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Enzyme Replacement Therapy (ERT) Monitoring Labs

Important Note

These tests are only offered to Seattle Children's Biochemical Genetics providers. We do not offer these tests or coordinate pass throughs for external clients.

 

If you are not associated with the Seattle Children's Biochemical Genetics clinic, please contact the performing laboratory directly. Additional contact information is listed below.

 

Overview: Patients diagnosed with some inborn errors of metabolism may receive enzyme replacement therapy (ERT). Treatment protocols vary but may include metabolite monitoring and testing for antibodies reactive to the ERT. Specific tests and evaluation frequency is determined by the managing biochemical genetics physician.

Additional Codes

ERT

Performing Lab

EPIC Order

Sample

Lab Page

Shipping

IgE  (adverse events)

 Neutralizing Antibody Testing*

Alpha-Mannosidosis

Anti-rhLaman IgG Antibody

Frontage

Miscellaneous

4 mL whole blood, red tube

 

Kit required

yes

  

Alpha-Mannosidosis oligosaccharide analysis

Greenwood Genetics

Miscellaneous

2-4 mL whole blood, red tube

 

Kit required

N/A

  

Gaucher

Cerezyme/Imiglucerase IgG Antibody

LabCorp/Sanofi

LAB2908

2-5 mL whole blood, gold or red tube

LAB2908

frozen serum

yes

  

Anti-Vela Antibody

Covance/Shire

Miscellaneous

1-6 mL whole blood, red tube

Anti-Vela Antibodies - Seattle Children's Hospital (testcatalog.org)

Kit required

yes

  

Chitotriosidase Enzyme Analysis (Chito)

Greenwood Genetics

Miscellaneous

5-7 mL whole blood, green tube

Greenwood Genetic Center (ggc.org)

 

N/A

  
Glucopsychosine, Plasma Mayo Clinic Laboratories Miscellaneous

1.0 mL Whole Blood in, Lavender Top Tube, Dark Green NaHep, Lt Green/Mint Top-LiHep, Lt Green LiHep Microtainer, or Yellow ACD B

 Glucopsychosine, Plasma (GPSYP)      

Lyso-GL1

LabCorp/Sanofi

Miscellaneous

1 mL plasma, EDTA purple tube

 

frozen

 

  

Fabry

Anti-Agalsidase Antibody (alpha)

Covance/Shire

Miscellaneous

1-6 mL whole blood, red tube

 

Kit required

yes

  

Fabrazyme- IgG Antibody (agalsidase beta)

LabCorp/Sanofi

LAB3029

2-5 mL whole blood, gold or red tube

Fabrazyme IgG Antibody - Seattle Children's Hospital (testcatalog.org)

frozen serum

yes

 yes

GL-3 Level (aka Gb3)

LabCorp/Sanofi (alt. Covance/Shire)

 LAB3075 (Plasma)

1 mL plasma green sodium heparin tube

GL-3 Level, Plasma - Seattle Children's Hospital (testcatalog.org)

frozen

N/A

  

Lyso-GL3 (aka Lyso-Gb3)

LabCorp/Sanofi (alt. Mayo)

LAB3075

1 mL plasma, green tube

 

frozen

N/A

  
GL3 Level, urine LabCorp/Sanofi Miscellaneous 1 mL urine clean catch transfer to C+S preservative tube   frozen N/A  

Mucopolysaccharidoses

Laronidase/Aldurazyme IgG Antibody (MPS1)

LabCorp/Sanofi

LAB2708

2-5 mL whole blood, gold or red tube

Aldurazyme IgG Antibody - Seattle Children's Hospital (testcatalog.org)

frozen serum

yes

 yes

Elaprase/Idursulfase Antibodies (MPS II)

Covance/Shire

Miscellaneous

1-6 mL whole blood, red tube

Idursulfase Antibodies - Seattle Children's Hospital (testcatalog.org)

Kit required

N/A

  

MPS IV Monitoring

Greenwood Genetics

LAB1958

3-5 mL urine

MPS IV Monitoring, Urine - Seattle Children's Hospital (testcatalog.org)

 

N/A

  

Pompe

Avalglucosidase alpha/Nexviazyme IgG Antibody

LabCorp/Sanofi

LAB3834

2-5 mL whole blood, gold or red tube

Nexviazyme (Avalglucosidase alfa) IgG IgM Antibody - Seattle Children's Hospital (testcatalog.org)

frozen serum

yes

 yes

Lumizyme/Myozyme/Aglucosidase alfa IgG Antibody

LabCorp/Sanofi

LAB3258

2-5 mL whole blood, gold or red tube

Lumizyme IgG Antibody - Seattle Children's Hospital (testcatalog.org)

frozen serum

yes

 yes

Hex4 (aka Glc4)

Duke (alt. LabCorp/Sanofi)

LAB1980

0.5-1 mL urine

Urine Glucose Tetrasaccharide (Hex4) - Seattle Children's Hospital (testcatalog.org)

frozen 

N/A

  

Infusion Reaction Testing**

 
Complement Activation: C3a LabCorp/Sanofi Miscellaneous 1 mL plasma, EDTA purple tube   frozen    
Mast Cell Activation Serum Tryptase LabCorp/Sanofi Miscellaneous 1 mL serum, red tube   frozen    

*Recommend sample collection at least 3 days after infusion or prior to subsequent infusion.

**Recommend sample collection 1-3 hours after onset of Infusion reaction.

 

Department

Department: Send Outs

Phone Number: (206) 987-2563

Performing Laboratories

Chiesi (Frontage and Greenwood Genetics testing for alpha-mannosidosis)

Fax: (855) 929-2828

Phone: (866) 758-7071

chiesitotalcare.com

 

LabCorp / Sanofi Genzyme's Rare Disease Program

Phone: (888) 681-1701

Email: RareDiseaseProgram@labcorp.com.

 

Covance/Shire

Phone: (844) 466-8507

Email: LabServices@shire.com