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MetabolicMucopolysaccharidosis Type I (MPS I) (Hurler-Scheie Syndrome)

Mucopolysaccharidosis I (MPS I)

Measures alpha-L-iduronidase (IDUA) enzyme activity; if low, reflexes to IDUA gene sequencing to evaluate MPS I.

Accessed through the Roadmap2Rare Sponsored Testing Program•Sponsored by Sanofi•Performed by Revvity Omics, Inc.

Designed to support clinical decision-making and ordering in your practice.

For healthcare providers

Overview

Roadmap2Rare (Canada) provides complimentary genetic and biochemical testing for suspected rare diseases, delivered by Revvity Omics in collaboration with Sanofi Canada. The program is designed to address unmet testing needs and is available to Canadian clinicians. Test selection and treatment decisions remain at the discretion of the healthcare professional.

When to consider this test

Patient selection

  • Clinical suspicion of MPS I based on signs/symptoms, or presumptive positive NBS for MPS I.
  • Order alpha-L-iduronidase enzyme activity with reflex to IDUA sequencing when deficiency is detected.
  • Not intended for carrier screening; use separate workflows for carrier evaluation.

Workflow

How to use this program

  1. 1

    Select & order

    Assess clinical fit (e.g., suspected Pompe/NBS flag) and submit the Roadmap2Rare requisition.

  2. 2

    Collect & ship

    Collect per kit instructions (e.g., DBS for enzyme; panel as directed) and ship to Revvity.

  3. 3

    Review results

    Review the report, counsel the family, and, if enzyme-deficient, note reflex sequencing status.

Billing overview

Program billing

For eligible patients, the sponsor has arrangements with the laboratory that may reduce or cover some or all of the patient's out-of-pocket cost. Actual cost and patient responsibility vary by program criteria. Refer to lab or sponsor materials for details.

Quality & privacy

Laboratory quality & data handling

Testing is performed by Revvity Omics, Inc., a CLIA-certified laboratory (CLIA 39D0673919). Your sample is processed using validated methods and quality controls to support reliable genetic results.

Your health information is handled under privacy and security practices that are intended to align with applicable regulations. For program- and lab-specific details, review the privacy information provided by the laboratory and sponsor.

FAQ

Frequently asked questions

When should I order?

For patients with clinical features consistent with MPS I or presumptive positive NBS requiring confirmatory evaluation.

What is the testing algorithm?

Primary alpha-L-iduronidase (IDUA) enzyme activity with reflex to comprehensive IDUA sequencing when enzyme deficiency is detected.

Why start with enzyme activity?

It provides a functional readout for IDUA; molecular testing confirms and characterizes variants when activity is low.

Any role for carrier testing here?

No. This pathway is not intended for carrier screening; use separate workflows if carrier status is the clinical question.

How are results reported?

The lab reports enzyme activity with interpretation and, if performed, IDUA sequencing findings according to standard reporting policies.

Typical turnaround times?

TAT varies by assay and program guidance; set expectations with families accordingly.

How should I integrate results?

Interpret enzyme and molecular findings in the context of phenotype, family history, and current guidelines; consider genetics and specialty referral as needed.

Any scope limitations I should note?

This pathway is not designed to resolve all complex structural variants or to serve as carrier screening; use additional testing as clinically indicated.

Test details

  • Formal test nameAlpha-L-iduronidase enzyme assay with reflex to IDUA sequencing SAN023> SAN024
  • ConditionMucopolysaccharidosis Type I (MPS I) (Hurler-Scheie Syndrome)
  • Test typeBiochemical — Enzymatic Activity
  • Genes / markers
    1
  • Key genes / markersAlpha-L-iduronidase enzyme activity
  • Specimen
    Dried blood spot (DBS)
  • Turnaround time12 Days
  • LabRevvity Omics, Inc.

Getting started

Use the links below to review program details, place orders, print requisitions, or request collection kits.

Performing lab

Revvity Omics, Inc.

Order testPrint requisitionOrder collection kitLearn more

Privacy & ordering: This directory doesn't handle PHI. Orders occur on participating labs' systems. Privacy & Compliance

Other Tests in This Program

Pompe Disease Test

Roadmap2Rare•Sanofi

Hypertrophic Cardiomyopathy (HCM) Panel

Roadmap2Rare•Sanofi

Fabry Disease Test

Roadmap2Rare•Sanofi

Gaucher Disease and ASMD (Niemann-Pick A/B)

Roadmap2Rare•Sanofi

Muscle Disorders Panel

Roadmap2Rare•Sanofi

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