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Chapter 4: Endocrine, nutritional and metabolic diseases
E70-E88

Metabolic disorders

Explore comprehensive insights into ICD-10 codes E70-E88, covering metabolic disorders. Enhance clinical documentation with precise coding specificity for conditions like phenylketonuria and lipid metabolism disorders. Ideal for healthcare professionals seeking detailed coding guidance and improved patient record accuracy.

About This Range
342+
Total codes
267+
Billable codes
75+
Non-billable codes
4
Chapters
Codes in this Range (342+)
Chapter 4: Endocrine, nutritional and metabolic diseases Range: E00-E89
E70
Disorders of aromatic amino-acid metabolism
E70.0
Classical phenylketonuria
Billable
E70.1
Other hyperphenylalaninemias
Billable
E70.2
Disorders of tyrosine metabolism
E70.20
Disorder of tyrosine metabolism, unspecified
Billable
E70.21
Tyrosinemia
Billable
E70.29
Other disorders of tyrosine metabolism
Billable
E70.3
Albinism
E70.30
Albinism, unspecified
Billable
E70.31
Ocular albinism
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
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
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
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
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
E71.0
Maple-syrup-urine disease
Billable
E71.1
Other disorders of branched-chain amino-acid metabolism
E71.11
Branched-chain organic acidurias
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
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
E71.30
Disorder of fatty-acid metabolism, unspecified
Billable
E71.31
Disorders of fatty-acid oxidation
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
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
E71.440
Ruvalcaba-Myhre-Smith syndrome
Billable
E71.448
Other secondary carnitine deficiency
Billable
E71.5
Peroxisomal disorders
E71.50
Peroxisomal disorder, unspecified
Billable
E71.51
Disorders of peroxisome biogenesis
E71.510
Zellweger syndrome
Billable
E71.511
Neonatal adrenoleukodystrophy
Billable
E71.518
Other disorders of peroxisome biogenesis
Billable
E71.52
X-linked adrenoleukodystrophy
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
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
E72.0
Disorders of amino-acid transport
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
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
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
E72.50
Disorder of glycine metabolism, unspecified
Billable
E72.51
Non-ketotic hyperglycinemia
Billable
E72.52
Trimethylaminuria
Billable
E72.53
Primary hyperoxaluria
Billable
E72.59
Other disorders of glycine metabolism
Billable
E72.8
Other specified disorders of amino-acid metabolism
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
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
E74.0
Glycogen storage disease
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
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
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
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
E74.81
Disorders of glucose transport, not elsewhere classified
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
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
E75.0
GM2 gangliosidosis
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
E75.10
Unspecified gangliosidosis
Billable
E75.11
Mucolipidosis IV
Billable
E75.19
Other gangliosidosis
Billable
E75.2
Other sphingolipidosis
E75.21
Fabry (-Anderson) disease
Billable
E75.22
Gaucher disease
Billable
E75.23
Krabbe disease
Billable
E75.24
Niemann-Pick disease
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
E76.0
Mucopolysaccharidosis, type I
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
E76.21
Morquio mucopolysaccharidoses
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
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
E78.0
Pure hypercholesterolemia
E78.00
Pure hypercholesterolemia, unspecified
Billable
E78.01
Familial hypercholesterolemia
Billable
E78.1
Pure hyperglyceridemia
Billable
E78.2
Mixed hyperlipidemia
Billable
E78.3
Hyperchylomicronemia
Billable
E78.4
Other hyperlipidemia
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
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
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
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
E79.81
Aicardi-Goutières 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
E80.0
Hereditary erythropoietic porphyria
Billable
E80.1
Porphyria cutanea tarda
Billable
E80.2
Other and unspecified porphyria
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
E83.0
Disorders of copper metabolism
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
E83.10
Disorder of iron metabolism, unspecified
Billable
E83.11
Hemochromatosis
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
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
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
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
E83.81
Hungry bone syndrome
Billable
E83.89
Other disorders of mineral metabolism
Billable
E83.9
Disorder of mineral metabolism, unspecified
Billable
E84
Cystic fibrosis
E84.0
Cystic fibrosis with pulmonary manifestations
Billable
E84.1
Cystic fibrosis with intestinal manifestations
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
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
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
E86.0
Dehydration
Billable
E86.1
Hypovolemia
Billable
E86.9
Volume depletion, unspecified
Billable
E87
Other disorders of fluid, electrolyte and acid-base balance
E87.0
Hyperosmolality and hypernatremia
Billable
E87.1
Hypo-osmolality and hyponatremia
Billable
E87.2
Acidosis
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
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
E88.0
Disorders of plasma-protein metabolism, not elsewhere classified
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
Billable
E88.2
Lipomatosis, not elsewhere classified
Billable
E88.3
Tumor lysis syndrome
Billable
E88.4
Mitochondrial metabolism disorders
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
E88.81
Metabolic syndrome and other insulin resistance
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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Frequently Asked Questions
What are common symptoms of metabolic disorders in the ICD-10 code range E70-E88?

Common symptoms of metabolic disorders, such as those in the ICD-10 code range E70-E88, include fatigue, weight changes, muscle weakness, and developmental delays. These symptoms vary depending on the specific disorder, such as phenylketonuria or glycogen storage diseases.

How are metabolic disorders diagnosed according to ICD-10 guidelines?

Metabolic disorders in the ICD-10 code range E70-E88 are diagnosed using a combination of clinical evaluation, blood tests, genetic testing, and metabolic screening. Early diagnosis is crucial for effective management and treatment.

What treatment options are available for metabolic disorders classified under ICD-10 codes E70-E88?

Treatment for metabolic disorders in the ICD-10 range E70-E88 often includes dietary management, enzyme replacement therapy, and medications to manage symptoms. The specific treatment plan depends on the type of disorder, such as galactosemia or hyperlipidemia.

Metabolic disorders