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C5/C3 IVA

Complement

C5/C3C5 to C3 ratioIsovalerylcarnitine to propionylcarnitine ratio

Review status

Currently under review

Pending specialist review and validation.

What it shows

This test measures the ratio of C5 (isovalerylcarnitine) to C3 (propionylcarnitine) in blood. These are acylcarnitines, small molecules that reflect how your body breaks down certain amino acids and fats. The measurement is typically performed by tandem mass spectrometry, often on a dried blood spot or plasma sample.

The ratio helps identify a biochemical pattern associated with isovaleric acidemia, a rare inherited condition that affects the enzyme isovaleryl-CoA dehydrogenase. On its own, the ratio is a screening or adjunct marker and is interpreted together with other lab findings and clinical information.

Why it matters

Doctors use this ratio to help flag possible isovaleric acidemia, especially in newborn screening or when someone has symptoms that could suggest a metabolic disorder. Finding a concerning pattern early can guide confirmatory testing and prompt treatment to prevent metabolic crises.

The result can also support monitoring in people already diagnosed with the condition. Because many factors can shift acylcarnitine patterns, the ratio is not diagnostic by itself; it helps your care team decide whether further evaluation is needed.

Understanding your results

If the ratio is higher than expected, it raises suspicion for isovaleric acidemia, but it does not confirm the diagnosis. Your clinician may repeat the test and order additional studies, such as a full acylcarnitine profile, urine organic acids to look for isovalerylglycine, and sometimes genetic testing. Results are interpreted in the context of symptoms, diet, medications, and age.

If the ratio is within the expected range, that is generally reassuring, although follow-up may still be recommended if there are persistent symptoms or other test abnormalities. When results are borderline or influenced by feeding status or illness, a repeat sample after recovery or after regular feeding has been established can clarify the picture.

Reference ranges

00.45 nan
All sexes
0 days – 150 years

Reference intervals vary by laboratory, analyzer, methodology, population, and units. The ranges shown here are for education only. Always interpret your results against the reference interval printed on your own lab report.

Factors that could impact C5/C3 IVA

  • Age and feeding status

    Very early collection and limited feeding can shift acylcarnitines, especially propionylcarnitine, which may change the ratio. Results often stabilize after regular feeding is established.

  • Illness and fasting

    Fever, infection, dehydration, or prolonged fasting can increase catabolism, altering acylcarnitine patterns and potentially raising the ratio temporarily.

  • Medications and supplements

    Pivalate-containing antibiotics, valproate, and some topical or oral products that release pivalic acid can raise certain acylcarnitines. Carnitine or glycine supplements may also influence results.

  • Sample quality and handling

    Improperly collected or stored dried blood spots, hemolysis, or excessive heat and humidity can degrade analytes and lead to inaccurate ratios.

  • Dietary protein and anabolic state

    High protein intake, low caloric intake, or a catabolic state can modify leucine metabolism and acylcarnitine profiles, affecting the ratio.

  • Prematurity and maternal influences

    Premature infants and those with low carnitine stores may have different acylcarnitine patterns. Maternal metabolic status, diet, or medications can also affect newborn results.

2026

References

  1. McGill University Health Centre. (2018, December 04). C5/C3 IVA (Task CD 22048155). Laboratory reference ranges.
  2. American College of Medical Genetics and Genomics. (2022). ACT sheet: Elevated C5 acylcarnitine (Isovaleric acidemia).
  3. Clinical and Laboratory Standards Institute. (2017). Newborn screening by tandem mass spectrometry; Approved guideline (2nd ed.).