E74.01
Billablevon Gierke disease
Last updated: FY2026 ICD-10-CM (Oct 1, 2025 – Sep 30, 2026) | CMS-HCC V28 (100% phase-in, PY2026)
Is E74.01 an HCC code?
Yes. E74.01 maps to Glycogen/Amino-Acid/Other Metabolic Disorders under the CMS-HCC V28 risk adjustment model (and Other Significant Endocrine and Metabolic Disorders under V24).
HCC Category Mapping
RAF weights shown are the community, non-dual, aged base weights from the CMS risk adjustment model file. Actual per-patient RAF contribution depends on member segment, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains in use during the transition and for historical data.
MEAT Criteria for E74.01
For E74.01 to count as a valid HCC diagnosis in a given encounter, the provider's documentation must show MEAT: Monitor, Evaluate, Assess, or Treat. A diagnosis from a prior year does not carry forward automatically — it has to be re-documented and supported each calendar year.
- MMonitor: signs, symptoms, disease progression, or lab trending documented in the note
- EEvaluate: test results, medication response, or physical findings reviewed by the provider
- AAssess: explicit mention in the assessment or plan with acknowledgment of status
- TTreat: medication, referral, procedure, therapy, or counseling tied to the diagnosis
Only one of M/E/A/T is required to support the code, but the documentation must be specific enough to show that the provider actually addressed E74.01 during that encounter — not just copy-forwarded from a problem list.
What This Code Means
E74.01 is the ICD-10-CM diagnosis code for von gierke disease. A rare inherited metabolic disorder where the body cannot break down glucose-6-phosphate, leading to severe low blood sugar, liver enlargement, and growth problems. E74.01 sits in the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89), within the section covering metabolic disorders (e70-e88).
Under the CMS-HCC V28 risk adjustment model, E74.01 maps to Glycogen/Amino-Acid/Other Metabolic Disorders (HCC 50) with a community, non-dual, aged base RAF weight of 0.289. Under the older CMS-HCC V24 model, E74.01 maps to Other Significant Endocrine and Metabolic Disorders (HCC 23) with a community, non-dual, aged base RAF weight of 0.230. V28 is the CMS-HCC risk adjustment model that reached 100% phase-in for payment year 2026, replacing V24 which was used during the PY2024–PY2025 transition.
This is the most severe form of glycogen storage disease; document presence of hepatomegaly, hypoglycemia, and lactic acidosis. Because E74.01 maps to a payment HCC, the provider's documentation must satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) for the encounter to count toward the patient's Medicare Advantage risk adjustment score. When documentation is ambiguous, coders should issue a provider query rather than assume the highest-specificity variant.
HCC Buddy maintains structured V28 and V24 mapping, RAF weights, and MEAT documentation criteria for E74.01 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.
Coding Tips
- •This is the most severe form of glycogen storage disease; document presence of hepatomegaly, hypoglycemia, and lactic acidosis
- •Genetic testing confirmation should be documented when available
Clinical Significance
Von Gierke disease (Glycogen Storage Disease Type Ia) is caused by glucose-6-phosphatase deficiency, leading to severe hypoglycemia, hepatomegaly, growth failure, lactic acidosis, and hyperlipidemia. Without continuous glucose management, patients face life-threatening metabolic crises. This is the most common and one of the most severe forms of glycogen storage disease, requiring complex lifelong dietary management.
Documentation Requirements
- ✓Confirmed diagnosis of von Gierke disease or GSD Type Ia
- ✓Enzyme assay or genetic testing confirming glucose-6-phosphatase deficiency
- ✓Documentation of hepatomegaly on physical exam or imaging
- ✓Laboratory values including fasting glucose levels, lactate, uric acid, and lipid panel
- ✓Current dietary management plan including cornstarch therapy or continuous glucose feeds
Commonly Confused Codes
- •E74.00 — Glycogen storage disease, unspecified: do not use when von Gierke is confirmed
- •E74.03 — Cori disease (Type III): also causes hepatomegaly and hypoglycemia but involves debranching enzyme
- •E74.09 — Other glycogen storage disease: for GSD types without specific codes (IV, VI, IX, etc.)
- •E16.2 — Hypoglycemia, unspecified: hypoglycemia is a manifestation of GSD I, not the primary diagnosis
- •E74.02 — Pompe disease: affects muscles/heart primarily, not liver-predominant like von Gierke