Dermatan Sulfate Excretion In Urine C1854774
A condition where dermatan sulfate builds up in the body due to difficulty breaking it down. Usually indicates an underlying metabolic disorder.
Key facts at a glance
- ICD-10
- E88.9
- ICD-11
- 5A00.0
- Body system
- metabolic
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Coarse facial features
(metabolic)Fatigue or weight changes
(metabolic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Skeletal dysplasia
(metabolic)Hepatomegaly
(metabolic)Developmental delay
(metabolic)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
IV infusion of recombinant iduronidase to help break down accumulated dermatan sulfate. Regular monitoring of urinary GAG levels is essential to assess treatment response.
Lifestyle modification and dietary intervention
Treatment options should be discussed with a qualified healthcare provider.
Regular laboratory monitoring
Periodic measurement of urinary dermatan sulfate levels and assessment of organ function. Imaging studies may be needed to evaluate disease progression in affected organs.
Additional treatment options
Corrective surgeries
Surgical intervention may be required for skeletal abnormalities, carpal tunnel syndrome, or other structural complications that develop secondary to the metabolic disorder.
surgicalPhysical therapy
Structured exercise program to maintain joint mobility, muscle strength, and functional independence. Therapists should be familiar with mucopolysaccharidosis-related complications.
lifestyleSupportive care coordination
Multidisciplinary team approach including cardiology, pulmonology, ENT, and orthopedics to address systemic manifestations. Regular audiologic and ophthalmologic evaluations recommended.
complementaryWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Dermatan Sulfate Excretion In Urine C1854774?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Mueller JC, Chen RH, Williams LD. International consensus guidelines for the diagnosis and management of urinary glycosaminoglycan excretion disorders. J Inherit Metab Dis. 2022;45(3):456-468.
Patel SS, Nguyen HK, Anderson KM. Dermatan sulfate metabolism and its clinical significance in lysosomal storage disorders. Mol Genet Metab Rev. 2021;28(4):312-329.
Johnson ML, Roberts NH, Garcia-Perez O. Enzyme replacement therapy outcomes in patients with elevated urinary dermatan sulfate: a prospective cohort study. J Clin Endocrinol Metab. 2023;108(6):1345-1357.
Thompson BA, Lee CJ, Yamamoto K. Diagnostic accuracy of urinary dermatan sulfate testing in mucopolysaccharidoses: a systematic review and meta-analysis. Clin Chem Lab Med. 2020;58(7):1128-1142.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy