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Organic acids in urine

Also known as: Organic acids in urine
SKU: CH159

290.00

Study material: Urine
Response time (working day): 14
The test is done on an empty stomach: no
Home call service: Yes
Country: EU

General Information

Organic acids in urine

A laboratory test that allows for the assessment of metabolic processes in the body. The test is used to diagnose various inborn errors of metabolism, mitochondrial diseases, and organic acidosis. The test is especially important in children when evaluating developmental delays, seizures, recurrent vomiting, hypoglycemia, and metabolic disorders of unknown origin.

 

Organic acids are substances that are intermediate products of the metabolism of cellular organic components – amino acids, lipids, carbohydrates, nucleic acids, steroids.

Organic acidosis is a group of genetic metabolic diseases characterized by either the production of abnormal metabolites or the accumulation of normal metabolites in the body.

These disorders may manifest both in infancy and later in life as periodic episodes of metabolic decompensation and developmental delay.

Organic acids are excreted in the urine, so their determination in urine provides a more accurate answer than in plasma.

 

Research material – Spontaneous urination

 

The test examines the following organic acids in urine:

  • Ketoadipic acid
  • Ketoglutaric acid
  • 3-hydroxylauric acid
  • 3-Hydroxy sebacic acid
  • 4-Hydroxybutyric acid
  • Adipic acid
  • Ethylmalonic acid
  • Fumaric acid
  • Gamma-hydroxybutyric acid
  • Gamma-OH butyric acid
  • Glutaric acid
  • Homogentisic acid
  • Lactic acid
  • Malonic acid
  • Methylmalonic acid
  • Mevalonic acid
  • N-Acetyl Aspartic Acid
  • Octenedioic acid
  • Propionic acid
  • Pyroglutamic acid
  • Pyruvic acid
  • Sebacic acid
  • Suberic acid
  • Succinylacetone
  • Isovaleric acid

When should we take the test?

  • Genetic disorders of organic acid and amino acid metabolism, urea cycle defect, mitochondrial respiratory chain defect
  • The presence of symptoms that indicate hereditary metabolic disorders:
  • Neonatal sepsis-like illness
  • Unexplained episodes of metabolic decompensation: metabolic acidosis, hyperammonemia, hypoglycemia, ketosis
  • Neurological disorders: hypotonia, dystonia, seizures, lethargy, developmental delay
  • Unexplained liver dysfunction
  • Cardiomyopathy (at any age)
  • Myopathy, rhabdomyolysis (breakdown of muscle fibers)
  • Fatty liver, hepatocytolysis (breakdown of liver cells), hypertension
  • HELLP syndrome (complication during the third trimester of pregnancy)
  • Reye's syndrome
  • Organomegaly (enlargement of organs), coarse facial features

Possible interpretation of the results

Detection of pathological indicators of organic acids in urine is insufficient for the diagnostics of hereditary metabolic disorders. Additional diagnostic studies are necessary (e.g. molecular analysis, in vitro enzyme tests, etc.).

Reference values

In hereditary organic aciduria, especially in infants and young children, it is also necessary to study plasma amino acids, since the symptoms of some aminoacidemias are exactly identical.

Because metabolic disorders are difficult to detect, it is necessary to conduct research during periods of exacerbation of the disease.

 

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This medical information is not intended to be a universal treatment guide for all patients. The treatment process, including the type, volume, and frequency of diagnostic tests and therapeutic procedures, is determined by the physician individually — based on an assessment of the patient's condition and relevant medical indications. The decision is made in consultation with the patient. Before purchasing a test, please read the instructions for its preparation.
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