2027 ICD-10-CM Diagnosis Code E74.03Cori disease
ICD-10-CM Codes›E00–E89›E70-E88›E74
- Billable — Valid for Submission
- CC — Complication or Comorbidity
- Risk Adjusts — HCC 50
- Chronic Condition
E74.03 is a billable ICD-10-CM diagnosis code for cori disease. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 642. As a secondary diagnosis, it counts as a complication or comorbidity (CC) and moves an inpatient stay to a higher severity level within its MS-DRG family. It does not count, however, when the principal diagnosis is one of 140 closely related codes. Coders also document this condition as autoinflammatory syndrome with pyogenic bacterial infection and amylopectinosis. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified nutritional and metabolic disorders.
For Medicare Advantage risk adjustment, E74.03 maps to CMS-HCC Category 50 (Amyloidosis, Porphyria, and Other Specified Metabolic Disorders) under the V28 model, adding a risk factor of about 0.648 for a community, non-dual, aged beneficiary in payment year 2027.
Code Identity
Code Classification
Medicare Risk Adjustment (HCC)Billing
E74.03 maps to a payment category in the CMS-HCC model used to risk-adjust Medicare Advantage payments. Weights are the published community factors for payment year 2027.
Source: CMS Payment Year 2027 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.
Approximate SynonymsGuidance
Alternate terms and clinical phrases that map to this code.
- Autoinflammatory syndrome with pyogenic bacterial infection and amylopectinosis
- Autoinflammatory syndrome with pyogenic bacterial infection and amylopectinosis due to HOIP deficiency
- Glycogen storage disease type III
- Glycogen storage disease, hepatic form
- Glycogen storage disease, muscular form
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to E74.03: its own notes plus those printed at E74, block E70-E88, and Chapter 4. A note printed at a category, block or chapter applies to every code under it.
Applicable To
Conditions this code is used for: synonyms of the title or, for "other specified" codes, the conditions assigned to it. The list is not exhaustive.
- Forbes disease
- Type III glycogen storage disease
Excludes1
Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.
Excludes2
Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.
- Ehlers-Danlos syndromes (Q79.6-)
Notes
General instructions on how these codes are used.
- All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.
Source: CMS ICD-10-CM Tabular List. How to read instructional notes.
Referenced in Other NotesGuidance
Instructional notes printed at other codes that name E74.03, its category, or a range that includes it.
Excludes1 2
These codes carry an Excludes1 note naming E74.03: they are not reported together with it, unless the two conditions are unrelated.
- R79 Other abnormal findings of blood chemistry
- Block R80-R82 Abnormal findings on examination of urine, without diagnosis
Code First 3
These codes say to code first a condition named in the note, and E74.03 is one of the conditions named.
- G73.7 Myopathy in diseases classified elsewhereglycogen storage disease (E74.0-) names E74.0-, which includes this code
- I43 Cardiomyopathy in diseases classified elsewhereglycogen storage disease (E74.0-) names E74.0-, which includes this code
- N16 Renal tubulo-interstitial disorders in diseases classified elsewhereglycogen storage disease (E74.0-) names E74.0-, which includes this code
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
Disease, diseased See Also: Syndrome;
Cori's (glycogenosis III) E74.03
Forbes' (glycogenosis III) E74.03
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
Clinical InformationClinical
Glycogen Storage Disease Type III
an autosomal recessive metabolic disorder due to deficient expression of amylo-1,6-glucosidase (one part of the glycogen debranching enzyme system). the clinical course of the disease is similar to that of glycogen storage disease type i, but milder. massive hepatomegaly, which is present in young children, diminishes and occasionally disappears with age. levels of glycogen with short outer branches are elevated in muscle, liver, and erythrocytes. six subgroups have been identified, with subgroups type iiia and type iiib being the most prevalent.
Patient EducationClinical
Carbohydrate Metabolism Disorders
Metabolism is the process your body uses to make energy from the food you eat. Food is made up of proteins, carbohydrates, and fats. Chemicals in your digestive system (enzymes) break the food parts down into sugars and acids, your body's fuel. Your body can use this fuel right away, or it can store the energy in your body tissues.
Read the full article at MedlinePlus
Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.
Convert E74.03 to ICD-9-CMHistory
The closest ICD-9-CM equivalents under the General Equivalence Mappings.
Code HistoryHistory
Questions About E74.03Overview
What is the ICD-10 code for cori disease?
The ICD-10-CM code for cori disease is E74.03 (sometimes written as E7403). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
Is E74.03 (Glycogen storage disease) a billable code?
Yes. This is a billable ICD-10-CM code, specific enough to report cori disease on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
What MS-DRG does E74.03 group to?
When cori disease is the principal diagnosis on an inpatient stay, it groups to MS-DRG 642 (inborn and Other Disorders of Metabolism), which carries a relative weight of 1.3226. Higher weights mean higher Medicare reimbursement.
Is E74.03 a CC or MCC?
CMS lists E74.03 as a CC (complication or comorbidity) for FY 2027. Reported as a secondary diagnosis, it moves the inpatient stay to a higher-weighted DRG within its severity family. It does not count when the principal diagnosis is one of the 140 closely related codes in its exclusion list.
Can E74.03 be reported with E16.2?
Not as a rule. The Excludes1 note at E74, which applies to E74.03, lists "hypoglycemia NOS (E16.2)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).
What HCC is E74.03?
E74.03 (cori disease) maps to CMS-HCC Category 50 (Amyloidosis, Porphyria, and Other Specified Metabolic Disorders), commonly written as HCC 50, in the CMS-HCC V28 model used for Medicare Advantage risk adjustment in payment year 2027. It mapped to HCC 23 under the retired V24 model. It also maps in the PACE (CMS-HCC V22), ESRD (V21), and ESRD (V24) models. In the Part D prescription drug model it maps to RxHCC 43.
Does E74.03 risk-adjust for Medicare Advantage payment?
Yes. When documented and reported on a Medicare Advantage encounter, E74.03 adds a risk adjustment factor of about 0.648 to the beneficiary's RAF score for a community, non-dual, aged enrollee (published V28 weights range from 0.362 to 0.883 depending on the payment segment). HCC 50 sits at the top of its hierarchy, so no other condition category supersedes it. See the full factor table on the HCC 50 category page.
