This serum test is a comprehensive profile that provides information regarding mitochondrial and peroxisomal fatty acid metabolism, and the patient's nutritional status

Monitoring patients undergoing diet therapy for mitochondrial or peroxisomal disorders (possibly inducing essential fatty acid deficiency in response to restricted fat intake)

Monitoring treatment of essential fatty acid deficiency

Monitoring the response to provocative tests (fasting tests, loading tests)

Octanoic Acid, C8:0

<1 year: 7-63 nmol/mL

1-17 years: 9-41 nmol/mL

> or =18 years: 8-47 nmol/mL

 

Decenoic Acid, C10:1

<1 year: 0.8-4.8 nmol/mL

1-17 years: 1.6-6.6 nmol/mL

> or =18 years: 1.8-5.0 nmol/mL

 

Decanoic Acid, C10:0

<1 year: 2-62 nmol/mL

1-17 years: 3-25 nmol/mL

> or =18 years: 2-18 nmol/mL

 

Lauroleic Acid, C12:1

<1 year: 0.6-4.8 nmol/mL

1-17 years: 1.3-5.8 nmol/mL

> or =18 years: 1.4-6.6 nmol/mL

 

Lauric Acid, C12:0

<1 year: 6-190 nmol/mL

1-17 years: 5-80 nmol/mL

> or =18 years: 6-90 nmol/mL

 

Tetradecadienoic Acid, C14:2

<1 year: 0.3-6.5 nmol/mL

1-17 years: 0.2-5.8 nmol/mL

> or =18 years: 0.8-5.0 nmol/mL

 

Myristoleic Acid, C14:1

<1 year: 1-46 nmol/mL

1-17 years: 1-31 nmol/mL

> or =18 years: 3-64 nmol/mL

 

Myristic Acid, C14:0

<1 year: 30-320 nmol/mL

1-17 years: 40-290 nmol/mL

> or =18 years: 30-450 nmol/mL

 

Hexadecadienoic Acid, C16:2

<1 year: 4-27 nmol/mL

1-17 years: 3-29 nmol/mL

> or =18 years: 10-48 nmol/mL

 

Hexadecenoic Acid, C16:1w9

<1 year: 21-69 nmol/mL

1-17 years: 24-82 nmol/mL

> or =18 years: 25-105 nmol/mL

 

Palmitoleic Acid, C16:1w7

<1 year: 20-1,020 nmol/mL

1-17 years: 100-670 nmol/mL

> or =18 years: 110-1,130 nmol/mL

 

Palmitic Acid, C16:0

<1 year: 720-3,120 nmol/mL

1-17 years: 960-3,460 nmol/mL

> or =18 years: 1,480-3,730 nmol/mL

 

Gamma-Linolenic Acid, C18:3w6

<1 year: 6-110 nmol/mL

1-17 years: 9-130 nmol/mL

> or =18 years: 16-150 nmol/mL

 

Alpha-Linolenic Acid, C18:3w3

<1 year: 10-190 nmol/mL

1-17 years: 20-120 nmol/mL

> or =18 years: 50-130 nmol/mL

 

Linoleic Acid, C18:2w6

< or =31 days: 350-2,660 nmol/mL

32 days-11 months: 1,000-3,300 nmol/mL

1-17 years: 1,600-3,500 nmol/mL

> or =18 years: 2,270-3,850 nmol/mL

 

Oleic Acid, C18:1w9

<1 year: 250-3,500 nmol/mL

1-17 years: 350-3,500 nmol/mL

> or =18 years: 650-3,500 nmol/mL

 

Vaccenic Acid, C18:1w7

<1 year: 140-720 nmol/mL

1-17 years: 320-900 nmol/mL

> or =18 years: 280-740 nmol/mL

 

Stearic Acid, C18:0

<1 year: 270-1,140 nmol/mL

1-17 years: 280-1,170 nmol/mL

> or =18 years: 590-1,170 nmol/mL

 

EPA, C20:5w3

<1 year: 2-60 nmol/mL

1-17 years: 8-90 nmol/mL

> or =18 years: 14-100 nmol/mL

 

Arachidonic Acid, C20:4w6

<1 year: 110-1,110 nmol/mL

1-17 years: 350-1,030 nmol/mL

> or =18 years: 520-1,490 nmol/mL

 

Mead Acid, C20:3w9

< or =31 days: 8-60 nmol/mL

32 days-11 months: 3-24 nmol/mL

> or =1 year: 7-30 nmol/mL

 

Homo-Gamma-Linolenic Acid, C20:3w6

<1 year: 30-170 nmol/mL

1-17 years: 60-220 nmol/mL

> or =18 years: 50-250 nmol/mL

 

Arachidic Acid, C20:0

<1 year: 30-120 nmol/mL

1-17 years: 30-90 nmol/mL

> or =18 years: 50-90 nmol/mL

 

DHA, C22:6w3

<1 year: 10-220 nmol/mL

1-17 years: 30-160 nmol/mL

> or =18 years: 30-250 nmol/mL

 

DPA, C22:5w6

<1 year: 3-70 nmol/mL

1-17 years: 10-50 nmol/mL

> or =18 years: 10-70 nmol/mL

 

DPA, C22:5w3

<1 year: 6-110 nmol/mL

1-17 years: 30-270 nmol/mL

> or =18 years: 20-210 nmol/mL

 

DTA, C22:4w6

<1 year: 2-50 nmol/mL

1-17 years: 10-40 nmol/mL

> or =18 years: 10-80 nmol/mL

 

Docosenoic Acid, C22:1

<1 year: 2-20 nmol/mL

> or =1 year: 4-13 nmol/mL

 

Docosanoic Acid, C22:0

0.0-96.3 nmol/mL

 

Nervonic Acid, C24:1

<1 year: 30-150 nmol/mL

1-17 years: 50-130 nmol/mL

> or =18 years: 60-100 nmol/mL

 

Tetracosanoic Acid, C24:0

0.0-91.4 nmol/mL

 

Hexacosenoic Acid, C26:1

<1 year: 0.2-2.1 nmol/mL

> or =1 year: 0.3-0.7 nmol/mL

 

Hexacosanoic Acid, C26:0

0.00-1.30 nmol/mL

 

Pristanic Acid, C15:0(CH3)4

< or =4 months: 0.00-0.60 nmol/mL

5-8 months: 0.00-0.84 nmol/mL

9-12 months: 0.00-0.77 nmol/mL

13-23 months: 0.00-1.47 nmol/mL

> or =2 years: 0.00-2.98 nmol/mL

 

Phytanic Acid, C16:0(CH3)4

< or =4 months: 0.00-5.28 nmol/mL

5-8 months: 0.00-5.70 nmol/mL

9-12 months: 0.00-4.40 nmol/mL

13-23 months: 0.00-8.62 nmol/mL

> or =2 years: 0.00-9.88 nmol/mL

 

Triene/Tetraene Ratio

< or =31 days: 0.017-0.083

32 days-17 years: 0.013-0.050

> or =18 years: 0.010-0.038

 

Total Saturated Acid

<1 year: 1.2-4.6 mmol/L

1-17 years: 1.4-4.9 mmol/L

> or =18 years: 2.5-5.5 mmol/L

 

Total Monounsaturated Acid

<1 year: 0.3-4.6 mmol/L

1-17 years: 0.5-4.4 mmol/L

> or =18 years: 1.3-5.8 mmol/L

 

Total Polyunsaturated Acid

<1 year: 1.1-4.9 mmol/L

1-17 years: 1.7-5.3 mmol/L

> or =18 years: 3.2-5.8 mmol/L

 

Total w3

<1 year: 0.0-0.4 mmol/L

1-17 years: 0.1-0.5 mmol/L

> or =18 years: 0.2-0.5 mmol/L

 

Total w6

<1 year: 0.9-4.4 mmol/L

1-17 years: 1.6-4.7 mmol/L

> or =18 years: 3.0-5.4 mmol/L

 

Total Fatty Acids

<1 year: 3.3-14.0 mmol/L

1-17 years: 4.4-14.3 mmol/L

> or =18 years: 7.3-16.8 mmol/L

An increased triene:tetraene ratio is consistent with essential fatty acid deficiency.

 

Fatty acid oxidation disorders are recognized on the basis of disease-specific patterns that are correlated to the results of other investigations in plasma (carnitine, acylcarnitines) and urine (organic acids, acylglycines).

 

Increased concentrations of serum very long-chain fatty acids (VLCFA) C24:0 and C26:0 are seen in peroxisomal disorders, X-linked adrenoleukodystrophy, adrenomyeloneuropathy, and Zellweger syndrome (cerebrohepatorenal syndrome).

 

Increased concentrations of serum phytanic acid (along with normal pristanic acid concentrations) are seen in the Refsum disease (phytanase deficiency). Serum phytanic acid concentration also may be increased in other peroxisomal disorders and, when combined with the VLCFA, pristanoic acid and pipecolic acid allow differential diagnosis of peroxisomal disorders.