Chapter 4

E00–E89: Endocrine, Nutritional and Metabolic Diseases

Endocrine, Nutritional and Metabolic Diseases diagnostic classification updated for 2026.

Chapter Instructions

Excludes1(Not coded here. Use a different code.)

transitory endocrine and metabolic disorders specific to newborn (P70-P74)

All Codes Billable Only

Internal Codes

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E75.19|
Other gangliosidosis
E75.2|
Other sphingolipidosis
E75.21|
Fabry (-Anderson) disease
E75.22|
Gaucher disease
E75.23|
Krabbe disease
E75.24|
Niemann-Pick disease
E75.240|
Niemann-Pick disease type A
E75.241|
Niemann-Pick disease type B
E75.242|
Niemann-Pick disease type C
E75.243|
Niemann-Pick disease type D
E75.244|
Niemann-Pick disease type A/B
E75.248|
Other Niemann-Pick disease
E75.249|
Niemann-Pick disease, unspecified
E75.25|
Metachromatic leukodystrophy
E75.26|
Sulfatase deficiency
E75.27|
Pelizaeus-Merzbacher disease
E75.28|
Canavan disease
E75.29|
Other sphingolipidosis
E75.3|
Sphingolipidosis, unspecified
E75.4|
Neuronal ceroid lipofuscinosis
E75.5|
Other lipid storage disorders
E75.6|
Lipid storage disorder, unspecified
E76|
Disorders of glycosaminoglycan metabolism
E76.0|
Mucopolysaccharidosis, type I
E76.01|
Hurler's syndrome
E76.02|
Hurler-Scheie syndrome
E76.03|
Scheie's syndrome
E76.1|
Mucopolysaccharidosis, type II
E76.2|
Other mucopolysaccharidoses
E76.21|
Morquio mucopolysaccharidoses
E76.210|
Morquio A mucopolysaccharidoses
E76.211|
Morquio B mucopolysaccharidoses
E76.219|
Morquio mucopolysaccharidoses, unspecified
E76.22|
Sanfilippo mucopolysaccharidoses
E76.29|
Other mucopolysaccharidoses
E76.3|
Mucopolysaccharidosis, unspecified
E76.8|
Other disorders of glucosaminoglycan metabolism
E76.9|
Glucosaminoglycan metabolism disorder, unspecified
E77|
Disorders of glycoprotein metabolism
E77.0|
Defects in post-translational modification of lysosomal enzymes
E77.1|
Defects in glycoprotein degradation
E77.8|
Other disorders of glycoprotein metabolism
E77.9|
Disorder of glycoprotein metabolism, unspecified
E78|
Disorders of lipoprotein metabolism and other lipidemias
E78.0|
Pure hypercholesterolemia
E78.00|
Pure hypercholesterolemia, unspecified
E78.01|
Familial hypercholesterolemia
E78.010|
Homozygous familial hypercholesterolemia [HoFH]
E78.011|
Heterozygous familial hypercholesterolemia [HeFH]
E78.019|
Familial hypercholesterolemia, unspecified

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