Ordering Recommendation

Evaluate suspected aminolevulinic acid dehydratase deficiency (ADP) porphyria or hereditary tyrosinemia.

New York DOH Approval Status

This test is New York state approved.

Specimen Required

Patient Preparation

Refrain from alcohol consumption 24 hours prior to collection.

Collect

24 hour or random urine. Refrigerate 24-hour specimens during collection.

Specimen Preparation

Transfer a 4 mL aliquot from a well-mixed 24 hour or random collection to an ARUP Standard Transport Tube. (Min: 1.2 mL)

Storage/Transport Temperature

Refrigerated.

Unacceptable Conditions

Body fluids other than urine.

Remarks

Record total volume and collection time interval on transport tube and test request form.

Stability

Ambient: Unacceptable; Refrigerated: 2 weeks; Frozen: 1 month

Methodology

Quantitative Ion Exchange Chromatography/Spectrophotometry

Performed

Mon, Wed, Fri

Reported

1-4 days

Reference Interval

Test Number
Components
Reference Interval
  Aminolevulinic Acid - per volume 0-35 µmol/L
  Aminolevulinic Acid - per 24h 0-60 µmol/d
  Creatinine, Urine - per 24h
Age
Male (mg/d)
Female (mg/d)
3-8 years 140-700 140-700
9-12 years 300-1300 300-1300
13-17 years 500-2300 400-1600
18-50 years 1000-2500 700-1600
51-80 years 800-2100 500-1400
81 years and older 600-2000 400-1300

Interpretive Data

Per 24h calculations are provided to aid interpretation for collections with a duration of 24 hours and an average daily urine volume. For specimens with notable deviations in collection time or volume, ratios of analytes to a corresponding urine creatinine concentration may assist in result interpretation.

Compliance Category

Standard

Note

Increased ALA concentration is associated with exposure to alcohol, lead, and a variety of other agents.  Massive elevation of ALA occurs in the acute porphyrias and hereditary tyrosinemia.

Specimen preservation with acid or base is discouraged and may cause assay interference.  When collecting urine for additional tests that require acid or base preservation, the ALA aliquot should be removed prior to the addition of the acid or base.

Hotline History

N/A

CPT Codes

82135

Components

Component Test Code* Component Chart Name LOINC
0020207 Creatinine, Urine - per volume 2161-8
0020208 Creatinine, Urine - per 24h 2162-6
0080104 Aminolevulinic Acid - per volume 34284-0
0080105 Aminolevulinic Acid - per 24h 14689-4
0097110 Total Volume 19153-6
0097111 Hours Collected 30211-7
* Component test codes cannot be used to order tests. The information provided here is not sufficient for interface builds; for a complete test mix, please click the sidebar link to access the Interface Map.

Aliases

  • 5-Aminolevulinic Acid
  • ALA
  • Delta-ALA
  • Delta-Aminolevulinic Acid
Aminolevulinic Acid (ALA), Urine