E70-E88
Metabolic disorders
Block coding notes
Excludes 1 never together · Excludes 2 may co-exist
Codes in this block
366 codes
Billableusable codeNon-billableheader / grouping codeRisk-adjusting
- E70Disorders of aromatic amino-acid metabolismNon-billable
- E70.0Classical phenylketonuriaBillable
- E70.1Other hyperphenylalaninemiasBillable
- E70.2Disorders of tyrosine metabolismNon-billable
- E70.20Disorder of tyrosine metabolism, unspecifiedBillable
- E70.21TyrosinemiaBillable
- E70.29Other disorders of tyrosine metabolismBillable
- E70.3AlbinismNon-billable
- E70.30Albinism, unspecifiedBillable
- E70.31Ocular albinismNon-billable
- E70.310X-linked ocular albinismBillable
- E70.311Autosomal recessive ocular albinismBillable
- E70.318Other ocular albinismBillable
- E70.319Ocular albinism, unspecifiedBillable
- E70.32Oculocutaneous albinismNon-billable
- E70.320Tyrosinase negative oculocutaneous albinismBillable
- E70.321Tyrosinase positive oculocutaneous albinismBillable
- E70.328Other oculocutaneous albinismBillable
- E70.329Oculocutaneous albinism, unspecifiedBillable
- E70.33Albinism with hematologic abnormalityNon-billable
- E70.330Chediak-Higashi syndromeBillable
- E70.331Hermansky-Pudlak syndromeBillable
- E70.338Other albinism with hematologic abnormalityBillable
- E70.339Albinism with hematologic abnormality, unspecifiedBillable
- E70.39Other specified albinismBillable
- E70.4Disorders of histidine metabolismNon-billable
- E70.40Disorders of histidine metabolism, unspecifiedBillable
- E70.41HistidinemiaBillable
- E70.49Other disorders of histidine metabolismBillable
- E70.5Disorders of tryptophan metabolismBillable
- E70.8Other disorders of aromatic amino-acid metabolismNon-billable
- E70.81Aromatic L-amino acid decarboxylase deficiencyBillable
- E70.89Other disorders of aromatic amino-acid metabolismBillable
- E70.9Disorder of aromatic amino-acid metabolism, unspecifiedBillable
- E71Disorders of branched-chain amino-acid metabolism and fatty-acid metabolismNon-billable
- E71.0Maple-syrup-urine diseaseBillable
- E71.1Other disorders of branched-chain amino-acid metabolismNon-billable
- E71.11Branched-chain organic aciduriasNon-billable
- E71.110Isovaleric acidemiaBillable
- E71.1113-methylglutaconic aciduriaBillable
- E71.118Other branched-chain organic aciduriasBillable
- E71.12Disorders of propionate metabolismNon-billable
- E71.120Methylmalonic acidemiaBillable
- E71.121Propionic acidemiaBillable
- E71.128Other disorders of propionate metabolismBillable
- E71.19Other disorders of branched-chain amino-acid metabolismBillable
- E71.2Disorder of branched-chain amino-acid metabolism, unspecifiedBillable
- E71.3Disorders of fatty-acid metabolismNon-billable
- E71.30Disorder of fatty-acid metabolism, unspecifiedBillable
- E71.31Disorders of fatty-acid oxidationNon-billable
- E71.310Long chain/very long chain acyl CoA dehydrogenase deficiencyBillable
- E71.311Medium chain acyl CoA dehydrogenase deficiencyBillable
- E71.312Short chain acyl CoA dehydrogenase deficiencyBillable
- E71.313Glutaric aciduria type IIBillable
- E71.314Muscle carnitine palmitoyltransferase deficiencyBillable
- E71.318Other disorders of fatty-acid oxidationBillable
- E71.32Disorders of ketone metabolismBillable
- E71.39Other disorders of fatty-acid metabolismBillable
- E71.4Disorders of carnitine metabolismNon-billable
- E71.40Disorder of carnitine metabolism, unspecifiedBillable
- E71.41Primary carnitine deficiencyBillable
- E71.42Carnitine deficiency due to inborn errors of metabolismBillable
- E71.43Iatrogenic carnitine deficiencyBillable
- E71.44Other secondary carnitine deficiencyNon-billable
- E71.440Ruvalcaba-Myhre-Smith syndromeBillable
- E71.448Other secondary carnitine deficiencyBillable
- E71.5Peroxisomal disordersNon-billable
- E71.50Peroxisomal disorder, unspecifiedBillable
- E71.51Disorders of peroxisome biogenesisNon-billable
- E71.510Zellweger syndromeBillable
- E71.511Neonatal adrenoleukodystrophyBillable
- E71.518Other disorders of peroxisome biogenesisBillable
- E71.52X-linked adrenoleukodystrophyNon-billable
- E71.520Childhood cerebral X-linked adrenoleukodystrophyBillable
- E71.521Adolescent X-linked adrenoleukodystrophyBillable
- E71.522AdrenomyeloneuropathyBillable
- E71.528Other X-linked adrenoleukodystrophyBillable
- E71.529X-linked adrenoleukodystrophy, unspecified typeBillable
- E71.53Other group 2 peroxisomal disordersBillable
- E71.54Other peroxisomal disordersNon-billable
- E71.540Rhizomelic chondrodysplasia punctataBillable
- E71.541Zellweger-like syndromeBillable
- E71.542Other group 3 peroxisomal disordersBillable
- E71.548Other peroxisomal disordersBillable
- E72Other disorders of amino-acid metabolismNon-billable
- E72.0Disorders of amino-acid transportNon-billable
- E72.00Disorders of amino-acid transport, unspecifiedBillable
- E72.01CystinuriaBillable
- E72.02Hartnup's diseaseBillable
- E72.03Lowe's syndromeBillable
- E72.04CystinosisBillable
- E72.09Other disorders of amino-acid transportBillable
- E72.1Disorders of sulfur-bearing amino-acid metabolismNon-billable
- E72.10Disorders of sulfur-bearing amino-acid metabolism, unspecifiedBillable
- E72.11HomocystinuriaBillable
- E72.12Methylenetetrahydrofolate reductase deficiencyBillable
- E72.19Other disorders of sulfur-bearing amino-acid metabolismBillable
- E72.2Disorders of urea cycle metabolismNon-billable
- E72.20Disorder of urea cycle metabolism, unspecifiedBillable
- E72.21ArgininemiaBillable
- E72.22Arginosuccinic aciduriaBillable
- E72.23CitrullinemiaBillable
- E72.29Other disorders of urea cycle metabolismBillable
- E72.3Disorders of lysine and hydroxylysine metabolismBillable
- E72.4Disorders of ornithine metabolismBillable
- E72.5Disorders of glycine metabolismNon-billable
- E72.50Disorder of glycine metabolism, unspecifiedBillable
- E72.51Non-ketotic hyperglycinemiaBillable
- E72.52TrimethylaminuriaBillable
- E72.53Primary hyperoxaluriaNon-billable
- E72.530Primary hyperoxaluria, type 1Billable
- E72.538Other specified primary hyperoxaluriaBillable
- E72.539Primary hyperoxaluria, unspecifiedBillable
- E72.54Secondary hyperoxaluriaNon-billable
- E72.540Dietary hyperoxaluriaBillable
- E72.541Enteric hyperoxaluriaBillable
- E72.548Other secondary hyperoxaluriaBillable
- E72.549Secondary hyperoxaluria, unspecifiedBillable
- E72.59Other disorders of glycine metabolismBillable
- E72.8Other specified disorders of amino-acid metabolismNon-billable
- E72.81Disorders of gamma aminobutyric acid metabolismBillable
- E72.89Other specified disorders of amino-acid metabolismBillable
- E72.9Disorder of amino-acid metabolism, unspecifiedBillable
- E73Lactose intoleranceNon-billable
- E73.0Congenital lactase deficiencyBillable
- E73.1Secondary lactase deficiencyBillable
- E73.8Other lactose intoleranceBillable
- E73.9Lactose intolerance, unspecifiedBillable
- E74Other disorders of carbohydrate metabolismNon-billable
- E74.0Glycogen storage diseaseNon-billable
- E74.00Glycogen storage disease, unspecifiedBillable
- E74.01von Gierke diseaseBillable
- E74.02Pompe diseaseBillable
- E74.03Cori diseaseBillable
- E74.04McArdle diseaseBillable
- E74.05Lysosome-associated membrane protein 2 [LAMP2] deficiencyBillable
- E74.09Other glycogen storage diseaseBillable
- E74.1Disorders of fructose metabolismNon-billable
- E74.10Disorder of fructose metabolism, unspecifiedBillable
- E74.11Essential fructosuriaBillable
- E74.12Hereditary fructose intoleranceBillable
- E74.19Other disorders of fructose metabolismBillable
- E74.2Disorders of galactose metabolismNon-billable
- E74.20Disorders of galactose metabolism, unspecifiedBillable
- E74.21GalactosemiaBillable
- E74.29Other disorders of galactose metabolismBillable
- E74.3Other disorders of intestinal carbohydrate absorptionNon-billable
- E74.31Sucrase-isomaltase deficiencyBillable
- E74.39Other disorders of intestinal carbohydrate absorptionBillable
- E74.4Disorders of pyruvate metabolism and gluconeogenesisBillable
- E74.8Other specified disorders of carbohydrate metabolismNon-billable
- E74.81Disorders of glucose transport, not elsewhere classifiedNon-billable
- E74.810Glucose transporter protein type 1 deficiencyBillable
- E74.818Other disorders of glucose transportBillable
- E74.819Disorders of glucose transport, unspecifiedBillable
- E74.82Disorders of citrate metabolismNon-billable
- E74.820SLC13A5 Citrate Transporter DisorderBillable
- E74.829Other disorders of citrate metabolismBillable
- E74.89Other specified disorders of carbohydrate metabolismBillable
- E74.9Disorder of carbohydrate metabolism, unspecifiedBillable
- E75Disorders of sphingolipid metabolism and other lipid storage disordersNon-billable
- E75.0GM2 gangliosidosisNon-billable
- E75.00GM2 gangliosidosis, unspecifiedBillable
- E75.01Sandhoff diseaseBillable
- E75.02Tay-Sachs diseaseBillable
- E75.09Other GM2 gangliosidosisBillable
- E75.1Other and unspecified gangliosidosisNon-billable
- E75.10Unspecified gangliosidosisBillable
- E75.11Mucolipidosis IVBillable
- E75.19Other gangliosidosisBillable
- E75.2Other sphingolipidosisNon-billable
- E75.21Fabry (-Anderson) diseaseBillable
- E75.22Gaucher diseaseBillable
- E75.23Krabbe diseaseBillable
- E75.24Niemann-Pick diseaseNon-billable
- E75.240Niemann-Pick disease type ABillable
- E75.241Niemann-Pick disease type BBillable
- E75.242Niemann-Pick disease type CBillable
- E75.243Niemann-Pick disease type DBillable
- E75.244Niemann-Pick disease type A/BBillable
- E75.248Other Niemann-Pick diseaseBillable
- E75.249Niemann-Pick disease, unspecifiedBillable
- E75.25Metachromatic leukodystrophyBillable
- E75.26Sulfatase deficiencyBillable
- E75.27Pelizaeus-Merzbacher diseaseBillable
- E75.28Canavan diseaseBillable
- E75.29Other sphingolipidosisBillable
- E75.3Sphingolipidosis, unspecifiedBillable
- E75.4Neuronal ceroid lipofuscinosisBillable
- E75.5Other lipid storage disordersBillable
- E75.6Lipid storage disorder, unspecifiedBillable
- E76Disorders of glycosaminoglycan metabolismNon-billable
- E76.0Mucopolysaccharidosis, type INon-billable
- E76.01Hurler's syndromeBillable
- E76.02Hurler-Scheie syndromeBillable
- E76.03Scheie's syndromeBillable
- E76.1Mucopolysaccharidosis, type IIBillable
- E76.2Other mucopolysaccharidosesNon-billable
- E76.21Morquio mucopolysaccharidosesNon-billable
- E76.210Morquio A mucopolysaccharidosesBillable
- E76.211Morquio B mucopolysaccharidosesBillable
- E76.219Morquio mucopolysaccharidoses, unspecifiedBillable
- E76.22Sanfilippo mucopolysaccharidosesBillable
- E76.29Other mucopolysaccharidosesBillable
- E76.3Mucopolysaccharidosis, unspecifiedBillable
- E76.8Other disorders of glucosaminoglycan metabolismBillable
- E76.9Glucosaminoglycan metabolism disorder, unspecifiedBillable
- E77Disorders of glycoprotein metabolismNon-billable
- E77.0Defects in post-translational modification of lysosomal enzymesBillable
- E77.1Defects in glycoprotein degradationBillable
- E77.8Other disorders of glycoprotein metabolismBillable
- E77.9Disorder of glycoprotein metabolism, unspecifiedBillable
- E78Disorders of lipoprotein metabolism and other lipidemiasNon-billable
- E78.0Pure hypercholesterolemiaNon-billable
- E78.00Pure hypercholesterolemia, unspecifiedBillable
- E78.01Familial hypercholesterolemiaNon-billable
- E78.010Homozygous familial hypercholesterolemia [HoFH]Billable
- E78.011Heterozygous familial hypercholesterolemia [HeFH]Billable
- E78.019Familial hypercholesterolemia, unspecifiedBillable
- E78.1Pure hyperglyceridemiaBillable
- E78.2Mixed hyperlipidemiaBillable
- E78.3HyperchylomicronemiaBillable
- E78.4Other hyperlipidemiaNon-billable
- E78.41Elevated Lipoprotein(a)Billable
- E78.49Other hyperlipidemiaBillable
- E78.5Hyperlipidemia, unspecifiedBillable
- E78.6Lipoprotein deficiencyBillable
- E78.7Disorders of bile acid and cholesterol metabolismNon-billable
- E78.70Disorder of bile acid and cholesterol metabolism, unspecifiedBillable
- E78.71Barth syndromeBillable
- E78.72Smith-Lemli-Opitz syndromeBillable
- E78.79Other disorders of bile acid and cholesterol metabolismBillable
- E78.8Other disorders of lipoprotein metabolismNon-billable
- E78.81Lipoid dermatoarthritisBillable
- E78.89Other lipoprotein metabolism disordersBillable
- E78.9Disorder of lipoprotein metabolism, unspecifiedBillable
- E79Disorders of purine and pyrimidine metabolismNon-billable
- E79.0Hyperuricemia without signs of inflammatory arthritis and tophaceous diseaseBillable
- E79.1Lesch-Nyhan syndromeBillable
- E79.2Myoadenylate deaminase deficiencyBillable
- E79.8Other disorders of purine and pyrimidine metabolismNon-billable
- E79.81Aicardi-Goutieres syndromeBillable
- E79.82Hereditary xanthinuriaBillable
- E79.89Other specified disorders of purine and pyrimidine metabolismBillable
- E79.9Disorder of purine and pyrimidine metabolism, unspecifiedBillable
- E80Disorders of porphyrin and bilirubin metabolismNon-billable
- E80.0Hereditary erythropoietic porphyriaBillable
- E80.1Porphyria cutanea tardaBillable
- E80.2Other and unspecified porphyriaNon-billable
- E80.20Unspecified porphyriaBillable
- E80.21Acute intermittent (hepatic) porphyriaBillable
- E80.29Other porphyriaBillable
- E80.3Defects of catalase and peroxidaseBillable
- E80.4Gilbert syndromeBillable
- E80.5Crigler-Najjar syndromeBillable
- E80.6Other disorders of bilirubin metabolismBillable
- E80.7Disorder of bilirubin metabolism, unspecifiedBillable
- E83Disorders of mineral metabolismNon-billable
- E83.0Disorders of copper metabolismNon-billable
- E83.00Disorder of copper metabolism, unspecifiedBillable
- E83.01Wilson's diseaseBillable
- E83.09Other disorders of copper metabolismBillable
- E83.1Disorders of iron metabolismNon-billable
- E83.10Disorder of iron metabolism, unspecifiedBillable
- E83.11HemochromatosisNon-billable
- E83.110Hereditary hemochromatosisBillable
- E83.111Hemochromatosis due to repeated red blood cell transfusionsBillable
- E83.118Other hemochromatosisBillable
- E83.119Hemochromatosis, unspecifiedBillable
- E83.19Other disorders of iron metabolismBillable
- E83.2Disorders of zinc metabolismBillable
- E83.3Disorders of phosphorus metabolism and phosphatasesNon-billable
- E83.30Disorder of phosphorus metabolism, unspecifiedBillable
- E83.31Familial hypophosphatemiaBillable
- E83.32Hereditary vitamin D-dependent rickets (type 1) (type 2)Billable
- E83.39Other disorders of phosphorus metabolismBillable
- E83.4Disorders of magnesium metabolismNon-billable
- E83.40Disorders of magnesium metabolism, unspecifiedBillable
- E83.41HypermagnesemiaBillable
- E83.42HypomagnesemiaBillable
- E83.49Other disorders of magnesium metabolismBillable
- E83.5Disorders of calcium metabolismNon-billable
- E83.50Unspecified disorder of calcium metabolismBillable
- E83.51HypocalcemiaBillable
- E83.52HypercalcemiaBillable
- E83.59Other disorders of calcium metabolismBillable
- E83.8Other disorders of mineral metabolismNon-billable
- E83.81Hungry bone syndromeBillable
- E83.82Disorders of pyrophosphate metabolismNon-billable
- E83.820Generalized arterial calcification of infancy with unspecified genetic causalityBillable
- E83.821ENPP1 deficiency causing generalized arterial calcification of infancyBillable
- E83.822ENPP1 deficiency causing autosomal recessive hypophosphatemic rickets type 2Billable
- E83.823ABCC6 deficiency causing generalized arterial calcification of infancyBillable
- E83.824ABCC6 deficiency causing pseudoxanthoma elasticumBillable
- E83.825CD73 deficiency causing arterial calcificationBillable
- E83.89Other disorders of mineral metabolismBillable
- E83.9Disorder of mineral metabolism, unspecifiedBillable
- E84Cystic fibrosisNon-billable
- E84.0Cystic fibrosis with pulmonary manifestationsBillable
- E84.1Cystic fibrosis with intestinal manifestationsNon-billable
- E84.11Meconium ileus in cystic fibrosisBillable
- E84.19Cystic fibrosis with other intestinal manifestationsBillable
- E84.8Cystic fibrosis with other manifestationsBillable
- E84.9Cystic fibrosis, unspecifiedBillable
- E85AmyloidosisNon-billable
- E85.0Non-neuropathic heredofamilial amyloidosisBillable
- E85.1Neuropathic heredofamilial amyloidosisBillable
- E85.2Heredofamilial amyloidosis, unspecifiedBillable
- E85.3Secondary systemic amyloidosisBillable
- E85.4Organ-limited amyloidosisBillable
- E85.8Other amyloidosisNon-billable
- E85.81Light chain (AL) amyloidosisBillable
- E85.82Wild-type transthyretin-related (ATTR) amyloidosisBillable
- E85.89Other amyloidosisBillable
- E85.9Amyloidosis, unspecifiedBillable
- E86Volume depletionNon-billable
- E86.0DehydrationBillable
- E86.1HypovolemiaBillable
- E86.9Volume depletion, unspecifiedBillable
- E87Other disorders of fluid, electrolyte and acid-base balanceNon-billable
- E87.0Hyperosmolality and hypernatremiaBillable
- E87.1Hypo-osmolality and hyponatremiaBillable
- E87.2AcidosisNon-billable
- E87.20Acidosis, unspecifiedBillable
- E87.21Acute metabolic acidosisBillable
- E87.22Chronic metabolic acidosisBillable
- E87.29Other acidosisBillable
- E87.3AlkalosisBillable
- E87.4Mixed disorder of acid-base balanceBillable
- E87.5HyperkalemiaBillable
- E87.6HypokalemiaBillable
- E87.7Fluid overloadNon-billable
- E87.70Fluid overload, unspecifiedBillable
- E87.71Transfusion associated circulatory overloadBillable
- E87.79Other fluid overloadBillable
- E87.8Other disorders of electrolyte and fluid balance, not elsewhere classifiedBillable
- E88Other and unspecified metabolic disordersNon-billable
- E88.0Disorders of plasma-protein metabolism, not elsewhere classifiedNon-billable
- E88.01Alpha-1-antitrypsin deficiencyBillable
- E88.02Plasminogen deficiencyBillable
- E88.09Other disorders of plasma-protein metabolism, not elsewhere classifiedBillable
- E88.1Lipodystrophy, not elsewhere classifiedNon-billable
- E88.10Lipodystrophy, unspecifiedBillable
- E88.11Partial lipodystrophyBillable
- E88.12Generalized lipodystrophyBillable
- E88.13Localized lipodystrophyBillable
- E88.14HIV-associated lipodystrophyBillable
- E88.19Other lipodystrophy, not elsewhere classifiedBillable
- E88.2Lipomatosis, not elsewhere classifiedBillable
- E88.3Tumor lysis syndromeBillable
- E88.4Mitochondrial metabolism disordersNon-billable
- E88.40Mitochondrial metabolism disorder, unspecifiedBillable
- E88.41MELAS syndromeBillable
- E88.42MERRF syndromeBillable
- E88.43Disorders of mitochondrial tRNA synthetasesBillable
- E88.49Other mitochondrial metabolism disordersBillable
- E88.8Other specified metabolic disordersNon-billable
- E88.81Metabolic syndrome and other insulin resistanceNon-billable
- E88.810Metabolic syndromeBillable
- E88.811Insulin resistance syndrome, Type ABillable
- E88.818Other insulin resistanceBillable
- E88.819Insulin resistance, unspecifiedBillable
- E88.82Obesity due to disruption of MC4R pathwayBillable
- E88.89Other specified metabolic disordersBillable
- E88.9Metabolic disorder, unspecifiedBillable
- E88.AWasting disease (syndrome) due to underlying conditionBillable
Official Guidelines for E70-E88
FY 2026 ›I.C.4Chapter-specific guidanceChapter 4: Endocrine, Nutritional, and Metabolic Diseases (E00-E89)
Subsections
Sections that reference codes in E70-E88