2025 ICD-10-CM Diagnosis Code E77.0

Defects in post-translational modification of lysosomal enzymes

• E77.0 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
• Short description: Defects in post-translational mod of lysosomal enzymes
• The 2025 edition of ICD-10-CM E77.0 became effective on October 1, 2024.
• This is the American ICD-10-CM version of E77.0 – other international versions of ICD-10 E77.0 may differ.

Applicable To

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

The following code(s) above E77.0 contain annotation back-references

 that may be applicable to E77.0:

• E00-E89  Endocrine, nutritional and metabolic diseases
• E70-E88  Metabolic disorders

Approximate Synonyms

• I-cell disease
• Pseudo-hurler polydystrophy

ICD-10-CM E77.0 is grouped within Diagnostic Related Group(s) (MS-DRG v42.0):

• 642 Inborn and other disorders of metabolism

Convert E77.0 to ICD-9-CMCode 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
• 2024 (effective 10/1/2023): No change
• 2025 (effective 10/1/2024): No change

Code annotations containing back-references to E77.0:

• Type 1 Excludes: E75

Diagnosis Index entries containing back-references to E77.0:

• Defect, defectiveQ89.9
  • lysosomal enzyme, post-translational modification E77.0
  • modification, lysosomal enzymes, post-translational E77.0
• Disease, diseased – see also Syndrome
  • I-cell E77.0
  • pseudo-Hurler’s E77.0
• Mucolipidosis
  • II, III E77.0
• Polydystrophy, pseudo-Hurler E77.0
• Pseudo-Hurler’s polydystrophy E77.0