Cori disease

This code identifies Cori disease, also known as Glycogen Storage Disease Type III (GSD III). It is an autosomal recessive metabolic disorder characterized by a deficiency of the glycogen debranching enzyme, leading to abnormal glycogen accumulation in the liver, muscle, and heart. Patients typically present with hepatomegaly, hypoglycemia, and muscle weakness.

Use this code for patients diagnosed with Cori disease based on clinical presentation, biochemical testing (e.g., enzyme assay, genetic testing), and/or liver/muscle biopsy findings. Documentation should clearly state the diagnosis of Glycogen Storage Disease Type III or Cori disease.

• Differentiate from other glycogen storage diseases; ensure documentation specifies Type III.
• Consider sequencing this code as the primary diagnosis when it is the reason for the encounter.
• Look for associated manifestations such as hypoglycemia (E16.2), hepatomegaly (R16.0), or myopathy (G72.41) to code concurrently.