title: Oxysterol changes along with cholesterol and vitamin D changes in adult phenylketonuric patients diagnosed by newborn mass-screening.
abstract: BACKGROUND
  Phenylketonuria (PKU) possibly leads to hypocholesterolemia and lowered vitamin D (VD) status. Metabolism of oxysterols linking with those of cholesterol and VD has never been examined in PKU.
  
  
  METHODS
  Blood oxysterols along with blood phenylalanine, lipids and VD were examined for 33 PKU adults aged 21-38 years and 20 age-matched healthy controls.
  
  
  RESULTS
  Total- and low-density cholesterols, and 25-hydroxy VD(3) were decreased significantly in the PKU group (cholesterols, 10% decrease; 25-hydroxy VD(3) 35% decrease vs. the control group). 24S-hydroxycholesterol (24S-OHC) eliminating brain cholesterol, and 27-OHC and 7α-hydroxycholesterol (7α-OHC) representing peripheral and hepatic cholesterol elimination, respectively, were significantly decreased in PKU group: 24S-OHC, 25% decrease, p<.01; 27-OHC and 7α-OHC, 35-40% decrease, p<.001. 7β-Hydroxycholesterol (7β-OHC) reflecting oxidative stress was increased significantly in PKU group (p<.05). 7α-OHC and 27-OHC levels in PKU group always showed similar values, regardless of other parameters while the 24S-OHC and 7β-OHC levels decreased and increased, respectively, showing significant correlations with phenylalanine level (p<.005). 27-OHC level showed a significant positive correlation with the 25-hydroxy VD(3) level in this group (p<.001).
  
  
  CONCLUSIONS
  Blood oxysterol changes predominate over blood cholesterol changes and influence on VD status in adult PKU patients.
authors.affiliation: Department of Pediatrics, Takarazuka City Hospital, Takarazuka, Japan. nagasa-hirono@k2.dion.ne.jp
authors.forename: Hironori
authors.initials: H
authors.lastname: Nagasaka
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Okano
authors.forename: Akihiko
authors.initials: A
authors.lastname: Kimura
authors.forename: Tatsuki
authors.initials: T
authors.lastname: Mizuochi
authors.forename: Yoshitami
authors.initials: Y
authors.lastname: Sanayama
authors.forename: Tomozumi
authors.initials: T
authors.lastname: Takatani
authors.forename: Saori
authors.initials: S
authors.lastname: Nakagawa
authors.forename: Eri
authors.initials: E
authors.lastname: Hasegawa
authors.forename: Ken-ichi
authors.initials: K
authors.lastname: Hirano
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Mochizuki
authors.forename: Toshihiro
authors.initials: T
authors.lastname: Ohura
authors.forename: Mika
authors.initials: M
authors.lastname: Ishige-Wada
authors.forename: Hiromi
authors.initials: H
authors.lastname: Usui
authors.forename: Tohru
authors.initials: T
authors.lastname: Yorifuji
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Tsukahara
authors.forename: Satoshi
authors.initials: S
authors.lastname: Hirayama
authors.forename: Akira
authors.initials: A
authors.lastname: Ohtake
authors.forename: Susumu
authors.initials: S
authors.lastname: Yamato
authors.forename: Takashi
authors.initials: T
authors.lastname: Miida
mesh_terms: D000328:Adult; D002784:Cholesterol / Q000097:blood*; D005260:Female; D006801:Humans; D007653:Ketocholesterols / Q000097:blood*; D008297:Male; D008403:Mass Screening; D010661:Phenylketonurias / Q000097:blood* / Q000175:diagnosis; D014807:Vitamin D / Q000097:blood*
chemical_list: D007653:Ketocholesterols; D014807:Vitamin D; D002784:Cholesterol