Lab Information Manual

Laboratory:

Clinical Biochemistry (METABOLIC)

Test Name:

CYSTINE, Urine - (U)

Test Code: AAQU

Clinical Information:

Test Indications: Evaluation and monitoring of patients’ urinary excretion of cystine.

Cystinuria is an inherited disorder of amino acid transport characterized by excessive excretion of cystine, ornithine, lysine and arginine (COLA). Urolithiasis is caused by the relative insolubility of cystine in the urine which cause repeated stone formation. Symptoms often occur in childhood or early adult life, but cystinuria may become evident at any age.

Cystine, ornithine, lysine and arginine are included in the urine amino acid profile offers in the Metabolic Lab. Please select "CYSTINE, U" in the urine section of the Biochemical Genetics Laboratory Requisition.

For questions/information: Call metabolic lab at 204-787-4530 and ask for the biochemical geneticist.

Collection Devices:

Preferred Device:	2 - Urine Collection Container, Random 100 mL Urine Collection Container, Random 100 mL Manufacturer: Starplex Scientific Inc. (Etobicoke, ON) Catalogue #: 3008-1
Alternate Device:	1 - Urine Collection Container, Leak-Proof Random 100mL Urine Collection Container, Leak-Proof Random 100mL Manufacturer: Kendall, A Tyco International Co. Catalogue #: 2205 SA

Fresh random urine

No preservatives accepted.

Please Note: Cystine analysis on 24 hour urine collection is not available.

Specimen Required:

Urine: 10.0 mL

Pediatric Urine: 1.0 mL

Pediatric patient: If unable to get enough urine, please call the Metabolic Lab at 204-787-4530.

Collection Information: Prepare TWO aliquots immediately after collection.

One aliquot for urine creatinine to Automated chemistry can be sent refrigerated and the second aliquot for amino acid testing MUST be FROZEN within 1 hour of collection.

Only ship frozen in Two Aliquots. DO NOT ship in original 100 mL urine container.

Specimen Stability:

Ambient: Not stable

Refrigerated: Not stable

Frozen: 14 days at -20C or 60 days at -80C

Referral:

Urine: 10.0 mL

Pediatric Urine: 1.0 mL

Shipping & Storage: Samples must be shipped frozen in separate aliquots.

Testing Laboratory (MB): Send specimen to Health Sciences Centre –MS5 (Biochemical Genetics)

Requisition:

_SH Biochemical Genetics Laboratory Requisition (R250-10-69)

Reference Values:

Reference Intervals: A descriptive report will be sent.

Method of Analysis: Liquid Chromatography Tandem Mass Spectrometry

Availability:

Within 1 Week

If this analysis needs to be performed urgently, please communicate with the biochemical geneticist/ Metabolic Lab at 204-787-4530.