Disorders of glycosaminoglycan metabolism (E76)
The ICD-10 code section E76 covers disorders of glycosaminoglycan metabolism, including various types of mucopolysaccharidoses (MPS). These conditions are rare inherited metabolic diseases characterized by enzyme deficiencies affecting the breakdown of glycosaminoglycans.
Codes like E76.0 through E76.3 specify different mucopolysaccharidosis types. For example, E76.01 refers to Hurler's syndrome, also known as MPS-I-H or dysostosis multiplex, while E76.1 represents MPS-II, commonly called Hunter's syndrome. The section further categorizes variants including Morquio syndromes (codes E76.210 and E76.211) and Sanfilippo syndrome (E76.22). Specific distinctions among these codes help medical coders accurately identify the exact disorder, such as differentiating between Morquio A and B forms. Unspecified or other mucopolysaccharidoses and glucosaminoglycan metabolism disorders are captured under codes like E76.3 and E76.9. Using these codes correctly supports precise documentation and treatment planning for these complex metabolic diseases.
Endocrine, nutritional and metabolic diseases (E00–E89)
Metabolic disorders (E70-E88)
E76 Disorders of glycosaminoglycan metabolism
E76.0 Mucopolysaccharidosis, type I
- E76.01 Hurler's syndrome
- E76.02 Hurler-Scheie syndrome
- E76.03 Scheie's syndrome
- E76.1 Mucopolysaccharidosis, type II
E76.2 Other mucopolysaccharidoses
E76.21 Morquio mucopolysaccharidoses
- E76.210 Morquio A mucopolysaccharidoses
- E76.211 Morquio B mucopolysaccharidoses
- E76.219 Morquio mucopolysaccharidoses, unspecified
- E76.22 Sanfilippo mucopolysaccharidoses
- E76.29 Other mucopolysaccharidoses
- E76.3 Mucopolysaccharidosis, unspecified
- E76.8 Other disorders of glucosaminoglycan metabolism
- E76.9 Glucosaminoglycan metabolism disorder, unspecified
Disorders of glycosaminoglycan metabolism (E76)
Clinical Terms
The following clinical terms provide additional context, helping users better understand the clinical background and common associations for each diagnosis listed in this section. Including related terms alongside ICD-10-CM codes supports coders, billers, and healthcare professionals in improving accuracy, enhancing documentation, and facilitating research or patient education.
Mucopolysaccharidoses
Group of lysosomal storage diseases each caused by an inherited deficiency of an enzyme involved in the degradation of glycosaminoglycans (mucopolysaccharides). The diseases are progressive and often display a wide spectrum of clinical severity within one enzyme deficiency.
