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HHH Syndrome Hyperornithinemia - Hyperammonemia - Homocitrullinuria Syndrome Test

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HHH Syndrome Hyperornithinemia - Hyperammonemia - Homocitrullinuria Syndrome Test

Short Name: HHH Syndrome Test

Also known as: HHH Syndrome, Hyperornithinemia-Hyperammonemia-Homocitrullinuria Syndrome

HHH Syndrome Hyperornithinemia - Hyperammonemia - Homocitrullinuria Syndrome Test test available at DNA Labs India for ₹9,500. Uses LC-MS/MS, GC-MS on Random Urine samples. Results in Reports are typically available within 5 days after sample collection.. Free home collection in 300+ cities across India.

Genetic Test🏠 Home Collection

🩺 Medically Reviewed By

Overview

To diagnose HHH Syndrome by detecting elevated levels of ornithine in blood, ammonia in blood, and homocitrulline in urine, aiding in early intervention and management.

Test Code
749
Price
₹9,500
Sample Type
Random Urine
Result Time
Reports are typically available within 5 days after sample collection.
Fasting Required
No
Method
LC-MS/MS, GC-MS
Step 1

Sample Collection

Provide brief clinical history, including symptoms and family history.

Method: Urine collection

Step 2

Laboratory Analysis

Collect 15 mL of random urine in a sterile screw-capped container without preservatives.

Step 3

Report Delivery

Ship the sample refrigerated or frozen to maintain stability.

Timeline: Reports are typically available within 5 days after sample collection.

Patient Instructions

1
Before the Test:No specific preparation required, but provide clinical history.
2
During the Test:Simple urine collection process, non-invasive.
3
After the Test:Sample sent to lab; results available in 5 days.

About This Test

Who Should Get This Test

To diagnose HHH Syndrome by detecting elevated levels of ornithine in blood, ammonia in blood, and homocitrulline in urine, aiding in early intervention and management.

How to Prepare

  • Collect 15 mL (minimum 10 mL) of random urine in a sterile screw-capped container.
  • No preservative is required.
  • Ship the sample refrigerated or frozen.
  • Provide brief clinical history with the sample.

Doctor's Notes

Reviewed by — MBBS, MD (Medical Genetics) · Reg. No. 8532

"Early diagnosis through genetic testing and consultation with a genetic counselor is crucial for managing HHH Syndrome and improving patient outcomes."

Last medically reviewed: September 7, 2026

Test Parameters & Specifications

Sample TypeRandom Urine
Sample Volume15 mL (10 mL min.)
ContainerSterile screw capped container
Collection MethodUrine collection

Sample Stability

Room Temperature6 hours
Refrigerator72 hours
Frozen4 weeks
Sample Rejection Criteria:
  • Insufficient sample volume
  • Improper container or preservative used
  • Sample not shipped under required conditions

Understanding Your Results

Results indicate levels of ornithine, ammonia, and homocitrulline; elevated levels may suggest HHH Syndrome, requiring further clinical correlation.
📊

Ornithine

📊

Ammonia

📊

Homocitrulline

⚠️ When to Consult a Doctor:

Consult a doctor if you experience symptoms like seizures, developmental delays, or if test results show elevated levels of ornithine, ammonia, or homocitrulline.

Limitations

  • False negatives possible if sample is not collected or stored correctly
  • Genetic confirmation may be needed for definitive diagnosis
  • Test does not differentiate between severity levels of HHH Syndrome

Risks & Considerations

  • No significant risks as it involves urine collection, which is non-invasive.

Interfering Factors

  • Diet high in protein
  • Medications affecting amino acid metabolism
  • Improper sample collection or storage

Compare With Similar Tests

TestHHH Syndrome Hyperornithinemia - Hyperammonemia - Homocitrullinuria Syndrome TestAmino Acid Profile TestOrganic Acid TestGenetic Panel for Metabolic DisordersAmmonia Test
ComparisonHHH Syndrome Hyperornithinemia - Hyperammonemia - Homocitrullinuria Syndrome Test

Frequently Asked Questions

What is HHH Syndrome?
HHH Syndrome is a rare genetic disorder affecting amino acid metabolism, leading to high levels of ornithine, ammonia, and homocitrulline.
What are the common symptoms of HHH Syndrome?
Symptoms include hyperammonemia, seizures, mental retardation, developmental delays, and in severe cases, coma.
How is HHH Syndrome diagnosed?
Diagnosis involves blood and urine tests to measure ornithine, ammonia, and homocitrulline levels, often confirmed with genetic testing.
What does the HHH Syndrome test involve?
The test requires a urine sample to detect homocitrulline and may include blood tests for ornithine and ammonia.
Is fasting required for the HHH Syndrome test?
No, fasting is not required, but a brief clinical history should be provided.
How much does the HHH Syndrome test cost in India?
The test costs INR 9500 at DNA Labs India, with home collection available.
Is home sample collection available for this test?
Yes, DNA Labs India offers free home sample collection across multiple cities in India.
How long does it take to get results?
Results are typically available within 5 days after sample collection.
What should I do if the test results are positive?
Consult a healthcare professional or genetic counselor for further evaluation and management.
Is the HHH Syndrome test covered by insurance?
Coverage depends on your insurance plan; check with your provider for details.
Can children undergo this test?
Yes, the test is suitable for all ages, including pediatric patients.
How accurate is the HHH Syndrome test?
The test uses advanced methods like LC-MS/MS and GC-MS for high accuracy, but genetic confirmation may be recommended.
Worried about the process? Our certified phlebotomists collect thousands of samples every month across India. The process takes under 5 minutes and is virtually painless. Questions? Message us on WhatsApp — we're here 7 days a week.

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Stored in India: All patient records are stored on servers located in India. No data is transferred outside the country.

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