ALA/Creatinine Ratio
What is the ALA/Creatinine Ratio?
The ALA/Creatinine Ratio measures the amount of delta-aminolevulinic acid (ALA) in your urine, standardized against creatinine to account for urine concentration differences.
ALA is a key intermediate in the heme synthesis pathway, which your body uses to make heme—a crucial component of hemoglobin in red blood cells.
This ratio is especially useful in diagnosing acute porphyrias and other disorders of heme metabolism. By adjusting for creatinine, the test gives a more accurate view of ALA excretion regardless of how diluted or concentrated the urine is.
Why is this test ordered?
This test is often ordered when a healthcare provider suspects a porphyria, particularly:
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Acute Intermittent Porphyria (AIP)
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Hereditary Coproporphyria (HCP)
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Variegate Porphyria (VP)
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Lead poisoning
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Tyrosinemia Type I (in infants/children)
You may be asked to do this test if you're experiencing unexplained, recurring symptoms, such as:
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Severe abdominal pain
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Nausea or vomiting
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Muscle weakness
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Anxiety, confusion, or hallucinations
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Seizures
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Reddish or dark urine (especially during acute episodes)
What does an elevated ALA/Creatinine Ratio mean?
An elevated ALA/Creatinine Ratio typically indicates increased production and urinary excretion of delta-aminolevulinic acid. This often points to disrupted heme biosynthesis, and is most commonly seen in:
1. Acute Hepatic Porphyrias
When an enzyme deficiency prevents the body from completing heme synthesis, intermediates like ALA build up and spill into the urine. ALA and porphobilinogen (PBG) are both elevated during acute attacks.
2. Lead Poisoning
Lead inhibits multiple enzymes in the heme synthesis pathway, especially ALA dehydratase, causing a marked rise in urinary ALA without a proportional rise in PBG. This pattern helps distinguish lead toxicity from porphyria.
3. Tyrosinemia Type I
In infants, especially those with failure to thrive, liver dysfunction, or neurological symptoms, high ALA levels may point to this rare metabolic disorder.
What should you do if your ALA/Creatinine Ratio is elevated?
If your ALA/Creatinine Ratio is high:
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Your doctor may order additional confirmatory tests, including:
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Porphobilinogen (PBG)/Creatinine Ratio
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Total urine porphyrins
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Blood lead levels
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Genetic testing for porphyrias
-
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If porphyria is suspected, it's critical to collect urine during an acute attack, as levels may normalize between episodes.
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Avoid known triggers of porphyria attacks, such as:
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Fasting or crash diets
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Alcohol
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Hormonal shifts (e.g., menstruation)
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Certain prescription drugs (barbiturates, sulfa drugs, some anticonvulsants)
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If lead poisoning is suspected, urgent chelation therapy and environmental investigation may be required.
How is this different from just measuring ALA?
Urine concentration can vary widely depending on hydration. Measuring ALA alone can be misleading—dilute urine might show a falsely low level. By expressing it as a ratio to creatinine, clinicians get a clearer, more reliable picture of your body’s ALA excretion, allowing for better diagnostic accuracy.
Bottom Line
An elevated ALA/Creatinine Ratio in random urine is a strong biochemical clue for acute porphyria, lead poisoning, or other heme-related metabolic disorders. If you're experiencing recurrent, unexplained symptoms—especially involving the nervous system or abdomen—this test may provide critical answers. Follow-up testing and early treatment can prevent serious complications.
What does it mean if your ALA/Creatinine Ratio result is too high?
What does it mean if your ALA/Creatinine Ratio is elevated?
An elevated ALA/Creatinine Ratio means your body is producing and excreting too much delta-aminolevulinic acid (ALA) in the urine. ALA is a natural substance your body uses to make heme, a vital component of red blood cells that helps carry oxygen.
Too much ALA in your urine can signal a problem in the heme production process, often due to a rare condition called porphyria or, in some cases, lead poisoning.
Possible causes of elevated ALA/Creatinine Ratio
-
Acute Hepatic Porphyrias
These are rare genetic disorders that affect how your body makes heme. When certain enzymes don’t work properly, substances like ALA build up and cause symptoms, especially during attacks. -
Lead Poisoning
Lead interferes with enzymes in the heme pathway, causing ALA to rise without a corresponding increase in other markers like PBG. This can affect both children and adults. -
Tyrosinemia Type I (in infants/children)
A rare inherited disorder that can also increase ALA levels, often showing up early in life with liver or kidney problems.
Symptoms to watch for
If you have high ALA levels, you may experience:
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Severe, unexplained abdominal pain
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Nausea or vomiting
-
Muscle weakness or numbness
-
Anxiety, confusion, or hallucinations
-
Seizures
-
Reddish or dark urine (especially during attacks)
These symptoms can come on suddenly and may be mistaken for other conditions, so testing is key.
What to do next if your levels are elevated
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Talk to your healthcare provider
Elevated ALA levels require follow-up. Your doctor may recommend:-
Repeat testing during a symptomatic episode
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A Porphobilinogen (PBG)/Creatinine Ratio
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Total urine porphyrins
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Blood lead level testing
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Genetic testing for porphyria, if indicated
-
-
Avoid potential triggers (if porphyria is suspected):
-
Fasting or crash diets
-
Alcohol
-
Certain medications (like sulfa drugs, barbiturates, or some antibiotics)
-
Hormonal changes (such as around menstruation)
-
-
Seek emergency care if symptoms worsen
Acute porphyria attacks can be life-threatening if untreated. If you’re experiencing severe symptoms—especially abdominal pain, confusion, or seizures—go to the emergency room and tell them you are being evaluated for porphyria.
Can elevated ALA/Creatinine Ratio go back to normal?
Yes. In some cases, levels return to normal once the underlying trigger is removed or the acute episode resolves. But if you have an underlying disorder like porphyria, lifelong management may be needed.
Bottom Line
An elevated ALA/Creatinine Ratio is a strong clue that something may be disrupting your body’s ability to make heme. Whether it’s a rare genetic disorder like acute porphyria or lead exposure, early diagnosis and action can help prevent serious complications. Don’t ignore this result—follow up with your provider to get the answers and care you need.
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