Metabolic disorders (E70-E88)
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Instructional notes
Excludes1: not coded here, use a different code
Excludes2: not included here, code separately if applicable
- Ehlers-Danlos syndromes (Q79.6-)
Official coding guidelines
From the ICD-10-CM Official Guidelines for Coding and Reporting, FY 2027 (CMS and NCHS). Links open the PDF at the right page.
Covers diabetes mellitus coding, including type, insulin and drug use, pump malfunction and secondary diabetes, plus obesity and obesity class codes.
- Diabetes mellitus (page 39)
- Obesity (page 42)
All codes under E70-E88
E70-E88 can't be used on a claim. Pick a billable code below; grey headings group them.
- E70Disorders of aromatic amino-acid metabolism
- E70.0Classical phenylketonuria
- E70.1Other hyperphenylalaninemias
- E70.2Disorders of tyrosine metabolism
- E70.20Disorder of tyrosine metabolism, unspecified
- E70.21Tyrosinemia
- E70.29Other disorders of tyrosine metabolism
- E70.3Albinism
- E70.30Albinism, unspecified
- E70.31Ocular albinism
- E70.310X-linked ocular albinism
- E70.311Autosomal recessive ocular albinism
- E70.318Other ocular albinism
- E70.319Ocular albinism, unspecified
- E70.32Oculocutaneous albinism
- E70.320Tyrosinase negative oculocutaneous albinism
- E70.321Tyrosinase positive oculocutaneous albinism
- E70.328Other oculocutaneous albinism
- E70.329Oculocutaneous albinism, unspecified
- E70.33Albinism with hematologic abnormality
- E70.330Chediak-Higashi syndrome
- E70.331Hermansky-Pudlak syndrome
- E70.338Other albinism with hematologic abnormality
- E70.339Albinism with hematologic abnormality, unspecified
- E70.39Other specified albinism
- E70.4Disorders of histidine metabolism
- E70.40Disorders of histidine metabolism, unspecified
- E70.41Histidinemia
- E70.49Other disorders of histidine metabolism
- E70.5Disorders of tryptophan metabolism
- E70.8Other disorders of aromatic amino-acid metabolism
- E70.81Aromatic L-amino acid decarboxylase deficiency
- E70.89Other disorders of aromatic amino-acid metabolism
- E70.9Disorder of aromatic amino-acid metabolism, unspecified
- E71Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
- E71.0Maple-syrup-urine disease
- E71.1Other disorders of branched-chain amino-acid metabolism
- E71.11Branched-chain organic acidurias
- E71.110Isovaleric acidemia
- E71.1113-methylglutaconic aciduria
- E71.118Other branched-chain organic acidurias
- E71.12Disorders of propionate metabolism
- E71.120Methylmalonic acidemia
- E71.121Propionic acidemia
- E71.128Other disorders of propionate metabolism
- E71.19Other disorders of branched-chain amino-acid metabolism
- E71.2Disorder of branched-chain amino-acid metabolism, unspecified
- E71.3Disorders of fatty-acid metabolism
- E71.30Disorder of fatty-acid metabolism, unspecified
- E71.31Disorders of fatty-acid oxidation
- E71.310Long chain/very long chain acyl CoA dehydrogenase deficiency
- E71.311Medium chain acyl CoA dehydrogenase deficiency
- E71.312Short chain acyl CoA dehydrogenase deficiency
- E71.313Glutaric aciduria type II
- E71.314Muscle carnitine palmitoyltransferase deficiency
- E71.318Other disorders of fatty-acid oxidation
- E71.32Disorders of ketone metabolism
- E71.39Other disorders of fatty-acid metabolism
- E71.4Disorders of carnitine metabolism
- E71.40Disorder of carnitine metabolism, unspecified
- E71.41Primary carnitine deficiency
- E71.42Carnitine deficiency due to inborn errors of metabolism
- E71.43Iatrogenic carnitine deficiency
- E71.44Other secondary carnitine deficiency
- E71.440Ruvalcaba-Myhre-Smith syndrome
- E71.448Other secondary carnitine deficiency
- E71.5Peroxisomal disorders
- E71.50Peroxisomal disorder, unspecified
- E71.51Disorders of peroxisome biogenesis
- E71.510Zellweger syndrome
- E71.511Neonatal adrenoleukodystrophy
- E71.518Other disorders of peroxisome biogenesis
- E71.52X-linked adrenoleukodystrophy
- E71.520Childhood cerebral X-linked adrenoleukodystrophy
- E71.521Adolescent X-linked adrenoleukodystrophy
- E71.522Adrenomyeloneuropathy
- E71.528Other X-linked adrenoleukodystrophy
- E71.529X-linked adrenoleukodystrophy, unspecified type
- E71.53Other group 2 peroxisomal disorders
- E71.54Other peroxisomal disorders
- E71.540Rhizomelic chondrodysplasia punctata
- E71.541Zellweger-like syndrome
- E71.542Other group 3 peroxisomal disorders
- E71.548Other peroxisomal disorders
- E72Other disorders of amino-acid metabolism
- E72.0Disorders of amino-acid transport
- E72.00Disorders of amino-acid transport, unspecified
- E72.01Cystinuria
- E72.02Hartnup's disease
- E72.03Lowe's syndrome
- E72.04Cystinosis
- E72.09Other disorders of amino-acid transport
- E72.1Disorders of sulfur-bearing amino-acid metabolism
- E72.10Disorders of sulfur-bearing amino-acid metabolism, unspecified
- E72.11Homocystinuria
- E72.12Methylenetetrahydrofolate reductase deficiency
- E72.19Other disorders of sulfur-bearing amino-acid metabolism
- E72.2Disorders of urea cycle metabolism
- E72.20Disorder of urea cycle metabolism, unspecified
- E72.21Argininemia
- E72.22Arginosuccinic aciduria
- E72.23Citrullinemia
- E72.29Other disorders of urea cycle metabolism
- E72.3Disorders of lysine and hydroxylysine metabolism
- E72.4Disorders of ornithine metabolism
- E72.5Disorders of glycine metabolism
- E72.50Disorder of glycine metabolism, unspecified
- E72.51Non-ketotic hyperglycinemia
- E72.52Trimethylaminuria
- E72.53Primary hyperoxaluria
- E72.530Primary hyperoxaluria, type 1
- E72.538Other specified primary hyperoxaluria
- E72.539Primary hyperoxaluria, unspecified
- E72.54Secondary hyperoxaluria
- E72.540Dietary hyperoxaluria
- E72.541Enteric hyperoxaluria
- E72.548Other secondary hyperoxaluria
- E72.549Secondary hyperoxaluria, unspecified
- E72.59Other disorders of glycine metabolism
- E72.8Other specified disorders of amino-acid metabolism
- E72.81Disorders of gamma aminobutyric acid metabolism
- E72.89Other specified disorders of amino-acid metabolism
- E72.9Disorder of amino-acid metabolism, unspecified
- E73Lactose intolerance
- E73.0Congenital lactase deficiency
- E73.1Secondary lactase deficiency
- E73.8Other lactose intolerance
- E73.9Lactose intolerance, unspecified
- E74Other disorders of carbohydrate metabolism
- E74.0Glycogen storage disease
- E74.00Glycogen storage disease, unspecified
- E74.01von Gierke disease
- E74.02Pompe disease
- E74.03Cori disease
- E74.04McArdle disease
- E74.05Lysosome-associated membrane protein 2 [LAMP2] deficiency
- E74.09Other glycogen storage disease
- E74.1Disorders of fructose metabolism
- E74.10Disorder of fructose metabolism, unspecified
- E74.11Essential fructosuria
- E74.12Hereditary fructose intolerance
- E74.19Other disorders of fructose metabolism
- E74.2Disorders of galactose metabolism
- E74.20Disorders of galactose metabolism, unspecified
- E74.21Galactosemia
- E74.29Other disorders of galactose metabolism
- E74.3Other disorders of intestinal carbohydrate absorption
- E74.31Sucrase-isomaltase deficiency
- E74.39Other disorders of intestinal carbohydrate absorption
- E74.4Disorders of pyruvate metabolism and gluconeogenesis
- E74.8Other specified disorders of carbohydrate metabolism
- E74.81Disorders of glucose transport, not elsewhere classified
- E74.810Glucose transporter protein type 1 deficiency
- E74.818Other disorders of glucose transport
- E74.819Disorders of glucose transport, unspecified
- E74.82Disorders of citrate metabolism
- E74.820SLC13A5 Citrate Transporter Disorder
- E74.829Other disorders of citrate metabolism
- E74.89Other specified disorders of carbohydrate metabolism
- E74.9Disorder of carbohydrate metabolism, unspecified
- E75Disorders of sphingolipid metabolism and other lipid storage disorders
- E75.0GM2 gangliosidosis
- E75.00GM2 gangliosidosis, unspecified
- E75.01Sandhoff disease
- E75.02Tay-Sachs disease
- E75.09Other GM2 gangliosidosis
- E75.1Other and unspecified gangliosidosis
- E75.10Unspecified gangliosidosis
- E75.11Mucolipidosis IV
- E75.19Other gangliosidosis
- E75.2Other sphingolipidosis
- E75.21Fabry (-Anderson) disease
- E75.22Gaucher disease
- E75.23Krabbe disease
- E75.24Niemann-Pick disease
- E75.240Niemann-Pick disease type A
- E75.241Niemann-Pick disease type B
- E75.242Niemann-Pick disease type C
- E75.243Niemann-Pick disease type D
- E75.244Niemann-Pick disease type A/B
- E75.248Other Niemann-Pick disease
- E75.249Niemann-Pick disease, unspecified
- E75.25Metachromatic leukodystrophy
- E75.26Sulfatase deficiency
- E75.27Pelizaeus-Merzbacher disease
- E75.28Canavan disease
- E75.29Other sphingolipidosis
- E75.3Sphingolipidosis, unspecified
- E75.4Neuronal ceroid lipofuscinosis
- E75.5Other lipid storage disorders
- E75.6Lipid storage disorder, unspecified
- E76Disorders of glycosaminoglycan metabolism
- E76.0Mucopolysaccharidosis, type I
- E76.01Hurler's syndrome
- E76.02Hurler-Scheie syndrome
- E76.03Scheie's syndrome
- E76.1Mucopolysaccharidosis, type II
- E76.2Other mucopolysaccharidoses
- E76.21Morquio mucopolysaccharidoses
- E76.210Morquio A mucopolysaccharidoses
- E76.211Morquio B mucopolysaccharidoses
- E76.219Morquio mucopolysaccharidoses, unspecified
- E76.22Sanfilippo mucopolysaccharidoses
- E76.29Other mucopolysaccharidoses
- E76.3Mucopolysaccharidosis, unspecified
- E76.8Other disorders of glucosaminoglycan metabolism
- E76.9Glucosaminoglycan metabolism disorder, unspecified
- E77Disorders of glycoprotein metabolism
- E77.0Defects in post-translational modification of lysosomal enzymes
- E77.1Defects in glycoprotein degradation
- E77.8Other disorders of glycoprotein metabolism
- E77.9Disorder of glycoprotein metabolism, unspecified
- E78Disorders of lipoprotein metabolism and other lipidemias
- E78.0Pure hypercholesterolemia
- E78.00Pure hypercholesterolemia, unspecified
- E78.01Familial hypercholesterolemia
- E78.010Homozygous familial hypercholesterolemia [HoFH]
- E78.011Heterozygous familial hypercholesterolemia [HeFH]
- E78.019Familial hypercholesterolemia, unspecified
- E78.1Pure hyperglyceridemia
- E78.2Mixed hyperlipidemia
- E78.3Hyperchylomicronemia
- E78.4Other hyperlipidemia
- E78.41Elevated Lipoprotein(a)
- E78.49Other hyperlipidemia
- E78.5Hyperlipidemia, unspecified
- E78.6Lipoprotein deficiency
- E78.7Disorders of bile acid and cholesterol metabolism
- E78.70Disorder of bile acid and cholesterol metabolism, unspecified
- E78.71Barth syndrome
- E78.72Smith-Lemli-Opitz syndrome
- E78.79Other disorders of bile acid and cholesterol metabolism
- E78.8Other disorders of lipoprotein metabolism
- E78.81Lipoid dermatoarthritis
- E78.89Other lipoprotein metabolism disorders
- E78.9Disorder of lipoprotein metabolism, unspecified
- E79Disorders of purine and pyrimidine metabolism
- E79.0Hyperuricemia without signs of inflammatory arthritis and tophaceous disease
- E79.1Lesch-Nyhan syndrome
- E79.2Myoadenylate deaminase deficiency
- E79.8Other disorders of purine and pyrimidine metabolism
- E79.81Aicardi-Goutieres syndrome
- E79.82Hereditary xanthinuria
- E79.89Other specified disorders of purine and pyrimidine metabolism
- E79.9Disorder of purine and pyrimidine metabolism, unspecified
- E80Disorders of porphyrin and bilirubin metabolism
- E80.0Hereditary erythropoietic porphyria
- E80.1Porphyria cutanea tarda
- E80.2Other and unspecified porphyria
- E80.20Unspecified porphyria
- E80.21Acute intermittent (hepatic) porphyria
- E80.29Other porphyria
- E80.3Defects of catalase and peroxidase
- E80.4Gilbert syndrome
- E80.5Crigler-Najjar syndrome
- E80.6Other disorders of bilirubin metabolism
- E80.7Disorder of bilirubin metabolism, unspecified
- E83Disorders of mineral metabolism
- E83.0Disorders of copper metabolism
- E83.00Disorder of copper metabolism, unspecified
- E83.01Wilson's disease
- E83.09Other disorders of copper metabolism
- E83.1Disorders of iron metabolism
- E83.10Disorder of iron metabolism, unspecified
- E83.11Hemochromatosis
- E83.110Hereditary hemochromatosis
- E83.111Hemochromatosis due to repeated red blood cell transfusions
- E83.118Other hemochromatosis
- E83.119Hemochromatosis, unspecified
- E83.19Other disorders of iron metabolism
- E83.2Disorders of zinc metabolism
- E83.3Disorders of phosphorus metabolism and phosphatases
- E83.30Disorder of phosphorus metabolism, unspecified
- E83.31Familial hypophosphatemia
- E83.32Hereditary vitamin D-dependent rickets (type 1) (type 2)
- E83.39Other disorders of phosphorus metabolism
- E83.4Disorders of magnesium metabolism
- E83.40Disorders of magnesium metabolism, unspecified
- E83.41Hypermagnesemia
- E83.42Hypomagnesemia
- E83.49Other disorders of magnesium metabolism
- E83.5Disorders of calcium metabolism
- E83.50Unspecified disorder of calcium metabolism
- E83.51Hypocalcemia
- E83.52Hypercalcemia
- E83.59Other disorders of calcium metabolism
- E83.8Other disorders of mineral metabolism
- E83.81Hungry bone syndrome
- E83.82Disorders of pyrophosphate metabolism
- E83.820Generalized arterial calcification of infancy with unspecified genetic causality
- E83.821ENPP1 deficiency causing generalized arterial calcification of infancy
- E83.822ENPP1 deficiency causing autosomal recessive hypophosphatemic rickets type 2
- E83.823ABCC6 deficiency causing generalized arterial calcification of infancy
- E83.824ABCC6 deficiency causing pseudoxanthoma elasticum
- E83.825CD73 deficiency causing arterial calcification
- E83.89Other disorders of mineral metabolism
- E83.9Disorder of mineral metabolism, unspecified
- E84Cystic fibrosis
- E84.0Cystic fibrosis with pulmonary manifestations
- E84.1Cystic fibrosis with intestinal manifestations
- E84.11Meconium ileus in cystic fibrosis
- E84.19Cystic fibrosis with other intestinal manifestations
- E84.8Cystic fibrosis with other manifestations
- E84.9Cystic fibrosis, unspecified
- E85Amyloidosis
- E85.0Non-neuropathic heredofamilial amyloidosis
- E85.1Neuropathic heredofamilial amyloidosis
- E85.2Heredofamilial amyloidosis, unspecified
- E85.3Secondary systemic amyloidosis
- E85.4Organ-limited amyloidosis
- E85.8Other amyloidosis
- E85.81Light chain (AL) amyloidosis
- E85.82Wild-type transthyretin-related (ATTR) amyloidosis
- E85.89Other amyloidosis
- E85.9Amyloidosis, unspecified
- E86Volume depletion
- E86.0Dehydration
- E86.1Hypovolemia
- E86.9Volume depletion, unspecified
- E87Other disorders of fluid, electrolyte and acid-base balance
- E87.0Hyperosmolality and hypernatremia
- E87.1Hypo-osmolality and hyponatremia
- E87.2Acidosis
- E87.20Acidosis, unspecified
- E87.21Acute metabolic acidosis
- E87.22Chronic metabolic acidosis
- E87.29Other acidosis
- E87.3Alkalosis
- E87.4Mixed disorder of acid-base balance
- E87.5Hyperkalemia
- E87.6Hypokalemia
- E87.7Fluid overload
- E87.70Fluid overload, unspecified
- E87.71Transfusion associated circulatory overload
- E87.79Other fluid overload
- E87.8Other disorders of electrolyte and fluid balance, not elsewhere classified
- E88Other and unspecified metabolic disorders
- E88.0Disorders of plasma-protein metabolism, not elsewhere classified
- E88.01Alpha-1-antitrypsin deficiency
- E88.02Plasminogen deficiency
- E88.09Other disorders of plasma-protein metabolism, not elsewhere classified
- E88.1Lipodystrophy, not elsewhere classified
- E88.10Lipodystrophy, unspecified
- E88.11Partial lipodystrophy
- E88.12Generalized lipodystrophy
- E88.13Localized lipodystrophy
- E88.14HIV-associated lipodystrophy
- E88.19Other lipodystrophy, not elsewhere classified
- E88.2Lipomatosis, not elsewhere classified
- E88.3Tumor lysis syndrome
- E88.4Mitochondrial metabolism disorders
- E88.40Mitochondrial metabolism disorder, unspecified
- E88.41MELAS syndrome
- E88.42MERRF syndrome
- E88.43Disorders of mitochondrial tRNA synthetases
- E88.49Other mitochondrial metabolism disorders
- E88.8Other specified metabolic disorders
- E88.81Metabolic syndrome and other insulin resistance
- E88.810Metabolic syndrome
- E88.811Insulin resistance syndrome, Type A
- E88.818Other insulin resistance
- E88.819Insulin resistance, unspecified
- E88.82Obesity due to disruption of MC4R pathway
- E88.89Other specified metabolic disorders
- E88.9Metabolic disorder, unspecified
- E88.AWasting disease (syndrome) due to underlying condition
E70-E88 MS-DRGs
This code does not group to an MS-DRG as a principal diagnosis.
Other codes that refer to E70-E88
Notes on these codes point to E70-E88.
- CH17 Excludes2: inborn errors of metabolism (E70-E88)
- G11 Excludes2: metabolic disorders (E70-E88)
- G63 Code First: metabolic diseases (E70-E88)
- G71 Excludes2: metabolic disorders (E70-E88)
- H42 Code First: specified metabolic disorder (E70-E88)
- N25.0 Excludes2: metabolic disorders classifiable to E70-E88
- N25.81 Excludes2: metabolic disorders classifiable to E70-E88
- P59 Excludes1: jaundice due to inborn errors of metabolism (E70-E88)
About the condition
Plain-language background from MedlinePlus (National Library of Medicine), not coding guidance.
Metabolic Disorders
Metabolism is the process your body uses to get or make energy from the food you eat. Food is made up of proteins, carbohydrates, and fats. Chemicals in your digestive system break the food parts down into sugars and acids, your body's fuel. Your body can use this fuel right away, or it can store the energy in your body tissues, such as your liver, muscles, and body fat.
Read more about metabolic disorders on MedlinePlus