AHA Coding Clinic® for ICD-10-CM and ICD-10-PCS - 2023 Issue 4; New/Revised ICD-10-CM Codes

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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