ICD-10-CM Section · E70-E88
Metabolic disorders
366 codes in this section
366
Total Codes
286
Billable
80
Header (Non-Billable)
17
Top-Level Codes
About Section E70-E88
Section E70-E88 (E70-E88) covers metabolic disorders. It sits within the Endocrine, nutritional and metabolic diseases chapter (E00-E89). This section contains 366 ICD-10-CM codes, 286 billable diagnoses you can submit on a claim, and 80 non-billable header codes used to organize the hierarchy.
Codes are organized below by 3-character root. Click the root to see all subcodes underneath it, or click any individual code for full coverage data, payer policies, and linked CPT procedures.
Codes in E70-E88
Grouped by 3-character root code. Click any code for full detail.
E70
Disorders of aromatic amino-acid metabolism
33 subcodes
| E70.0 | Classical phenylketonuria | Billable |
| E70.1 | Other hyperphenylalaninemias | Billable |
| E70.2 | Disorders of tyrosine metabolism | Header |
| E70.20 | Disorder of tyrosine metabolism, unspecified | Billable |
| E70.21 | Tyrosinemia | Billable |
| E70.29 | Other disorders of tyrosine metabolism | Billable |
| E70.3 | Albinism | Header |
| E70.30 | Albinism, unspecified | Billable |
| E70.31 | Ocular albinism | Header |
| E70.310 | X-linked ocular albinism | Billable |
| E70.311 | Autosomal recessive ocular albinism | Billable |
| E70.318 | Other ocular albinism | Billable |
| E70.319 | Ocular albinism, unspecified | Billable |
| E70.32 | Oculocutaneous albinism | Header |
| E70.320 | Tyrosinase negative oculocutaneous albinism | Billable |
| E70.321 | Tyrosinase positive oculocutaneous albinism | Billable |
| E70.328 | Other oculocutaneous albinism | Billable |
| E70.329 | Oculocutaneous albinism, unspecified | Billable |
| E70.33 | Albinism with hematologic abnormality | Header |
| E70.330 | Chediak-Higashi syndrome | Billable |
| E70.331 | Hermansky-Pudlak syndrome | Billable |
| E70.338 | Other albinism with hematologic abnormality | Billable |
| E70.339 | Albinism with hematologic abnormality, unspecified | Billable |
| E70.39 | Other specified albinism | Billable |
| E70.4 | Disorders of histidine metabolism | Header |
| E70.40 | Disorders of histidine metabolism, unspecified | Billable |
| E70.41 | Histidinemia | Billable |
| E70.49 | Other disorders of histidine metabolism | Billable |
| E70.5 | Disorders of tryptophan metabolism | Billable |
| E70.8 | Other disorders of aromatic amino-acid metabolism | Header |
| E70.81 | Aromatic L-amino acid decarboxylase deficiency | Billable |
| E70.89 | Other disorders of aromatic amino-acid metabolism | Billable |
| E70.9 | Disorder of aromatic amino-acid metabolism, unspecified | Billable |
E71
Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
49 subcodes
| E71.0 | Maple-syrup-urine disease | Billable |
| E71.1 | Other disorders of branched-chain amino-acid metabolism | Header |
| E71.11 | Branched-chain organic acidurias | Header |
| E71.110 | Isovaleric acidemia | Billable |
| E71.111 | 3-methylglutaconic aciduria | Billable |
| E71.118 | Other branched-chain organic acidurias | Billable |
| E71.12 | Disorders of propionate metabolism | Header |
| E71.120 | Methylmalonic acidemia | Billable |
| E71.121 | Propionic acidemia | Billable |
| E71.128 | Other disorders of propionate metabolism | Billable |
| E71.19 | Other disorders of branched-chain amino-acid metabolism | Billable |
| E71.2 | Disorder of branched-chain amino-acid metabolism, unspecified | Billable |
| E71.3 | Disorders of fatty-acid metabolism | Header |
| E71.30 | Disorder of fatty-acid metabolism, unspecified | Billable |
| E71.31 | Disorders of fatty-acid oxidation | Header |
| E71.310 | Long chain/very long chain acyl CoA dehydrogenase deficiency | Billable |
| E71.311 | Medium chain acyl CoA dehydrogenase deficiency | Billable |
| E71.312 | Short chain acyl CoA dehydrogenase deficiency | Billable |
| E71.313 | Glutaric aciduria type II | Billable |
| E71.314 | Muscle carnitine palmitoyltransferase deficiency | Billable |
| E71.318 | Other disorders of fatty-acid oxidation | Billable |
| E71.32 | Disorders of ketone metabolism | Billable |
| E71.39 | Other disorders of fatty-acid metabolism | Billable |
| E71.4 | Disorders of carnitine metabolism | Header |
| E71.40 | Disorder of carnitine metabolism, unspecified | Billable |
| E71.41 | Primary carnitine deficiency | Billable |
| E71.42 | Carnitine deficiency due to inborn errors of metabolism | Billable |
| E71.43 | Iatrogenic carnitine deficiency | Billable |
| E71.44 | Other secondary carnitine deficiency | Header |
| E71.440 | Ruvalcaba-Myhre-Smith syndrome | Billable |
| E71.448 | Other secondary carnitine deficiency | Billable |
| E71.5 | Peroxisomal disorders | Header |
| E71.50 | Peroxisomal disorder, unspecified | Billable |
| E71.51 | Disorders of peroxisome biogenesis | Header |
| E71.510 | Zellweger syndrome | Billable |
| E71.511 | Neonatal adrenoleukodystrophy | Billable |
| E71.518 | Other disorders of peroxisome biogenesis | Billable |
| E71.52 | X-linked adrenoleukodystrophy | Header |
| E71.520 | Childhood cerebral X-linked adrenoleukodystrophy | Billable |
| E71.521 | Adolescent X-linked adrenoleukodystrophy | Billable |
| E71.522 | Adrenomyeloneuropathy | Billable |
| E71.528 | Other X-linked adrenoleukodystrophy | Billable |
| E71.529 | X-linked adrenoleukodystrophy, unspecified type | Billable |
| E71.53 | Other group 2 peroxisomal disorders | Billable |
| E71.54 | Other peroxisomal disorders | Header |
| E71.540 | Rhizomelic chondrodysplasia punctata | Billable |
| E71.541 | Zellweger-like syndrome | Billable |
| E71.542 | Other group 3 peroxisomal disorders | Billable |
| E71.548 | Other peroxisomal disorders | Billable |
E72
Other disorders of amino-acid metabolism
38 subcodes
| E72.0 | Disorders of amino-acid transport | Header |
| E72.00 | Disorders of amino-acid transport, unspecified | Billable |
| E72.01 | Cystinuria | Billable |
| E72.02 | Hartnup's disease | Billable |
| E72.03 | Lowe's syndrome | Billable |
| E72.04 | Cystinosis | Billable |
| E72.09 | Other disorders of amino-acid transport | Billable |
| E72.1 | Disorders of sulfur-bearing amino-acid metabolism | Header |
| E72.10 | Disorders of sulfur-bearing amino-acid metabolism, unspecified | Billable |
| E72.11 | Homocystinuria | Billable |
| E72.12 | Methylenetetrahydrofolate reductase deficiency | Billable |
| E72.19 | Other disorders of sulfur-bearing amino-acid metabolism | Billable |
| E72.2 | Disorders of urea cycle metabolism | Header |
| E72.20 | Disorder of urea cycle metabolism, unspecified | Billable |
| E72.21 | Argininemia | Billable |
| E72.22 | Arginosuccinic aciduria | Billable |
| E72.23 | Citrullinemia | Billable |
| E72.29 | Other disorders of urea cycle metabolism | Billable |
| E72.3 | Disorders of lysine and hydroxylysine metabolism | Billable |
| E72.4 | Disorders of ornithine metabolism | Billable |
| E72.5 | Disorders of glycine metabolism | Header |
| E72.50 | Disorder of glycine metabolism, unspecified | Billable |
| E72.51 | Non-ketotic hyperglycinemia | Billable |
| E72.52 | Trimethylaminuria | Billable |
| E72.53 | Primary hyperoxaluria | Header |
| E72.530 | Primary hyperoxaluria, type 1 | Billable |
| E72.538 | Other specified primary hyperoxaluria | Billable |
| E72.539 | Primary hyperoxaluria, unspecified | Billable |
| E72.54 | Secondary hyperoxaluria | Header |
| E72.540 | Dietary hyperoxaluria | Billable |
| E72.541 | Enteric hyperoxaluria | Billable |
| E72.548 | Other secondary hyperoxaluria | Billable |
| E72.549 | Secondary hyperoxaluria, unspecified | Billable |
| E72.59 | Other disorders of glycine metabolism | Billable |
| E72.8 | Other specified disorders of amino-acid metabolism | Header |
| E72.81 | Disorders of gamma aminobutyric acid metabolism | Billable |
| E72.89 | Other specified disorders of amino-acid metabolism | Billable |
| E72.9 | Disorder of amino-acid metabolism, unspecified | Billable |
E73
Lactose intolerance
4 subcodes
| E73.0 | Congenital lactase deficiency | Billable |
| E73.1 | Secondary lactase deficiency | Billable |
| E73.8 | Other lactose intolerance | Billable |
| E73.9 | Lactose intolerance, unspecified | Billable |
E74
Other disorders of carbohydrate metabolism
31 subcodes
| E74.0 | Glycogen storage disease | Header |
| E74.00 | Glycogen storage disease, unspecified | Billable |
| E74.01 | von Gierke disease | Billable |
| E74.02 | Pompe disease | Billable |
| E74.03 | Cori disease | Billable |
| E74.04 | McArdle disease | Billable |
| E74.05 | Lysosome-associated membrane protein 2 [LAMP2] deficiency | Billable |
| E74.09 | Other glycogen storage disease | Billable |
| E74.1 | Disorders of fructose metabolism | Header |
| E74.10 | Disorder of fructose metabolism, unspecified | Billable |
| E74.11 | Essential fructosuria | Billable |
| E74.12 | Hereditary fructose intolerance | Billable |
| E74.19 | Other disorders of fructose metabolism | Billable |
| E74.2 | Disorders of galactose metabolism | Header |
| E74.20 | Disorders of galactose metabolism, unspecified | Billable |
| E74.21 | Galactosemia | Billable |
| E74.29 | Other disorders of galactose metabolism | Billable |
| E74.3 | Other disorders of intestinal carbohydrate absorption | Header |
| E74.31 | Sucrase-isomaltase deficiency | Billable |
| E74.39 | Other disorders of intestinal carbohydrate absorption | Billable |
| E74.4 | Disorders of pyruvate metabolism and gluconeogenesis | Billable |
| E74.8 | Other specified disorders of carbohydrate metabolism | Header |
| E74.81 | Disorders of glucose transport, not elsewhere classified | Header |
| E74.810 | Glucose transporter protein type 1 deficiency | Billable |
| E74.818 | Other disorders of glucose transport | Billable |
| E74.819 | Disorders of glucose transport, unspecified | Billable |
| E74.82 | Disorders of citrate metabolism | Header |
| E74.820 | SLC13A5 Citrate Transporter Disorder | Billable |
| E74.829 | Other disorders of citrate metabolism | Billable |
| E74.89 | Other specified disorders of carbohydrate metabolism | Billable |
| E74.9 | Disorder of carbohydrate metabolism, unspecified | Billable |
E75
Disorders of sphingolipid metabolism and other lipid storage disorders
30 subcodes
| E75.0 | GM2 gangliosidosis | Header |
| E75.00 | GM2 gangliosidosis, unspecified | Billable |
| E75.01 | Sandhoff disease | Billable |
| E75.02 | Tay-Sachs disease | Billable |
| E75.09 | Other GM2 gangliosidosis | Billable |
| E75.1 | Other and unspecified gangliosidosis | Header |
| E75.10 | Unspecified gangliosidosis | Billable |
| E75.11 | Mucolipidosis IV | Billable |
| E75.19 | Other gangliosidosis | Billable |
| E75.2 | Other sphingolipidosis | Header |
| E75.21 | Fabry (-Anderson) disease | Billable |
| E75.22 | Gaucher disease | Billable |
| E75.23 | Krabbe disease | Billable |
| E75.24 | Niemann-Pick disease | Header |
| E75.240 | Niemann-Pick disease type A | Billable |
| E75.241 | Niemann-Pick disease type B | Billable |
| E75.242 | Niemann-Pick disease type C | Billable |
| E75.243 | Niemann-Pick disease type D | Billable |
| E75.244 | Niemann-Pick disease type A/B | Billable |
| E75.248 | Other Niemann-Pick disease | Billable |
| E75.249 | Niemann-Pick disease, unspecified | Billable |
| E75.25 | Metachromatic leukodystrophy | Billable |
| E75.26 | Sulfatase deficiency | Billable |
| E75.27 | Pelizaeus-Merzbacher disease | Billable |
| E75.28 | Canavan disease | Billable |
| E75.29 | Other sphingolipidosis | Billable |
| E75.3 | Sphingolipidosis, unspecified | Billable |
| E75.4 | Neuronal ceroid lipofuscinosis | Billable |
| E75.5 | Other lipid storage disorders | Billable |
| E75.6 | Lipid storage disorder, unspecified | Billable |
E76
Disorders of glycosaminoglycan metabolism
15 subcodes
| E76.0 | Mucopolysaccharidosis, type I | Header |
| E76.01 | Hurler's syndrome | Billable |
| E76.02 | Hurler-Scheie syndrome | Billable |
| E76.03 | Scheie's syndrome | Billable |
| E76.1 | Mucopolysaccharidosis, type II | Billable |
| E76.2 | Other mucopolysaccharidoses | Header |
| E76.21 | Morquio mucopolysaccharidoses | Header |
| E76.210 | Morquio A mucopolysaccharidoses | Billable |
| E76.211 | Morquio B mucopolysaccharidoses | Billable |
| E76.219 | Morquio mucopolysaccharidoses, unspecified | Billable |
| E76.22 | Sanfilippo mucopolysaccharidoses | Billable |
| E76.29 | Other mucopolysaccharidoses | Billable |
| E76.3 | Mucopolysaccharidosis, unspecified | Billable |
| E76.8 | Other disorders of glucosaminoglycan metabolism | Billable |
| E76.9 | Glucosaminoglycan metabolism disorder, unspecified | Billable |
E77
Disorders of glycoprotein metabolism
4 subcodes
| E77.0 | Defects in post-translational modification of lysosomal enzymes | Billable |
| E77.1 | Defects in glycoprotein degradation | Billable |
| E77.8 | Other disorders of glycoprotein metabolism | Billable |
| E77.9 | Disorder of glycoprotein metabolism, unspecified | Billable |
E78
Disorders of lipoprotein metabolism and other lipidemias
23 subcodes
| E78.0 | Pure hypercholesterolemia | Header |
| E78.00 | Pure hypercholesterolemia, unspecified | Billable |
| E78.01 | Familial hypercholesterolemia | Header |
| E78.010 | Homozygous familial hypercholesterolemia [HoFH] | Billable |
| E78.011 | Heterozygous familial hypercholesterolemia [HeFH] | Billable |
| E78.019 | Familial hypercholesterolemia, unspecified | Billable |
| E78.1 | Pure hyperglyceridemia | Billable |
| E78.2 | Mixed hyperlipidemia | Billable |
| E78.3 | Hyperchylomicronemia | Billable |
| E78.4 | Other hyperlipidemia | Header |
| E78.41 | Elevated Lipoprotein(a) | Billable |
| E78.49 | Other hyperlipidemia | Billable |
| E78.5 | Hyperlipidemia, unspecified | Billable |
| E78.6 | Lipoprotein deficiency | Billable |
| E78.7 | Disorders of bile acid and cholesterol metabolism | Header |
| E78.70 | Disorder of bile acid and cholesterol metabolism, unspecified | Billable |
| E78.71 | Barth syndrome | Billable |
| E78.72 | Smith-Lemli-Opitz syndrome | Billable |
| E78.79 | Other disorders of bile acid and cholesterol metabolism | Billable |
| E78.8 | Other disorders of lipoprotein metabolism | Header |
| E78.81 | Lipoid dermatoarthritis | Billable |
| E78.89 | Other lipoprotein metabolism disorders | Billable |
| E78.9 | Disorder of lipoprotein metabolism, unspecified | Billable |
E79
Disorders of purine and pyrimidine metabolism
8 subcodes
| E79.0 | Hyperuricemia without signs of inflammatory arthritis and tophaceous disease | Billable |
| E79.1 | Lesch-Nyhan syndrome | Billable |
| E79.2 | Myoadenylate deaminase deficiency | Billable |
| E79.8 | Other disorders of purine and pyrimidine metabolism | Header |
| E79.81 | Aicardi-Goutieres syndrome | Billable |
| E79.82 | Hereditary xanthinuria | Billable |
| E79.89 | Other specified disorders of purine and pyrimidine metabolism | Billable |
| E79.9 | Disorder of purine and pyrimidine metabolism, unspecified | Billable |
E80
Disorders of porphyrin and bilirubin metabolism
11 subcodes
| E80.0 | Hereditary erythropoietic porphyria | Billable |
| E80.1 | Porphyria cutanea tarda | Billable |
| E80.2 | Other and unspecified porphyria | Header |
| E80.20 | Unspecified porphyria | Billable |
| E80.21 | Acute intermittent (hepatic) porphyria | Billable |
| E80.29 | Other porphyria | Billable |
| E80.3 | Defects of catalase and peroxidase | Billable |
| E80.4 | Gilbert syndrome | Billable |
| E80.5 | Crigler-Najjar syndrome | Billable |
| E80.6 | Other disorders of bilirubin metabolism | Billable |
| E80.7 | Disorder of bilirubin metabolism, unspecified | Billable |
E83
Disorders of mineral metabolism
39 subcodes
| E83.0 | Disorders of copper metabolism | Header |
| E83.00 | Disorder of copper metabolism, unspecified | Billable |
| E83.01 | Wilson's disease | Billable |
| E83.09 | Other disorders of copper metabolism | Billable |
| E83.1 | Disorders of iron metabolism | Header |
| E83.10 | Disorder of iron metabolism, unspecified | Billable |
| E83.11 | Hemochromatosis | Header |
| E83.110 | Hereditary hemochromatosis | Billable |
| E83.111 | Hemochromatosis due to repeated red blood cell transfusions | Billable |
| E83.118 | Other hemochromatosis | Billable |
| E83.119 | Hemochromatosis, unspecified | Billable |
| E83.19 | Other disorders of iron metabolism | Billable |
| E83.2 | Disorders of zinc metabolism | Billable |
| E83.3 | Disorders of phosphorus metabolism and phosphatases | Header |
| E83.30 | Disorder of phosphorus metabolism, unspecified | Billable |
| E83.31 | Familial hypophosphatemia | Billable |
| E83.32 | Hereditary vitamin D-dependent rickets (type 1) (type 2) | Billable |
| E83.39 | Other disorders of phosphorus metabolism | Billable |
| E83.4 | Disorders of magnesium metabolism | Header |
| E83.40 | Disorders of magnesium metabolism, unspecified | Billable |
| E83.41 | Hypermagnesemia | Billable |
| E83.42 | Hypomagnesemia | Billable |
| E83.49 | Other disorders of magnesium metabolism | Billable |
| E83.5 | Disorders of calcium metabolism | Header |
| E83.50 | Unspecified disorder of calcium metabolism | Billable |
| E83.51 | Hypocalcemia | Billable |
| E83.52 | Hypercalcemia | Billable |
| E83.59 | Other disorders of calcium metabolism | Billable |
| E83.8 | Other disorders of mineral metabolism | Header |
| E83.81 | Hungry bone syndrome | Billable |
| E83.82 | Disorders of pyrophosphate metabolism | Header |
| E83.820 | Generalized arterial calcification of infancy with unspecified genetic causality | Billable |
| E83.821 | ENPP1 deficiency causing generalized arterial calcification of infancy | Billable |
| E83.822 | ENPP1 deficiency causing autosomal recessive hypophosphatemic rickets type 2 | Billable |
| E83.823 | ABCC6 deficiency causing generalized arterial calcification of infancy | Billable |
| E83.824 | ABCC6 deficiency causing pseudoxanthoma elasticum | Billable |
| E83.825 | CD73 deficiency causing arterial calcification | Billable |
| E83.89 | Other disorders of mineral metabolism | Billable |
| E83.9 | Disorder of mineral metabolism, unspecified | Billable |
E84
Cystic fibrosis
6 subcodes
| E84.0 | Cystic fibrosis with pulmonary manifestations | Billable |
| E84.1 | Cystic fibrosis with intestinal manifestations | Header |
| E84.11 | Meconium ileus in cystic fibrosis | Billable |
| E84.19 | Cystic fibrosis with other intestinal manifestations | Billable |
| E84.8 | Cystic fibrosis with other manifestations | Billable |
| E84.9 | Cystic fibrosis, unspecified | Billable |
E85
Amyloidosis
10 subcodes
| E85.0 | Non-neuropathic heredofamilial amyloidosis | Billable |
| E85.1 | Neuropathic heredofamilial amyloidosis | Billable |
| E85.2 | Heredofamilial amyloidosis, unspecified | Billable |
| E85.3 | Secondary systemic amyloidosis | Billable |
| E85.4 | Organ-limited amyloidosis | Billable |
| E85.8 | Other amyloidosis | Header |
| E85.81 | Light chain (AL) amyloidosis | Billable |
| E85.82 | Wild-type transthyretin-related (ATTR) amyloidosis | Billable |
| E85.89 | Other amyloidosis | Billable |
| E85.9 | Amyloidosis, unspecified | Billable |
E86
Volume depletion
3 subcodes
| E86.0 | Dehydration | Billable |
| E86.1 | Hypovolemia | Billable |
| E86.9 | Volume depletion, unspecified | Billable |
E87
Other disorders of fluid, electrolyte and acid-base balance
16 subcodes
| E87.0 | Hyperosmolality and hypernatremia | Billable |
| E87.1 | Hypo-osmolality and hyponatremia | Billable |
| E87.2 | Acidosis | Header |
| E87.20 | Acidosis, unspecified | Billable |
| E87.21 | Acute metabolic acidosis | Billable |
| E87.22 | Chronic metabolic acidosis | Billable |
| E87.29 | Other acidosis | Billable |
| E87.3 | Alkalosis | Billable |
| E87.4 | Mixed disorder of acid-base balance | Billable |
| E87.5 | Hyperkalemia | Billable |
| E87.6 | Hypokalemia | Billable |
| E87.7 | Fluid overload | Header |
| E87.70 | Fluid overload, unspecified | Billable |
| E87.71 | Transfusion associated circulatory overload | Billable |
| E87.79 | Other fluid overload | Billable |
| E87.8 | Other disorders of electrolyte and fluid balance, not elsewhere classified | Billable |
E88
Other and unspecified metabolic disorders
29 subcodes
| E88.0 | Disorders of plasma-protein metabolism, not elsewhere classified | Header |
| E88.01 | Alpha-1-antitrypsin deficiency | Billable |
| E88.02 | Plasminogen deficiency | Billable |
| E88.09 | Other disorders of plasma-protein metabolism, not elsewhere classified | Billable |
| E88.1 | Lipodystrophy, not elsewhere classified | Header |
| E88.10 | Lipodystrophy, unspecified | Billable |
| E88.11 | Partial lipodystrophy | Billable |
| E88.12 | Generalized lipodystrophy | Billable |
| E88.13 | Localized lipodystrophy | Billable |
| E88.14 | HIV-associated lipodystrophy | Billable |
| E88.19 | Other lipodystrophy, not elsewhere classified | Billable |
| E88.2 | Lipomatosis, not elsewhere classified | Billable |
| E88.3 | Tumor lysis syndrome | Billable |
| E88.4 | Mitochondrial metabolism disorders | Header |
| E88.40 | Mitochondrial metabolism disorder, unspecified | Billable |
| E88.41 | MELAS syndrome | Billable |
| E88.42 | MERRF syndrome | Billable |
| E88.43 | Disorders of mitochondrial tRNA synthetases | Billable |
| E88.49 | Other mitochondrial metabolism disorders | Billable |
| E88.8 | Other specified metabolic disorders | Header |
| E88.81 | Metabolic syndrome and other insulin resistance | Header |
| E88.810 | Metabolic syndrome | Billable |
| E88.811 | Insulin resistance syndrome, Type A | Billable |
| E88.818 | Other insulin resistance | Billable |
| E88.819 | Insulin resistance, unspecified | Billable |
| E88.82 | Obesity due to disruption of MC4R pathway | Billable |
| E88.89 | Other specified metabolic disorders | Billable |
| E88.9 | Metabolic disorder, unspecified | Billable |
| E88.A | Wasting disease (syndrome) due to underlying condition | Billable |
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