Lysosome-Associated Membrane Protein 2 [LAMP2] Deficiency

Subcategory E74.0, Glycogen storage disease, has been expanded with the creation of code E74.05, Lysosome-associated membrane protein 2 [LAMP2] deficiency. LAMP2 deficiency, otherwise known as Danon disease, is a rare X-linked dominant metabolic disorder. It is caused by a genetic mutation in the lysosome-associated membrane protein 2 (LAMP2) gene, resulting in a deficiency of the LAMP2 protein.Danon disease is a multi-system disorder that involves the heart, skeletal muscles, central nervous system, and retina. Symptoms may include hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), and musculoskeletal weakness. Current management focuses on surveillance and symptom management, and heart...

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Note:  The following article synopsis was NOT provided by the AHA. It was created by Find-A-Code/innoviHealth.

Article Overview

This article covers an ICD-10-CM update related to a rare inherited metabolic disorder also known as Danon disease. It is useful for coders, CDI specialists, and clinicians who need to understand the affected code category, the broader clinical picture, and the type of documentation or coding update being discussed. The article provides a brief overview of the condition, its multisystem involvement, and general management considerations.

Why This Topic Matters

It helps readers recognize a newly expanded diagnostic category in ICD-10-CM and understand the clinical context behind the update so records can be reviewed and coded accurately.

What You Will Learn

  • What condition is associated with the newly added diagnostic code
  • How the disorder is generally categorized clinically
  • Which body systems and symptom areas are commonly discussed in relation to the condition
  • What broad management themes are associated with the disorder

Who Should Read This

  • Medical coders
  • Coding auditors
  • Clinical documentation improvement specialists
  • Physicians
  • Health information management professionals

Codes Discussed

  • ICD-10-CM: E74.0
  • ICD-10-CM: E74.05

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