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MetabolicGaucher DiseaseAcid Sphingomyelinase (ASM) Deficiency (Niemann-Pick Disease Type A and B)

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

Parallel enzyme assay for Gaucher and ASMD measuring GCase and ASM activity, with reflex to GBA or SMPD1 sequencing as indicated; lyso-GL1 added when GCase is low.

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 Gaucher disease or ASMD based on signs/symptoms, or presumptive positive NBS for either disorder.
  • Order parallel GCase and ASM enzyme assays with reflex sequencing of GBA or SMPD1, as indicated by the deficient enzyme.
  • 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 clinical features suggestive of Gaucher disease or ASMD, or presumptive positive NBS for either disorder.

What is the testing algorithm?

Parallel GCase and ASM enzyme activity; reflex sequencing of GBA or SMPD1 is performed based on the deficient enzyme. Consider lyso-GL1 to support Gaucher assessment.

Why parallel enzymes?

It distinguishes Gaucher (GCase deficiency) from ASMD (ASM deficiency) efficiently within one pathway.

How should I use lyso-GL1?

Use it as an adjunct when Gaucher is suspected to aid interpretation and follow-up.

What about females or atypical presentations?

Molecular confirmation (GBA or SMPD1) is important when enzyme results or phenotype are equivocal.

Typical turnaround times?

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

Is this appropriate for carrier screening?

No. Use separate carrier workflows if that is the clinical question.

How are results reported?

Reports are issued to the ordering provider with enzyme activities and, if performed, molecular findings and interpretation.

Any scope limitations to note?

This pathway does not evaluate Niemann-Pick type C and is not designed to resolve all complex structural variants; integrate results with phenotype and family history.

Test details

  • Formal test nameGlucocerebrosidase enzyme assay (includes ASM enzyme assay in parallel) with reflex to GBA and Lyso-GL1 or SMPD1 sequencing as appropriate SAN021, SAN025> SAN022+ SAN017or SAN026
  • ConditionsGaucher Disease, Acid Sphingomyelinase (ASM) Deficiency (Niemann-Pick Disease Type A and B)
  • Test typeBiochemical — Enzymatic Activity
  • Genes / markers
    2
  • Key genes / markersGlucocerebrosidase enzyme assay, Acid sphingomyelinase (ASM) 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 test
Print requisition
Order collection kit
Learn more

Additional Resources

Sample Collection Instructions

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

Mucopolysaccharidosis I (MPS I)

Roadmap2Rare•Sanofi

Muscle Disorders Panel

Roadmap2Rare•Sanofi

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