 Disorders of glycosaminoglycan metabolism
    • E76.0 Mucopolysaccharidosis, type I
    • E76.1 Mucopolysaccharidosis, type II
    • E76.2 Other mucopolysaccharidoses
      • E76.21 Morquio mucopolysaccharidoses
        • E76.210 Morquio A mucopolysaccharidoses
        • E76.211 Morquio B mucopolysaccharidoses
        • E76.219 …… unspecified
      • E76.22 Sanfilippo mucopolysaccharidoses
      • E76.29 Other mucopolysaccharidoses
    • E76.3 Mucopolysaccharidosis, unspecified
    • E76.8 Other disorders of glucosaminoglycan metaboli...
    • E76.9 Glucosaminoglycan metabolism disorder, unspec...
ICD-10-CM Diagnosis Codes E77-*
  • E77 Disorders of glycoprotein metabolism
    • E77.0 Defects in post-translational modification of...
    • E77.1 Defects in glycoprotein degradation
    • E77.8 Other disorders of glycoprotein metabolism
    • E77.9 Disorder of glycoprotein metabolism, unspecif...
ICD-10-CM Diagnosis Codes E78-*
  • E78 Disorders of lipoprotein metabolism and other...
    • E78.0 Pure hypercholesterolemia
      • E78.00 …… unspecified
      • E78.01 Familial hypercholesterolemia
    • E78.1 Pure hyperglyceridemia
    • E78.2 Mixed hyperlipidemia
    • E78.3 Hyperchylomicronemia
    • E78.4 Other hyperlipidemia
    • E78.5 Hyperlipidemia, unspecified
    • E78.6 Lipoprotein deficiency
    • E78.7 Disorders of bile acid and cholesterol metabo...
      • E78.70 Disorder of bile acid and cholesterol metabol...
      • E78.71 Barth syndrome
      • E78.72 Smith-Lemli-Opitz syndrome
      • E78.79 Other disorders of bile acid and cholesterol ...
    • E78.8 Other disorders of lipoprotein metabolism
      • E78.81 Lipoid dermatoarthritis
      • E78.89 Other lipoprotein metabolism disorders
    • E78.9 Disorder of lipoprotein metabolism, unspecifi...
ICD-10-CM Diagnosis Codes E79-*
  • E79 Disorders of purine and pyrimidine metabolism...
    • E79.0 Hyperuricemia without signs of inflammatory a...
    • E79.1 Lesch-Nyhan syndrome
    • E79.2 Myoadenylate deaminase deficiency
    • E79.8 Other disorders of purine and pyrimidine meta...
    • E79.9 Disorder of purine and pyrimidine metabolism,...
ICD-10-CM Diagnosis Codes E80-*
  • E80 Disorders of porphyrin and bilirubin metaboli...
    • E80.0 Hereditary erythropoietic porphyria
    • E80.1 Porphyria cutanea tarda
    • E80.2 Other and unspecified porphyria
      • E80.20 Unspecified porphyria
      • E80.21 Acute intermittent (hepatic) porphyria
      • E80.29 Other porphyria
    • E80.3 Defects of catalase and peroxidase
    • E80.4 Gilbert syndrome
    • E80.5 Crigler-Najjar syndrome
    • E80.6 Other disorders of bilirubin metabolism
    • E80.7 Disorder of bilirubin metabolism, unspecified...
ICD-10-CM Diagnosis Codes E83-*
  • E83 Disorders of mineral metabolism
    • E83.0 Disorders of copper metabolism
      • E83.00 Disorder of copper metabolism, unspecified
      • E83.01 Wilson's disease
      • E83.09 Other disorders of copper metabolism
    • E83.1 Disorders of iron metabolism
      • E83.10 Disorder of iron metabolism, unspecified
      • E83.11 Hemochromatosis
        • E83.110 Hereditary hemochromatosis
        • E83.111 Hemochromatosis due to repeated red blood cel...
        • E83.118 Other hemochromatosis
        • E83.119 …… unspecified
      • E83.19 Other disorders of iron metabolism
    • E83.2 Disorders of zinc metabolism
    • E83.3 Disorders of phosphorus metabolism and phosph...
      • E83.30 Disorder of phosphorus metabolism, unspecifie...
      • E83.31 Familial hypophosphatemia
      • E83.32 Hereditary vitamin D-dependent rickets (type ...
      • E83.39 Other disorders of phosphorus metabolism
    • E83.4 Disorders of magnesium metabolism
      • E83.40 Disorders of magnesium metabolism, unspecifie...
      • E83.41 Hypermagnesemia
      • E83.42 Hypomagnesemia
      • E83.49 Other disorders of magnesium metabolism
    • E83.5 Disorders of calcium metabolism
      • E83.50 Unspecified disorder of calcium metabolism
      • E83.51 Hypocalcemia
      • E83.52 Hypercalcemia
      • E83.59 Other disorders of calcium metabolism
    • E83.8 Other disorders of mineral metabolism
      • E83.81 Hungry bone syndrome
      • E83.89 Other disorders of mineral metabolism
    • E83.9 Disorder of mineral metabolism, unspecified
ICD-10-CM Diagnosis Codes E84-*
  • E84 Cystic fibrosis
    • E84.0 Cystic fibrosis with pulmonary manifestations...
    • E84.1 Cystic fibrosis with intestinal manifestation...
      • E84.11 Meconium ileus in cystic fibrosis
      • E84.19 Cystic fibrosis with other intestinal manifes...
    • E84.8 Cystic fibrosis with other manifestations
    • E84.9 Cystic fibrosis, unspecified
ICD-10-CM Diagnosis Codes E85-*
  • E85 Amyloidosis
    • E85.0 Non-neuropathic heredofamilial amyloidosis
    • E85.1 Neuropathic heredofamilial amyloidosis
    • E85.2 Heredofamilial amyloidosis, unspecified
    • E85.3 Secondary systemic amyloidosis
    • E85.4 Organ-limited amyloidosis
    • E85.8 Other amyloidosis
      • E85.81 Light chain (AL) amyloidosis
      • E85.82 Wild-type transthyretin-related (ATTR) amyloi...
      • E85.89 Other amyloidosis
    • E85.9 Amyloidosis, unspecified
ICD-10-CM Diagnosis Codes E86-*
  • E86 Volume depletion
ICD-10-CM Diagnosis Codes E87-*
  • E87 Other disorders of fluid, electrolyte and aci...
    • E87.0 Hyperosmolality and hypernatremia
    • E87.1 Hypo-osmolality and hyponatremia
    • E87.2 Acidosis
    • E87.3 Alkalosis
    • E87.4 Mixed disorder of acid-base balance
    • E87.5 Hyperkalemia
    • E87.6 Hypokalemia
    • E87.7 Fluid overload
      • E87.70 …… unspecified
      • E87.71 Transfusion associated circulatory overload
      • E87.79 Other fluid overload
    • E87.8 Other disorders of electrolyte and fluid bala...
ICD-10-CM Diagnosis Codes E88-*
  • E88 Other and unspecified metabolic disorders
    • E88.0 Disorders of plasma-protein metabolism, not e...
      • E88.01 Alpha-1-antitrypsin deficiency
      • E88.02 Plasminogen deficiency
      • E88.09 Other disorders of plasma-protein metabolism,...
    • E88.1 Lipodystrophy, not elsewhere classified
    • E88.2 Lipomatosis, not elsewhere classified
    • E88.3 Tumor lysis syndrome
    • E88.4 Mitochondrial metabolism disorders
      • E88.40 Mitochondrial metabolism disorder, unspecifie...
      • E88.41 MELAS syndrome
      • E88.42 MERRF syndrome
      • E88.49 Other mitochondrial metabolism disorders
    • E88.8 Other specified metabolic disorders
      • E88.81 Metabolic syndrome
      • E88.89 Other specified metabolic disorders
    • E88.9 Metabolic disorder, unspecified