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Test Code U AAQNT Amino Acids Quant, Urine

Clinical System Name

Amino Acids Quant, Urine


Quantitation of 24 amino acids by ion exchange chromatography

Sample Requirements

Specimen: Urine

Container(s): Leak-Proof Container, 24 Hour Urine Jug

Preferred Vol:  5.0 mL

Minimum Vol: 1.0 mL


Note:  In most cases, random samples are adequate & 24-hr collection is not required.

Processing Instructions

Reject due to:



Temp:-20 C

Storage location: BCG Box


Off-site collection: Aliquotes should be taked from a thoroughly thawed & mixed refrigerated sample.  Ship frozen.


Temperature Time
Room temp ≤2 hrs
Refrigerated 24 hrs
Frozen 1 month



STAT Performed TAT
N M-F 2-5 days

Contact the Biochemical Genetics Lab for requests outside of stated availability (206)987-2216.

Performing Laboratory

Seattle Children's Laboratory    


Department:  Biochemical Genetics

Phone Number: 206-987-2216




Method: Ion exchange chromatography with post column derivatization

Analytical Volume: 0.2 mL


CPT Codes



Biochemical Genetics Requisition


On the requisition include clinical inforamtion needed for appropriate interpretation. (Age, gender, diet (e.g. TPN therapy), drug therapy and family history)

Clinical Utility

Urine amino acid analysis can be useful in the clinical work up of the following disorders:


Urea cycle defects (argininosuccinic aciduria, argininemia, CPS, OTC deficiency, citrullinemia), non-ketotic hyperglycinemia, homocystinuria, defects of cobalamin metabolism, cystinuria, ornithinemia, renal tubular acidosis/fanconi syndrome, and lysinuric protein intolerance.