E77.0 Defects in post-translational modification of lysosomal enzymes

The ICD-10-CM code for Defects in post-translational modification of lysosomal enzymes is E77.0 (FY2026). It is a billable, claim-ready diagnosis code.

Classification

Chapter

4: Endocrine, nutritional and metabolic diseases (E00-E89)

Section

E70-E88: Metabolic disorders (E70-E88)

Category E77

5 codes (4 billable)

FY2026 Status

Stable since FY2024

Also Known As

ICD-10-CM Alphabetic Index entries that lead to E77.0:

• Pseudo-Hurler's polydystrophy
• Polydystrophy, pseudo-Hurler
• Mucolipidosis › II, III
• Disease, diseased › pseudo-Hurler's
• Defect, defective › modification, lysosomal enzymes, post-translational
• Disease, diseased › I-cell
• Defect, defective › lysosomal enzyme, post-translational modification

Inclusion Terms

• Mucolipidosis II [I-cell disease]
• Mucolipidosis III [pseudo-Hurler polydystrophy]

U.S. Hospital Utilization

• An estimated 125 U.S. inpatient stays in 2023 included E77.0 among the documented diagnoses.

Source: National Inpatient Sample (NIS), Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality, 2016–2023. National survey-weighted estimates.

Official Coding Guidelines

Diabetes mellitus (TOC entry)

a. Diabetes mellitus ................................................................................................................... 39

Obesity (TOC entry)

b. Obesity .................................................................................................................................. 42

Source: CMS — ICD-10-CM Official Guidelines for Coding and Reporting, FY2026

References

• CMS — ICD-10-CM Official Code Set and Guidelines, FY2026
• CDC/NCHS — ICD-10-CM Files and Browser Tool
• AHRQ — HCUP National Inpatient Sample, Frequencies by Diagnosis Code (2016–2023)