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Test ID: HYOX Hyperoxaluria Panel, Random, Urine

Reporting Name

Hyperoxaluria Panel, U

Useful For

Distinguishing between primary and secondary hyperoxaluria


Distinguishing between primary hyperoxaluria types 1, 2, and 3

Specimen Type


Specimen Required

Supplies: Urine Tubes, 10 mL (T068)

Container/Tube: Plastic, 10-mL urine tube

Specimen Volume: 10 mL

Collection Instructions:

1. Collect a random urine specimen.

2. No preservative.

3. Immediately freeze specimen.

Specimen Minimum Volume

1.1 mL

Specimen Stability Information

Specimen Type Temperature Time Special Container
Urine Frozen (preferred) 90 days
  Refrigerated  14 days

Special Instructions

Reference Values


≤17 years: ≤75 mg/g creatinine

≥18 years: ≤50 mg/g creatinine



≤31 days: ≤75 mg/g creatinine

32 days - 4 years: ≤125 mg/g creatinine

5 - 10 years: ≤55 mg/g creatinine

≥11 years: ≤25 mg/g creatinine



≤6 months: ≤400 mg/g creatinine

7 months - 1 year: ≤300 mg/g creatinine

2 - 6 years: ≤150 mg/g creatinine

7 - 10 years: ≤100 mg/g creatinine

≥11 years: ≤75 mg/g creatinine



≤10 mg/g creatinine

Day(s) Performed


Test Classification

This test was developed, and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. This test has not been cleared or approved by the US Food and Drug Administration.

CPT Code Information


LOINC Code Information

Test ID Test Order Name Order LOINC Value
HYOX Hyperoxaluria Panel, U 53710-0


Result ID Test Result Name Result LOINC Value
50592 Glycolate 13751-3
50593 Glycerate 13749-7
50594 Oxalate 13483-3
38049 4-hydroxy-2-oxoglutarate 13678-8
29982 Interpretation 59462-2
29984 Reviewed By 18771-6

Testing Algorithm

See Hyperoxaluria Diagnostic Algorithm in Special Instructions.

Clinical Information

Increased urinary oxalate frequently leads to renal stone formation and renal insufficiency. Identifying the cause of hyperoxaluria has important implications in therapy, management, and prognosis.


Hyperoxalurias are classified as primary and secondary. Primary hyperoxaluria is an inherited disorder of oxalate metabolism while secondary hyperoxaluria is an acquired condition resulting from either increased intake of dietary oxalate or altered intestinal oxalate absorption. Primary hyperoxalurias are classified into types 1, 2, and 3.


Hyperoxaluria type 1 (PH1) is an autosomal recessive disorder resulting in a deficiency of peroxisomal alanine:glyoxylate aminotransferase due to variants in the AGXT gene. It is characterized by increased urinary oxalic, glyoxylic, and glycolic acids. PH1 is the most common type with manifestations that include deposition of calcium oxalate in the kidneys (nephrolithiasis, nephrocalcinosis) and end-stage kidney disease. Calcium oxalate deposits can be further deposited in other tissues, such as the heart and eyes, and lead to a variety of additional symptoms. Age of onset is variable with a small percentage of patients presenting in the first year of life with failure to thrive, nephrocalcinosis, and metabolic acidosis. Approximately half of affected individuals show manifestations of PH1 in late childhood or early adolescence, and the remainder present in adulthood with recurrent kidney stones. Some individuals with PH1 respond to supplemental pyridoxine therapy.


Hyperoxaluria type 2 (PH 2) is due to a defect in GRHPR gene resulting in a deficiency of the enzyme hydroxypyruvate reductase. PH2 is inherited in an autosomal recessive manner and is identified by an increase in urinary oxalic and glyceric acids. Like PH1, PH2 is characterized by deposition of calcium oxalate in the kidneys (nephrolithiasis, nephrocalcinosis), and end-stage kidney disease. Most individuals have symptoms of PH2 during childhood, and it is thought that PH2 is less common than PH1.


Hyperoxaluria type 3 (PH3), due to recessive variants in HOGA1 (formerly DHDPSL), occurs in a small percentage of individuals with primary hyperoxaluria. HOGA1 encodes a mitochondrial 4-hydroxy-2-oxoglutarate aldolase that catalyzes the 4th step in the hydroxyproline pathway. PH3 is characterized biochemically by increased urinary excretion of oxalate and 4-hydroxy-2-oxoglutarate (HOG). As with PH types 1 and 2, PH type 3 is characterized by calcium-oxalate deposition in the kidneys or kidney stone formation. Most individuals with PH3 have early onset disease with recurrent kidney stones and urinary tract infections as common symptoms. End-stage kidney disease is not a characteristic of PH3. Of note, individuals with heterozygous variants in HOGA1 can have variable and intermittent elevations of urine oxalate.


Secondary hyperoxalurias are due to hyperabsorption of oxalate (enteric hyperoxaluria); total parenteral nutrition in premature infants; ingestion of oxalate, ascorbic acid, or ethylene glycol; or pyridoxine deficiency, and may respond to appropriate therapy.


A diagnostic workup in an individual with hyperoxaluria demonstrates increased concentration of oxalate in urinary metabolite screening. If glycolate, glycerate, or HOG is present, a primary hyperoxaluria is indicated. Additional analyses can include molecular testing for PH1 (AGXTZ / AGXT Gene, Full Gene Analysis, Varies), PH2 (GRHPZ / GRHPR Gene, Full Gene Analysis, Varies), or PH3 (HOGA1 sequencing).


Increased concentrations of oxalate and glycolate indicate type 1 hyperoxaluria.


Increased concentrations of oxalate and glycerate indicate type 2 hyperoxaluria.


Increased concentrations of oxalate and 4-hydroxy-2-oxoglutarate indicate type 3 hyperoxaluria.


Increased concentrations of oxalate with normal concentrations of glycolate, glycerate, and 4-hydroxy-2-oxoglutarate indicate secondary hyperoxaluria.

Clinical Reference

1. Bhasin B, Urekli HM, Atta MG: Primary and secondary hyperoxaluria: Understanding the enigma. World J Nephrol. 2015;4(2):235-244. doi: 10.5527/wjn.v4.i2.235

2. Lorenzo V, Torres A, Salido E. Primary hyperoxaluria. Nefrologia. 2014;34(3):398-412

3. Milliner DS, Harris PC, Cogal AG, et al. Primary hyperoxaluria type 1. In: Adam MP, Ardinger HH, Pagon RA, et al, eds. GeneReviews [Internet]. University of Washington, Seattle; 2002. Updated Nov.30,2017. Accessed September 23, 2021. Available at:

4. Rumsby G, Hulton SA. Primary hyperoxaluria type 2. In: Adam MP, Ardinger HH, Pagon RA, et al, eds. GeneReviews [Internet]. University of Washington, Seattle; 2008. Updated Dec. 21,2017. Accessed September 23, 2021. Available at:

5. Milliner DS, Harris PC, Lieske JC. Primary hyperoxaluria type 3. In: Adam MP, Ardinger HH, Pagon RA, et al, eds. GeneReviews [Internet]. University of Washington, Seattle; 2015. Accessed September 23, 2021. Available at:

6. Fraser AD: Importance of glycolic acid analysis in ethylene glycol poisoning. Clin Chem. 1998;44(8):1769

Report Available

9 days

Method Name

Gas Chromatography-Mass Spectrometry (GC-MS)


If not ordering electronically, complete, print, and send 1 of the following forms with the specimen:

-Biochemical Genetics Test Request (T798)

-Renal Diagnostics Test Request (T830)

Mayo Clinic Laboratories | Genetics and Pharmacogenomics Catalog Additional Information: