E77.1 Defects in glycoprotein degradation

Billable ICD-10-CM code, FY2026.

Applicable to

• Aspartylglucosaminuria
• Fucosidosis
• Mannosidosis
• Sialidosis [mucolipidosis I]

Risk adjustment (HCC)

• CMS-HCC V22: HCC 23 — Other Significant Endocrine and Metabolic Disorders
• RxHCC V08: HCC 41 — Lysosomal Storage Disorders
• ESRD V24: HCC 23

MS-DRG index

Defines the logic of DRG(s) 642 in MDC 10.

Approximate ICD-9-CM (GEMs)

• 271.8 Other specified disorders of carbohydrate transport and metabolism (approximate)
• 272.7 Lipidoses (approximate)

Code annotations containing back-references to E77.1

Back-references to E77.1

• Type 1 Excludes: E75 Disorders of sphingolipid metabolism and other lipid storage disorders ("mucolipidosis, types I-III (E77.0-E77.1)")

Back-references to E77

• Type 2 Excludes: G11 Hereditary ataxia ("metabolic disorders (E70-E88)")
• Code First: G63 Polyneuropathy in diseases classified elsewhere ("metabolic diseases (E70-E88)")
• Type 2 Excludes: G71 Primary disorders of muscles ("metabolic disorders (E70-E88)")
• Code First: H42 Glaucoma in diseases classified elsewhere ("specified metabolic disorder (E70-E88)")
• Type 2 Excludes: N25.0 Renal osteodystrophy ("metabolic disorders classifiable to E70-E88")
• Type 2 Excludes: N25.81 Secondary hyperparathyroidism of renal origin ("metabolic disorders classifiable to E70-E88")
• Type 1 Excludes: P59 Neonatal jaundice from other and unspecified causes ("jaundice due to inborn errors of metabolism (E70-E88)")

Diagnosis Index entries for E77.1

• Aspartylglucosaminuria
• Defect, defective › degradation, glycoprotein
• Defect, defective › glycoprotein degradation
• Fucosidosis
• Mannosidosis
• Mucolipidosis › I
• Sialidosis

Nearby codes

• E77 Disorders of glycoprotein metabolism
• E77.0 Defects in post-translational modification of lysosomal enzymes
• E77.1 Defects in glycoprotein degradation (this code)
• E77.8 Other disorders of glycoprotein metabolism
• E77.9 Disorder of glycoprotein metabolism, unspecified