Mucopolysaccharidosis Mps Iv A C0086651
Also known as Morquio A syndrome, this inherited disorder primarily affects the skeleton and connective tissues due to difficulty breaking down certain sugar molecules. Individuals have short stature, bone abnormalities, and may need joint replacements. Treatment includes enzyme replacement therapy and orthopedic interventions.
Key facts at a glance
- ICD-10
- E88.9
- ICD-11
- 5A00.0
- Body system
- metabolic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Corneal clouding
Fatigue or weight changes
(metabolic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Short stature
(musculoskeletal)Joint pain
(musculoskeletal)Hearing loss
(sensory)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Enzyme Replacement Therapy (Elosulfase alfa)
IV-administered enzyme replacement that helps metabolize keratan sulfate. Standard first-line treatment for eligible MPS IVA patients to improve endurance and walking ability.
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Skeletal Deformity Correction
Surgical intervention for severe scoliosis, kyphosis, or other skeletal abnormalities common in Morquio syndrome to improve mobility and quality of life.
Additional treatment options
Structured Exercise Program
Low-impact exercises focusing on strength, balance, and maintaining joint mobility while protecting the cervical spine and weight-bearing joints.
physical_therapyCervical Spine Monitoring
Regular imaging and neurological assessment of the upper cervical spine due to atlantoaxial instability and potential cord compression risk.
monitoringRespiratory Support Therapy
Techniques and devices to support respiratory function, including airway clearance methods and sleep-disordered breathing management.
complementaryWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Mucopolysaccharidosis Mps Iv A C0086651?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy