Disorders of glycoprotein metabolism (E77) ICD-10-CM
The E77 code range covers disorders of glycoprotein metabolism with 5 ICD-10-CM diagnosis codes. 4 of them are billable and valid for claim submission in fiscal year 2026, and the category headers group them but cannot themselves be billed.
Codes in the E77 Range 5 codes · 4 billable
Clinical Terms in This Code Range
Definitions from the National Library of Medicine for conditions coded in the E77 range.
Aspartylglucosaminuria
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.
Fucosidosis
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)
About the E77 Code Range
The ICD-10 code section E77 covers disorders of glycoprotein metabolism, which are rare conditions affecting the body's processing of glycoproteins—proteins with sugar chains essential for various cellular functions. These codes are specifically used to classify different inherited and acquired metabolic disorders involving glycoprotein abnormalities.
This section includes codes like E77.0 for defects in post-translational modification of lysosomal enzymes, known by synonyms such as I-cell disease and pseudo-Hurler polydystrophy. It also features E77.1, which addresses disorders involving the breakdown of glycoproteins, including a range of conditions commonly called sialidosis, fucosidosis, and mannosidosis. The E77.8 code captures other varied and often congenital glycoprotein metabolism disorders, such as congenital disorders of glycosylation and carbohydrate-deficient glycoprotein syndromes, which can cause diverse symptoms like hypoproteinemia and anemia. Finally, E77.9 is used when a glycoprotein metabolism disorder is diagnosed but not specified further. Understanding these codes helps both healthcare professionals and coders accurately identify and classify these complex metabolic conditions efficiently.
Questions About This Page
How many billable codes are in the E77 range?
Of the 5 codes in this range, 4 are billable and valid for claim submission from October 1, 2025 through September 30, 2026. Category header codes group them but cannot be reported on claims.
What does the E77 range classify?
The range classifies disorders of glycoprotein metabolism. Each code links to its own reference page with billing status, MS-DRG grouping, coding notes, and clinical information.
Related References
Source: CMS FY 2026 ICD-10-CM Tabular List and order file, effective October 1, 2025 through September 30, 2026.
