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Aminoaciduria Test – Indications, Procedure, Results

Last updated: February 8, 2026 Medically reviewed by: RxHarun Orthopedic Specialist Doctor Reading time: 5 min read

Aminoaciduria is an abnormal amount of amino acids in the urine. Amino acids are the building blocks for proteins in the body.

How the Test is Performed

A clean-catch urine sample is needed. This is often done at your doctor’s office or health clinic.

How to Prepare for the Test

Most of the time you do not need to take special steps before this test. Make sure your doctor knows all of the medicines you recently used. If this test is being done on an infant who is breastfeeding, make sure the health care provider knows what medicines the nursing mother is taking.

How the Test will Feel

The test involves only normal urination.

Why the Test is Performed

This test is done to measure amino acid levels in the urine. There are many different types of amino acids. It is common for some of each kind to be found in the urine. Increased levels of individual amino acids can be a sign of a problem with metabolism.

Normal Results

The specific value is measured in micromoles per deciliter (micromol/dL) or micromoles per liter (micromol/l).

Alanine

  • Children: 65 to 190 micromol/dL or 650 to 1,900 micromol/L
  • Adults: 160 to 690 micromol/dL or 1600 to 6,900 micromol/L

Alpha-aminoadipic acid

  • Children: 25 to 78 micromol/dL or 250 to 780 micromol/L
  • Adults: 0 to 165 micromol/dL or 0 to 1,650 micromol/L

Alpha-amino-N-butyric acid

  • Children: 7 to 25 micromol/dL or 70 to 250 micromol/L
  • Adults: 0 to 28 micromol/dL or 0 to 280 micromol/L

Arginine

  • Children: 10 to 25 micromol/dL or 100 to 250 micromol/L
  • Adults: 13 to 64 micromol/dL or 130 to 640 micromol/L

Asparagine

  • Children: 15 to 40 micromol/dL or 150 to 400 micromol/L
  • Adults: 34 to 100 micromol/dL or 340 to 1,000 micromol/L

Aspartic acid

  • Children: 10 to 26 micromol/dL or 100 to 260 micromol/L
  • Adults: 14 to 89 micromol/dL or 140 to 890 micromol/L

Beta-alanine

  • Children: 0 to 42 micromol/dL or 0 to 420 micromol/L
  • Adults: 0 to 93 micromol/dL or 0 to 930 micromol/L

Beta-amino-isobutyric acid

  • Children: 25 to 96 micromol/dL or 250 to 960 micromol/L
  • Adults: 10 to 235 micromol/dL or 100 to 2,350 micromol/L

Carnosine

  • Children: 34 to 220 micromol/dL or 340 to 2,200 micromol/L
  • Adults: 16 to 125 micromol/dL or 160 to 1,250 micromol/L

Citrulline

  • Children: 0 to 13 micromol/dL or 0 to 130 micromol/L
  • Adults: 0 to 11 micromol/dL or 0 to 110 micromol/L

Cystine

  • Children: 11 to 53 micromol/dL or 110 to 530 micromol/L
  • Adults: 28 to 115 micromol/dL or 280 to 1,150 micromol/L

Glutamic acid

  • Children: 13 to 22 micromol/dL or 130 to 220 micromol/L
  • Adults: 27 to 105 micromol/dL or 270 to 1,050 micromol/L

Glutamine

  • Children: 150 to 400 micromol/dL or 1,500 to 4,000 micromol/L
  • Adults: 300 to 1,040 micromol/dL or 3,000 to 10,400 micromol/L

Glycine

  • Children: 195 to 855 micromol/dL or 1,950 to 8,550 micromol/L
  • Adults: 750 to 2,400 micromol/dL or 7,500 to 24,000 micromol/L

Histidine

  • Children: 46 to 725 micromol/dL or 460 to 7,250 micromol/L
  • Adults: 500 to 1500 micromol/dL or 5,000 to 15,000 micromol/L

Hydroxyproline

  • Children: not measured
  • Adults: not measured

Isoleucine

  • Children: 3 to 15 micromol/dL or 30 to 150 micromol/L
  • Adults: 4 to 23 micromol/dL or 40 to 230 micromol/L

Leucine

  • Children: 9 to 23 micromol/dL or 90 to 230 micromol/L
  • Adults: 20 to 77 micromol/dL or 200 to 770 micromol/L

Lysine

  • Children: 19 to 140 micromol/dL or 190 to 1,400 micromol/L
  • Adults: 32 to 290 micromol/dL or 320 to 2,900 micromol/L

Methionine

  • Children: 7 to 20 micromol/dL or 70 to 200 micromol/L
  • Adults: 5 to 30 micromol/dL or 50 to 300 micromol/L

1-methylhistidine

  • Children: 41 to 300 micromol/dL or 410 to 3,000 micromol/L
  • Adults: 68 to 855 micromol/dL or 680 to 8,550 micromol/L

3-methylhistidine

  • Children: 42 to 135 micromol/dL or 420 to 1,350 micromol/L
  • Adults: 64 to 320 micromol/dL or 640 to 3,200 micromol/L

Ornithine

  • Children: 3 to 16 micromol/dL or 30 to 160 micromol/L
  • Adults: 5 to 70 micromol/dL or 50 to 700 micromol/L

Phenylalanine

  • Children: 20 to 61 micromol/dL or 200 to 610 micromol/L
  • Adults: 36 to 90 micromol/dL or 360 to 900 micromol/L

Phosphoserine

  • Children: 16 to 34 micromol/dL or 160 to 340 micromol/L
  • Adults: 28 to 95 micromol/dL or 280 to 950 micromol/L

Phosphoethanolamine

  • Children: 24 to 66 micromol/dL or 240 to 660 micromol/L
  • Adults: 17 to 95 micromol/dL or 170 to 950 micromol/L

Proline

  • Children: not measured
  • Adults: not measured

Serine

  • Children: 93 to 210 micromol/dL or 930 to 2,100 micromol/L
  • Adults: 200 to 695 micromol/dL or 2,000 to 6,950 micromol/L

Taurine

  • Children: 62 to 970 micromol/dL or 620 to 9,700 micromol/L
  • Adults: 267 to 1290 micromol/dL or 2,670 to 12,900 micromol/L

Threonine

  • Children: 25 to 100 micromol/dL or 250 to 1,000 micromol/L
  • Adults: 80 to 320 micromol/dL or 800 to 3,200 micromol/L

Tyrosine

  • Children: 30 to 83 micromol/dL or 300 to 830 micromol/L
  • Adults: 38 to 145 micromol/dL or 380 1,450 micromol/L

Valine

  • Children: 17 to 37 micromol/dL or 170 to 370 micromol/L
  • Adults: 19 to 74 micromol/dL or 190 to 740 micromol/

Normal value ranges may vary slightly among different laboratories. Talk to your health care provider about the meaning of your specific test results.

The examples above show the common measurements for results for these tests. Some laboratories use different measurements or may test different specimens.

What Abnormal Results Mean

Increased total urine amino acids may be due to:

  • Alkaptonuria
  • Canavan disease
  • Cystinosis
  • Cystathioninuria
  • Fructose intolerance
  • Galactosemia
  • Hartnup disease
  • Homocystinuria
  • Hyperammonemia
  • Hyperparathyroidism
  • Maple syrup urine disease
  • Methylmalonic acidemia
  • Multiple myeloma
  • Ornithine transcarbamylase deficiency
  • Osteomalacia
  • Propionic acidemia
  • Rickets
  • Tyrosinemia type 1
  • Tyrosinemia type 2
  • Viral hepatitis
  • Wilson disease

Considerations

Screening infants for increased levels of amino acids can help detect problems with metabolism. Early treatment for these conditions may prevent complications in the future.

 

Frequently Asked Questions

Is this article a replacement for a doctor?

No. It is educational content only. Patients should consult a qualified clinician for diagnosis and treatment.

When should I seek urgent care?

Seek urgent care for severe symptoms, rapidly worsening condition, breathing difficulty, severe pain, neurological changes, or any emergency warning sign.

References

Add references, clinical guidelines, textbooks, journal articles, or trusted medical sources here. You can edit this area later with a custom field named _rx_references.

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Written by Dr. Harun Ar Rashid, MD - Arthritis, Bones, Joints Pain, Trauma, and Internal Medicine Specialist

Dr. Md. Harun Ar Rashid, MPH, MD, PhD, is a highly respected medical specialist celebrated for his exceptional clinical expertise and unwavering commitment to patient care. With advanced qualifications including MPH, MD, and PhD, he integrates cutting-edge research with a compassionate approach to medicine, ensuring that every patient receives personalized and effective treatment. His extensive training and hands-on experience enable him to diagnose complex conditions accurately and develop innovative treatment strategies tailored to individual needs. In addition to his clinical practice, Dr. Harun Ar Rashid is dedicated to medical education and research, writing and inventory creative thinking, innovative idea, critical care managementing make in his community to outreach, often participating in initiatives that promote health awareness and advance medical knowledge. His career is a testament to the high standards represented by his credentials, and he continues to contribute significantly to his field, driving improvements in both patient outcomes and healthcare practices. Born and educated in Bangladesh, Dr. Rashid earned his BPT from the University of Dhaka before pursuing postgraduate training internationally. He completed his MD in Internal Medicine at King’s College London, where he developed a special interest in inflammatory arthritis and metabolic bone disease. He then undertook a PhD in Orthopedic Science at the University of Oxford, conducting pioneering research on cytokine signaling pathways in rheumatoid arthritis. Following his doctoral studies, Dr. Rashid returned to clinical work with a fellowship in interventional pain management at the Rx University School of Medicine, refining his skills in image-guided joint injections and minimally invasive pain-relief techniques.