Aminoaciduria – Indications, Procedure, Results

Last updated: February 8, 2026Reviewed date: February 8, 2026Reading time: 4 min read
Patient Tools

Read, save, and share this guide

Use these quick tools to make this medical article easier to read, print, save, or share with a family member.

Patient Mode

Understand this article easily

Switch between simple English and easy Bangla patient notes. This is for education and does not replace a doctor consultation.

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 severe symptoms, danger signs, pregnancy, child illness, or sudden worsening, seek urgent medical care.

বাংলা রোগী নোট এখনো যোগ করা হয়নি। পোস্ট এডিটরে “RX Bangla Patient Mode” বক্স থেকে সহজ বাংলা সারাংশ যোগ করুন।

এই তথ্য শিক্ষা ও সচেতনতার জন্য। এটি ডাক্তারি পরীক্ষা, রোগ নির্ণয় বা প্রেসক্রিপশনের বিকল্প নয়।

Article Summary

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...

Key Takeaways

  • This article explains How the Test is Performed in simple medical language.
  • This article explains How to Prepare for the Test in simple medical language.
  • This article explains How the Test will Feel in simple medical language.
  • This article explains Why the Test is Performed in simple medical language.
Educational health guideWritten for patient understanding and clinical awareness.
Reviewed content workflowUse writer and reviewer profiles for stronger trust.
Emergency safety firstUrgent warning signs are highlighted below.
Choose your reading view

Patient View highlights a simple learning journey. Clinical View reveals structure, evidence, and editorial completeness.

Seek urgent medical care if you notice

These warning signs are general safety guidance. Local emergency numbers and clinical judgment should always come first.

  • Severe symptoms, breathing difficulty, fainting, confusion, or rapidly worsening illness.
  • New weakness, severe pain, high fever, or symptoms after a serious injury.
  • Any symptom that feels urgent, unusual, or unsafe for the patient.
1

Emergency now

Use emergency care for severe, sudden, rapidly worsening, or life-threatening symptoms.

2

See a doctor

Book a professional medical evaluation if symptoms persist, worsen, recur often, affect daily activities, or occur in a high-risk patient.

3

Learn safely

Use this article to understand possible causes, tests, treatment options, prevention, and questions to ask your clinician.

Before reading

RX Patient Tools

Use these quick guides before reading the article, or return to them when you need help preparing questions for a doctor.

Start here Choose the right pathway for symptoms, reports, medicines, or urgent warning signs. Disease article roadmap Read this topic step by step: meaning, symptoms, warning signs, diagnosis, treatment, prevention, and follow-up. Treatment planner Prepare questions about treatment choices, benefits, risks, side effects, and follow-up. Family & caregiver guide Organize symptoms, reports, medicines, questions, and follow-up safely. Nutrition & diet guide Prepare food, hydration, supplement, and medicine-timing questions safely. Prevention guide Organize risk factors, protective habits, screening, and warning signs. Recovery guide Prepare a safe plan for activity, rehabilitation, warning signs, and follow-up.
Definition

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.

RX Clinical Pathway Engine

Continue through a complete learning pathway

Move from understanding the topic to symptoms, tests, treatment, medicines, monitoring, and prevention.

Search the complete library
  1. Understand the condition Begin with the essential facts and a clear explanation of the topic.
  2. Recognize symptoms Learn common symptoms, signs, and patterns of presentation.
  3. Know when to seek help Review urgent warning signs and when professional assessment may be needed.
  4. Understand causes and risks Explore causes, risk factors, mechanisms, and contributing conditions.
  5. Explore tests and diagnosis Learn how clinicians assess the condition and which investigations may be discussed.
  6. Learn treatment approaches Review general treatment categories and management principles.
  7. Understand medicines safely Continue to medicine education, uses, precautions, and monitoring.
  8. Plan monitoring and follow-up Understand monitoring, complications, rehabilitation, and follow-up learning.
  9. Review prevention and self-care Explore prevention, healthy routines, and questions to discuss with a clinician.

Conditions & Diseases

Background, symptoms, causes, diagnosis, and care.

Explore this library

Tests & Investigations

Laboratory, imaging, screening, and diagnostic education.

Explore this library

Medicines

Uses, safety, monitoring, and related medicine knowledge.

Explore this library
Doctor visit helper

Prepare before seeing a doctor

A simple rural-patient checklist to help you explain symptoms clearly, ask better questions, and avoid unsafe self-treatment.

Safety note: This is not a prescription or diagnosis. For severe symptoms, pregnancy danger signs, children with serious illness, chest pain, breathing difficulty, stroke-like weakness, or major injury, seek urgent care.

Which doctor may help?

Start with a registered doctor or the nearest qualified health center.

What to tell the doctor

  • Write when the problem started and how it changed.
  • Bring old prescriptions, investigation reports, and current medicines.
  • Write allergies, pregnancy status, diabetes, kidney/liver disease, and major past illnesses.
  • Bring one family member if the patient is weak, elderly, confused, or a child.

Questions to ask

  • What is the most likely cause of my symptoms?
  • Which danger signs mean I should go to hospital quickly?
  • Which tests are necessary now, and which can wait?
  • How should I take medicines safely and what side effects should I watch for?
  • When should I come for follow-up?

Tests to discuss

  • Vital signs: temperature, pulse, blood pressure, oxygen saturation
  • Basic physical examination by a clinician
  • CBC, urine test, blood sugar, or imaging only when clinically needed

Avoid these mistakes

  • Do not use antibiotics, steroid tablets/injections, or strong painkillers without proper medical advice.
  • Do not hide pregnancy, kidney disease, ulcer, allergy, or blood thinner use.
  • Do not delay emergency care when danger signs are present.

Medicine safety and first-aid guide

This section is for patient education only. It does not replace a doctor, pharmacist, or emergency care.

Safe first steps

  • Rest, drink safe water, and observe symptoms carefully.
  • Keep a written note of symptoms, duration, temperature, medicines already taken, and allergy history.
  • Seek medical care quickly if symptoms are severe, worsening, or unusual for the patient.

OTC medicine safety

  • For mild pain or fever, ask a registered pharmacist or doctor before using common over-the-counter pain/fever medicines.
  • Do not combine multiple pain medicines without advice, especially if you have kidney disease, liver disease, stomach ulcer, asthma, pregnancy, or take blood thinners.
  • Do not give adult medicines to children unless a qualified clinician advises it.

Avoid these mistakes

  • Do not start antibiotics without a proper medical decision.
  • Do not use steroid tablets or injections casually for quick relief.
  • Do not delay emergency care because of home remedies.

Get urgent help if

  • Severe symptoms, confusion, fainting, breathing difficulty, chest pain, severe dehydration, or sudden weakness need urgent medical care.
Medicine names, dose, and timing must be decided by a qualified clinician or pharmacist after checking age, pregnancy, allergy, other diseases, and current medicines.

For rural patients and family caregivers

Patient health record and symptom diary

Write your symptoms, medicines already taken, test results, and questions before visiting a doctor. This note stays on your device unless you print or copy it.

Doctor to discuss: Doctor / qualified healthcare provider
Tests to discuss with doctor
  • Basic vital signs: temperature, pulse, blood pressure, oxygen level if needed
  • Relevant blood, urine, imaging, or specialist tests only after clinical assessment
Questions to ask
  • What is the most likely cause of my symptoms?
  • Which warning signs mean I should go to emergency care?
  • Which tests are really needed now?
  • Which medicines are safe for my age, pregnancy status, allergy, kidney/liver/stomach condition, and current medicines?

Emergency warning signs such as chest pain, severe breathing difficulty, sudden weakness, confusion, severe dehydration, major injury, or loss of bladder/bowel control need urgent medical care. Do not wait for online information.

Safe pathway to proper treatment

Care roadmap for: Aminoaciduria – Indications, Procedure, Results

Use this simple roadmap to understand the next safe steps. It is educational and does not replace examination by a doctor.

Go to emergency care if you notice:
  • Severe or rapidly worsening symptoms
  • Breathing difficulty, chest pain, fainting, confusion, severe weakness, major injury, or severe dehydration
Doctor / service to discuss: Qualified healthcare provider; specialist depends on symptoms and examination.
  1. Step 1

    Check danger signs first

    If danger signs are present, seek emergency care and do not wait for online information.

  2. Step 2

    Record the symptom story

    Write when symptoms started, severity, medicines already taken, allergies, pregnancy status, and test results.

  3. Step 3

    Visit a qualified clinician

    A doctor, nurse, or qualified healthcare provider can examine you and decide which tests or treatment are needed.

  4. Step 4

    Do only useful tests

    Do tests after clinical assessment. Avoid unnecessary tests, random antibiotics, or repeated medicines without diagnosis.

  5. Step 5

    Follow up and return early if worse

    If symptoms worsen, new warning signs appear, or treatment is not helping, return for review quickly.

Rural patient practical tips
  • Take a written symptom diary and all previous prescriptions/test reports.
  • Do not hide medicines already taken, even herbal or over-the-counter medicines.
  • Ask which warning signs mean urgent referral to hospital.

This roadmap is for education. A real diagnosis and treatment plan requires history, examination, and clinical judgment.

RX Patient Help

Ask a health question safely

Write your symptom story. A health professional or site editor can review it before any answer is prepared. This box is not for emergency care.

Emergency first: Severe chest pain, breathing trouble, unconsciousness, stroke signs, severe injury, heavy bleeding, or rapidly worsening symptoms need urgent local medical care now.

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.

Continue exploring

Explore this topic across the RX Medical Library

Open a focused A–Z pathway or continue with closely related indexed articles. These links are educational and do not replace personal medical care.

Search this topic
Diseases A–Z Drugs A–Z Lab Tests A–Z Cancer A–Z
Diseases A–Z

The abdominoplasty commonly referred to as a "tummy tuck," is a procedure to reduce the excess…

More from this topic
  1. Hemoglobin A1c Test

    DefinitionThe hemoglobin? A1c test—also known as glycated hemoglobin?, glycosylated hemoglobin, HbA1c, or simply A1c—is used to measure an individual’s glucose control levels. The test shows average blood sugar...

    November 6, 2025 • Rx Lab Test (A - Z)
  2. Orbital Decompression Surgery

    DefinitionOrbital decompression is an operation to make more room inside the eye socket. The eye socket is called the “orbit.” It is a cone-shaped space made...

    August 18, 2025 • Rx Lab Test (A - Z)
  3. Olfactory Nerve Disorders

    DefinitionOlfactory nerve disorders affect our sense of smell. This can impact our ability to enjoy food, detect danger like gas leaks, or even notice warning signs...

    May 8, 2024 • Rx Lab Test (A - Z)
  4. Biological Terrain Assessment (BTA)

    DefinitionBiological Terrain Assessment (BTA) is a holistic approach to understanding the health of our bodies by examining various aspects of our biological terrain. In simpler terms,...

    December 15, 2023 • Rx Lab Test (A - Z)
  5. Ingrown Toenai – onychocryptosis or uunguisincarnatus

    DefinitionIngrown toenail, also known as onychocryptosis or uunguisincarnatus, is the most common nail problem encountered in both general practice and dermatology.[rx] An ingrown toenail occurs when the nail...

    July 8, 2022 • Rx Lab Test (A - Z)
  6. Physical Exam HEENT

    DefinitionHEENT stands for “head, eyes, ears, nose, and throat.” If someone is experiencing symptoms that affect those areas, such as sneezing, congestion, and a sore throat,...

    May 6, 2022 • Rx Lab Test (A - Z)
  7. Thyroid Function Tests

    Thyroid? Function tests are used to tell if your thyroid? gland is functioning properly by measuring the number of thyroid hormones in your blood. They are...

    April 15, 2022 • Rx Lab Test (A - Z)
  8. Thyroid Gland Function Tests – Indications, Procedures, Results

    The TSH test and the T4 test are the two most common thyroid? function tests. The TSH test is often done first because it’s the best way...

    April 15, 2022 • Rx Lab Test (A - Z)
  9. TIBC Test

    DefinitionA total iron-binding capacity (TIBC) test is a blood test to see if you have too much or too little iron in your blood measures the...

    April 15, 2022 • Rx Lab Test (A - Z)
  10. Shaded Surface Display (SSD) Test

    A shaded surface display (SSD) test (sometimes called feature-extraction or iso-surfacing) is a surface-rendered 3D image-producing system that provides a realistically looking three-dimensional view image of...

    April 9, 2022 • Rx Lab Test (A - Z)