Morquio A mucopolysaccharidoses

📋Clinical Description

This code identifies Morquio A syndrome, also known as mucopolysaccharidosis type IVA (MPS IVA), a rare, inherited metabolic disorder characterized by the body's inability to break down specific complex sugars (keratan sulfate). This leads to their accumulation in various tissues, causing progressive skeletal dysplasia, short stature, and other systemic manifestations.

🎯When to Use

Use this code for patients diagnosed with Morquio A syndrome based on clinical presentation, enzyme analysis (deficient N-acetylgalactosamine-6-sulfatase), and/or genetic testing. Documentation should clearly state the diagnosis of Morquio A or MPS IVA.

💡Coding Tips

Differentiate from other mucopolysaccharidoses (e.g., Morquio B, MPS I, II, VI) which have distinct codes.
Code any associated manifestations or complications (e.g., skeletal deformities, cardiac issues, respiratory problems) as secondary diagnoses.
Ensure the medical record specifies "Morquio A" or "MPS IVA" to support this specific code.

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Clinical Notes

Inclusion Terms

Classic Morquio syndrome
Morquio syndrome A
Mucopolysaccharidosis, type IVA

Code Hierarchy

▲E76.21Morquio mucopolysaccharidoses

E76.210(current)

Crosswalks

277.5ApproxICD-9

Same Category

E76Disorders of glycosaminoglycan metabolism E76.0Mucopolysaccharidosis, type I E76.01Hurler's syndrome E76.02Hurler-Scheie syndrome E76.03Scheie's syndrome