Disorders of glycosaminoglycan metabolism
In Metabolic disorders (E70-E88), chapter 4: Endocrine, Nutritional and Metabolic Diseases
12 billable codes under E76: all are a CC; 10 map to HCC 49 (Specified Lysosomal Storage Disorders).A CC or MCC (major CC) is a secondary diagnosis that raises the MS-DRG payment. An HCC counts toward a Medicare Advantage patient's risk score.
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Instructional notes
Also applies, from Metabolic disorders (E70-E88)
These notes sit on E70-E88 and apply to every code under it, including E76.
Excludes1: not coded here, use a different code
Excludes2: not included here, code separately if applicable
- Ehlers-Danlos syndromes (Q79.6-)
Official coding guidelines
From the ICD-10-CM Official Guidelines for Coding and Reporting, FY 2027 (CMS and NCHS). Links open the PDF at the right page.
Covers diabetes mellitus coding, including type, insulin and drug use, pump malfunction and secondary diabetes, plus obesity and obesity class codes.
- Diabetes mellitus (page 39)
- Obesity (page 42)
All codes under E76
E76 can't be used on a claim. Pick a billable code below; grey headings group them.
- E76.0Mucopolysaccharidosis, type I
- E76.01Hurler's syndrome
- E76.02Hurler-Scheie syndrome
- E76.03Scheie's syndrome
- E76.1Mucopolysaccharidosis, type II
- E76.2Other mucopolysaccharidoses
- E76.21Morquio mucopolysaccharidoses
- E76.210Morquio A mucopolysaccharidoses
- E76.211Morquio B mucopolysaccharidoses
- E76.219Morquio mucopolysaccharidoses, unspecified
- E76.22Sanfilippo mucopolysaccharidoses
- E76.29Other mucopolysaccharidoses
- E76.3Mucopolysaccharidosis, unspecified
- E76.8Other disorders of glucosaminoglycan metabolism
- E76.9Glucosaminoglycan metabolism disorder, unspecified
E76 MS-DRG
As the principal diagnosis, all 12 billable codes under E76 group to MS-DRG 642 (v44.0).
About the condition
Plain-language background from MedlinePlus (National Library of Medicine), not coding guidance.
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. If you have a metabolic disorder, something goes wrong with this process.
Read more about carbohydrate metabolism disorders on MedlinePlusCode history
Valid since FY2016, when ICD-10-CM took effect on October 1, 2015. No changes since.
