Long-chain polyunsaturated fatty acid status in children, adolescents and adults with phenylketonuria.
Patients with phenylketonuria have been reported to be deficient in long-chain polyunsaturated fatty acids (LCPUFAs). It has been postulated that good compliance with the dietary regimen negatively influences LCPUFA status.



In 36 patients with phenylketonuria and 18 age-matched healthy control subjects LCPUFA-levels in plasma phospholipids and cholesteryl esters, erythrocyte phosphatidylcholine and phosphatidylethanolamine were evaluated.



Eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) levels did not differ significantly between patients and control subjects in plasma and erythrocyte fractions. There was a significant negative correlation between SDS (standard deviation) scores of DHA-levels in erythrocyte parameters from the respective age-matched control group and patients' concurrent and long-term phenylalanine levels for erythrocyte phosphatidylethanolamine and erythrocyte phosphatidylcholine. Patients with lower (higher) phenylalanine levels had positive (negative) DHA-SDS.



In contrast to previous reports we did not find lower LCPUFA-levels in patients with phenylketonuria compared to age-matched healthy control subjects. Good dietary control was associated with better LCPUFA status.
Centre for Paediatric and Adolescent Medicine, Division for Neuropaediatrics and Metabolic Medicine, Im Neuenheimer Feld 430, 69120 Heidelberg, Germany. Electronic address: gwendolyn.gramer@med.uni-heidelberg.de.
Gwendolyn
G
Gramer
Centre for Paediatric and Adolescent Medicine, Division for Neuropaediatrics and Metabolic Medicine, Im Neuenheimer Feld 430, 69120 Heidelberg, Germany. Electronic address: gisela.haege@med.uni-heidelberg.de.
Gisela
G
Haege
Centre for Paediatric and Adolescent Medicine, Division for Neuropaediatrics and Metabolic Medicine, Im Neuenheimer Feld 430, 69120 Heidelberg, Germany. Electronic address: claus-dieter.langhans@med.uni-heidelberg.de.
Claus-Dieter
CD
Langhans
Centre for Paediatric and Adolescent Medicine, Division for Neuropaediatrics and Metabolic Medicine, Im Neuenheimer Feld 430, 69120 Heidelberg, Germany. Electronic address: vera.schuhmann@med.uni-heidelberg.de.
Vera
V
Schuhmann
Centre for Paediatric and Adolescent Medicine, Division for Neuropaediatrics and Metabolic Medicine, Im Neuenheimer Feld 430, 69120 Heidelberg, Germany. Electronic address: peter.burgard@med.uni-heidelberg.de.
Peter
P
Burgard
Centre for Paediatric and Adolescent Medicine, Division for Neuropaediatrics and Metabolic Medicine, Im Neuenheimer Feld 430, 69120 Heidelberg, Germany. Electronic address: georg.hoffmann@med.uni-heidelberg.de.
Georg F
GF
Hoffmann
D000293:Adolescent; D000328:Adult; D002648:Child; D002788:Cholesterol Esters / Q000097:blood; D004281:Docosahexaenoic Acids / Q000097:blood; D015118:Eicosapentaenoic Acid / Q000097:blood; D004912:Erythrocytes / Q000378:metabolism*; D005231:Fatty Acids, Unsaturated / Q000097:blood*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009752:Nutritional Status; D010661:Phenylketonurias / Q000178:diet therapy* / Q000378:metabolism*; D010713:Phosphatidylcholines / Q000097:blood; D010714:Phosphatidylethanolamines / Q000097:blood; D010743:Phospholipids / Q000097:blood; D055815:Young Adult
D002788:Cholesterol Esters; D005231:Fatty Acids, Unsaturated; D010713:Phosphatidylcholines; D010714:Phosphatidylethanolamines; D010743:Phospholipids; D004281:Docosahexaenoic Acids; C483858:phosphatidylethanolamine; D015118:Eicosapentaenoic Acid
Docosahexaenoic acid; Long-chain polyunsaturated fatty acids; Phenylketonuria