2023 ICD-10-CM Diagnosis Code E77.1

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Defects in glycoprotein degradation

    2016 2017 2018 2019 2020 2021 2022 2023 Billable/Specific Code
  • E77.1 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2023 edition of ICD-10-CM E77.1 became effective on October 1, 2022.
  • This is the American ICD-10-CM version of E77.1 - other international versions of ICD-10 E77.1 may differ.
Applicable To
  • Aspartylglucosaminuria
  • Fucosidosis
  • Mannosidosis
  • Sialidosis [mucolipidosis I]
The following code(s) above E77.1 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to E77.1:
  • E00-E89
    2023 ICD-10-CM Range E00-E89

    Endocrine, nutritional and metabolic diseases

    • All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.
    Type 1 Excludes
    • transitory endocrine and metabolic disorders specific to newborn (P70-P74)
    Endocrine, nutritional and metabolic diseases
  • E70-E88
    2023 ICD-10-CM Range E70-E88

    Metabolic disorders

    Type 1 Excludes
    • androgen insensitivity syndrome (E34.5-)
    • congenital adrenal hyperplasia (E25.0)
    • hemolytic anemias attributable to enzyme disorders (D55.-)
    • Marfan's syndrome (Q87.4)
    • 5-alpha-reductase deficiency (E29.1)
    Type 2 Excludes
    • Ehlers-Danlos syndromes (Q79.6-)
    Metabolic disorders
Approximate Synonyms
  • Fucosidosis
  • Mannosidosis
  • Sialidosis
Clinical Information
  • A rare autosomal recessive lysosomal disorder characterized by deficiency of n-aspartyl-beta-glucosaminidase. It is characterized by developmental delays during childhood.
  • A rare autosomal recessive lysosomal storage disease characterized by a deficient activity of the enzymes alpha-d-mannosidase or beta-mannosidase. Clincal signs and symptoms include hepatomegaly, splenomegaly, hearing loss, mental retardation, skeletal abnormalities, and recurrent respiratory infections.
  • A recessively inherited, progressive lysosomal storage disease caused by a deficiency of glycosylasparaginase activity. The lack of this enzyme activity results in the accumulation of n-acetylglucosaminylasparagine (the linkage unit of asparagine-linked glycoproteins) in lysosomes.
  • An autosomal recessive lysosomal storage disease caused by a deficiency of alpha-l-fucosidase activity resulting in an accumulation of fucose containing sphingolipids; glycoproteins, and mucopolysaccharides (glycosaminoglycans) in lysosomes. The infantile form (type i) features psychomotor deterioration, muscle spasticity, coarse facial features, growth retardation, skeletal abnormalities, visceromegaly, seizures, recurrent infections, and macroglossia, with death occurring in the first decade of life. Juvenile fucosidosis (type ii) is the more common variant and features a slowly progressive decline in neurologic function and angiokeratoma corporis diffusum. Type ii survival may be through the fourth decade of life. (from menkes, textbook of child neurology, 5th ed, p87; am j med genet 1991 jan;38(1):111-31)
  • An autosomal recessive lysosomal storage disease characterized by a defective alpha-l-fucosidase. It results in accumulation of fucose in the tissues. Signs and symptoms include psychomotor retardation, dysostosis multiplex, and neural disturbances.
  • An inborn error of metabolism caused by deficient activity of the enzyme aspartylglucosaminidase. Clinical manifestations consist of psychomotor retardation, coarse facies, hepatosplenomegaly, ventral hernia, and skeletal abnormalities.
  • Diseases caused by the loss of one or more enzymes involved in the hydrolysis of mannoside linkages (mannosidases). The defects in enzyme activity are primarily associated with genetic mutation of the genes that codes for a particular mannosidase isoenzyme.
  • Lysosomal storage disease caused by defective alpha-l-fucosidase and accumulation of fucose containing glycoconjugates; clinical symptoms include psychomotor deterioration, growth retardation, hepatosplenomegaly, cardiomegaly, and seizures.
  • Lysosomal storage disease due to defective alpha-mannosidase with resultant oligosaccharide accumulation.
  • Lysosome storage disease due to alpha-l-fucosidase (e.c. deficiency in leukocytes manifested by abnormal accumulation in tissues and urinary excretion of partially catabolized oligosaccharides, glycoasparagines, and glycolipids with alpha-linked fucose at the nonreducing end of the glycogen chain. The phenotype is variable and may include delayed growth and mental development, progressive neurological deterioration, hurler-like (mucopolysaccharidosis i-h) coarse facies, recurrent infections, visceromegaly, skeletal abnormalities, joint contractures, deafness, and angiokeratoma corporis diffusum. Several types are recognized by different researchers. The form exhibiting a longer survival, mild neurological manifestations, and angiokeratoma is sometimes referred to as fucosidosis type ii. In a different scheme, three different types are recognized according to their age of onset. Types i and ii are the most severe and have their onsets at 10 and 18 months, respectively with life expectancy of 6 years. Type iii represents a juvenile form which is marked by a milder form of psychomotor retardation and a slower deterioration of neurological activities. Hurler-like (gargyloid) facies occur mainly in types i and ii and is less commonly in type iii.
ICD-10-CM E77.1 is grouped within Diagnostic Related Group(s) (MS-DRG v40.0):
  • 642 Inborn and other disorders of metabolism

Convert E77.1 to ICD-9-CM

Code History
  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change
  • 2021 (effective 10/1/2020): No change
  • 2022 (effective 10/1/2021): No change
  • 2023 (effective 10/1/2022): No change
Code annotations containing back-references to E77.1:
  • Type 1 Excludes: E75
    ICD-10-CM Diagnosis Code E75

    Disorders of sphingolipid metabolism and other lipid storage disorders

      2016 2017 2018 2019 2020 2021 2022 2023 Non-Billable/Non-Specific Code
    Type 1 Excludes

Diagnosis Index entries containing back-references to E77.1:
  • Aspartylglucosaminuria E77.1
  • Defect, defective Q89.9
    ICD-10-CM Diagnosis Code Q89.9

    Congenital malformation, unspecified

      2016 2017 2018 2019 2020 2021 2022 2023 Billable/Specific Code POA Exempt
    Applicable To
    • Congenital anomaly NOS
    • Congenital deformity NOS
    • degradation, glycoprotein E77.1
    • glycoprotein degradation E77.1
  • Fucosidosis E77.1
  • Mannosidosis E77.1
  • Mucolipidosis
    • I E77.1
  • Sialidosis E77.1

ICD-10-CM Codes Adjacent To E77.1
E76.210 Morquio A mucopolysaccharidoses
E76.211 Morquio B mucopolysaccharidoses
E76.219 …… 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 …… unspecified
E78.01 Familial hypercholesterolemia
E78.1 Pure hyperglyceridemia
E78.2 Mixed hyperlipidemia
E78.3 Hyperchylomicronemia
E78.4 Other hyperlipidemia

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.