Understanding Your Laboratory Test: A Patient's Guide to Testing, Results, and Follow-Up Care
This blood test measures the ratio of 3-hydroxyisovalerylcarnitine (C5-OH) to free carnitine (C0) to help detect certain metabolic disorders.
{"Screens for metabolic disorders that affect how the body processes certain amino acids","Elevated C5-OH with low free carnitine may indicate conditions like multiple carboxylase deficiency","Commonly used in newborn screening programs","A small blood sample is all that is needed for testing","Early detection allows for prompt treatment to prevent complications"}
Key facts at a glance
- LOINC
- 53171-5
- Short code
- C5OH-C0 ratio
- Synonyms
- 3OH-Isovalerylcarn/C0 DBS-sRto
- Category
- chemistry
- Sample
- serum
- Container
- Dried blood spot (DBS) collection card (Guthrie card)
- Units
- N/A
- Ref low
- 0.01
- Ref high
- 0.2
- Critical range
- — – 0.3 N/A
- Fasting
- false
- Population
- adult
- Sex
- all
- Age range
- 0–30 years
- Method
- Tandem Mass Spectrometry (MS/MS)
- CPT
- 82520
- SNOMED
- 390906007
- Specimen volume
- One complete dried blood spot (approximately 3.2 mm diameter punch)
- Stability
- DBS specimens are stable at room temperature for several days; store at 2-8°C for extended storage up to 1-2 weeks; avoid humidity and direct sunlight
- Hemolysis max
- 500
- Lipemia max
- 1000
- Icterus max
- 500
- TAT
- 24–72 hours
- Order
- routine
This test is a laboratory analysis performed on a sample of your blood, urine, or other body tissue to help your doctor evaluate how well your body is functioning and to detect signs of disease, infection, or other health conditions that may require further medical attention.
The C5-OH/C0 Carnitine Ratio test measures two substances in the blood: 3-hydroxyisovalerylcarnitine (C5-OH) and free carnitine (C0). Carnitine is a substance that helps the body convert fat into energy. When the body cannot properly process certain amino acids (protein building blocks), C5-OH can accumulate in the blood. This test compares the amount of C5-OH to free carnitine to help doctors determine if a metabolic disorder is present.
A trained healthcare professional will collect a small sample, usually by drawing blood from a vein in your arm with a sterile needle, or by asking you to provide a urine specimen. The collection process is quick, typically taking only a few minutes, and your sample is then sent to a certified laboratory for careful analysis.
A healthcare professional will collect a small blood sample, typically from a vein in the arm or from a finger prick. For newborn screening, the test is often done using a few drops of blood from the baby's heel placed on a special filter paper card (dried blood spot). The sample is then sent to a laboratory for analysis.
This test plays a key role in your overall health care because it provides objective information about your body that cannot be gathered from a physical examination alone. It helps confirm or rule out a diagnosis, monitor an existing condition, guide treatment decisions, and screen for potential problems before noticeable symptoms develop.
This test is primarily used to screen for inherited metabolic disorders, particularly multiple carboxylase deficiency and 3-methylcrotonyl-CoA carboxylase (3-MCC) deficiency. These conditions prevent the body from properly breaking down certain amino acids, leading to a buildup of harmful substances. Early detection through newborn screening allows for early treatment, which can prevent serious health problems including developmental delays, seizures, and organ damage.
Your results will be compared with established reference ranges, and values that fall outside the normal range may point to an underlying condition that needs attention. However, abnormal results do not always mean you are ill, since medications, diet, hydration, and normal body variations can affect them. Your doctor will review the findings with you, explain what they mean in your specific situation, and recommend any follow-up testing or treatment if needed.
An elevated C5-OH/C0 ratio may suggest a metabolic disorder. However, results must be interpreted by your healthcare provider in context with other clinical findings. If the test shows abnormal results, follow-up testing is usually recommended to confirm the diagnosis. It is important to remember that an abnormal screening result does not always mean the baby has the condition - false positives can occur, and confirmatory testing is always needed.
Reference range
C5-OH/C0 molar ratio: 0.01-0.20 (typical reference range for newborns)N/A
No special preparation required. This is a routine newborn screening test performed on dried blood spot specimens collected within 24-72 hours of birth. Ensure proper collection technique on Guthrie card allowing complete drying before transport.
Clinical interpretation
How abnormal results are interpreted and what conditions they suggest.
The C5-OH/C0 ratio is primarily used to detect multiple carboxylase deficiency (MCD), which includes biotinidase deficiency and holocarboxylase synthetase deficiency. These are rare inherited metabolic disorders where the body cannot properly use biotin (vitamin B7). Elevated C5-OH levels indicate that certain amino acids are not being metabolized correctly. The ratio is particularly useful because it accounts for overall carnitine status - in these conditions, free carnitine (C0) is often low because it is used to form excess acylcarnitines. Treatment typically involves biotin supplementation and dietary management, making early detection crucial for preventing long-term complications.
Frequently asked questions
Is fasting required before this test?
What happens if my baby's test result is abnormal?
Is this test only for newborns?
How accurate is this screening test?
Sources
- 1
- 2
- 3
Click any link to access the full source. PubMed Central (PMC) links provide free full-text access.
This information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 16, 2026. · Read our editorial policy