About Section E70-E88

Section E70-E88 covers Metabolic disorders within Chapter 4 of the ICD-10-CM Tabular List. This section includes diagnosis codes ranging from E70 to E88. Each code below includes its description, billable status, and links to detailed coding guidelines.
Diagnosis Codes 366 entries
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-Goutières 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›