DxCodebookFY2027 · Oct 1, 2026 – Sep 30, 2027

Category · FY2027

E76 Disorders of glycosaminoglycan metabolism

  • Header · not billable

E76 is not billable. The category heads 12 billable codes in FY2027, grouped under 6 subdivisions.

Index → code

Where it sits

  1. Chapter 4 Endocrine, nutritional and metabolic diseases (E00-E89)
  2. E70-E88 Metabolic disorders (E70-E88)
  3. E76 Disorders of glycosaminoglycan metabolism
Billable codes
12
Subdivisions
6
CC / MCC codes
12 / 0
HCC V28 codes
10
New FY2027
0
Index entries
43

Codes in E76

  1. E76.0
    Mucopolysaccharidosis, type I
    Header
  2. E76.01
    Hurler's syndrome
    BillableCC49
  3. E76.02
    Hurler-Scheie syndrome
    BillableCC49
  4. E76.03
    Scheie's syndrome
    BillableCC49
  5. E76.1
    Mucopolysaccharidosis, type II
    BillableCC49
    Inclusion terms 1

    Inclusion terms

    • Hunter's syndrome
  6. E76.2
    Other mucopolysaccharidoses
    Header
  7. E76.21
    Morquio mucopolysaccharidoses
    Header
  8. E76.210
    Morquio A mucopolysaccharidoses
    BillableCC49
    Inclusion terms 3

    Inclusion terms

    • Classic Morquio syndrome
    • Morquio syndrome A
    • Mucopolysaccharidosis, type IVA
  9. E76.211
    Morquio B mucopolysaccharidoses
    BillableCC49
    Inclusion terms 4

    Inclusion terms

    • Morquio-like mucopolysaccharidoses
    • Morquio-like syndrome
    • Morquio syndrome B
    • Mucopolysaccharidosis, type IVB
  10. E76.219
    Morquio mucopolysaccharidoses, unspecified
    BillableCC49
    Inclusion terms 2

    Inclusion terms

    • Morquio syndrome
    • Mucopolysaccharidosis, type IV
  11. E76.22
    Sanfilippo mucopolysaccharidoses
    BillableCC49
    Inclusion terms 5

    Inclusion terms

    • Mucopolysaccharidosis, type III (A) (B) (C) (D)
    • Sanfilippo A syndrome
    • Sanfilippo B syndrome
    • Sanfilippo C syndrome
    • Sanfilippo D syndrome
  12. E76.29
    Other mucopolysaccharidoses
    BillableCC49
    Inclusion terms 3

    Inclusion terms

    • beta-Glucuronidase deficiency
    • Maroteaux-Lamy (mild) (severe) syndrome
    • Mucopolysaccharidosis, types VI, VII
  13. E76.3
    Mucopolysaccharidosis, unspecified
    BillableCC49
  14. E76.8
    Other disorders of glucosaminoglycan metabolism
    BillableCC
  15. E76.9
    Glucosaminoglycan metabolism disorder, unspecified
    BillableCC

CC/MCC: as a secondary diagnosis, unless the principal diagnosis is on the code’s PDX exclusion list (MS-DRG v44 Appendix C; list on the code card).

Sources of each column.

Instructions

Notes that also apply

Block E70-E88 instructions (Excludes1 5, Excludes2 1): printed on the block page.

Chapter 4 instructions (Notes 1, Excludes1 1): printed on the chapter 4 page.

Index entries

  • Gargoylism → E76.01
  • Lipochondrodystrophy → E76.01
  • Hurler (-Scheie) disease or syndrome → E76.02
  • Scheie's syndrome → E76.03
  • Chondro-osteodysplasia (Morquio-Brailsford type) → E76.219
  • Sanfilippo (Type B) (Type C) (Type D) syndrome → E76.22
  • Chondro-osteodystrophy → E76.29
  • Eccentro-osteochondrodysplasia → E76.29
  • Maroteaux-Lamy syndrome (mild) (severe) → E76.29
  • Mucopolysaccharidosis → E76.3
  • Dysostosismultiplex → E76.01
  • MucopolysaccharidosisHurler's syndrome → E76.01
  • MucopolysaccharidosisHurler-Scheie syndrome → E76.02
  • MucopolysaccharidosisScheie's syndrome → E76.03
  • Hunter'ssyndrome → E76.1
  • MucopolysaccharidosisHunter's syndrome → E76.1
  • Kyphosis, kyphotic (acquired)Morquio-Brailsford type (spinal) → E76.219
  • MucopolysaccharidosisMorquio syndrome → E76.219
  • MucopolysaccharidosisSanfilippo syndrome → E76.22
  • Deficiency, deficientbeta-glucuronidase → E76.29
  • Mucopolysaccharidosisbeta-gluduronidase deficiency → E76.29
  • MucopolysaccharidosisMaroteaux-Lamy syndrome → E76.29
  • Mucopolysaccharidosisspecified NEC → E76.29
  • Cardiopathymucopolysaccharidosis → E76.3 [I52]
  • Mucopolysaccharidosiscardiopathy → E76.3 [I52]
  • MucopolysaccharidosistypeII → E76.1
  • MucopolysaccharidosisMorquio syndromeA → E76.210
  • MucopolysaccharidosisMorquio syndromeclassic → E76.210
  • MucopolysaccharidosistypeIVA → E76.210
  • MucopolysaccharidosisMorquio syndromeB → E76.211
  • MucopolysaccharidosistypeIVB → E76.211
  • MucopolysaccharidosistypeIV → E76.219
  • MucopolysaccharidosistypeIII → E76.22
  • MucopolysaccharidosistypeVI → E76.29
  • MucopolysaccharidosistypeVII → E76.29
  • Disorder (of)metabolism NOSglucosaminoglycan → E76.9
  • Disorder (of)metabolism NOSglycosaminoglycan → E76.9
  • MucopolysaccharidosistypeIHurler's syndrome → E76.01
  • MucopolysaccharidosistypeIHurler-Scheie syndrome → E76.02
  • MucopolysaccharidosistypeIScheie's syndrome → E76.03

3 more, longer entries are not listed; the search box finds them by word.

NCHS Alphabetic Index. Index first, then the Tabular List.

Hierarchy