Changes of lipoproteins in phenylalanine hydroxylase-deficient children during the first year of life

Hironori Nagasaka, Hirokazu Tsukahara, Yoshiyuki Okano, Ken ichi Hirano, Toshihiro Sakurai, Shu Ping Hui, Toshihiro Ohura, Hiromi Usui, Tohru Yorifuji, Satoshi Hirayama, Akira Ohtake, Takashi Miida

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

Background: Influence of hyperphenylalaninemia on lipoproteins in early life remains unclear. Methods: We enrolled 24 phenylalanine hydroxylase (PAH)-deficient children who were classified into a phenylketonuria (PKU) group (n = 12) lacking PAH activity and a benign hyperphenylalaninemia (HPA) group (n = 12) having partial PAH activity, and their 11 non-affected siblings. We measured serum total-cholesterol, low-density lipoprotein (LDL)-cholesterol, and high-density lipoprotein (HDL)-cholesterol levels together with apolipoproteins for the first year of life, and compared them with those of 30 age-matched healthy controls. Results: The affected groups invariably had lower cholesterol levels than non-affected groups. At birth, HDL-cholesterol decrease was greatest and predominated over the LDL-cholesterol decrease: total cholesterol, 28/36% decrease to the control level in HPA/PKU; HDL-cholesterol, 33/51%; LDL-cholesterol, 20/28%. At 3. months, the opposite changes were observed: total cholesterol, 16/28%; HDL-cholesterol, 13/23%; LDL-cholesterol, 16/33%. At 12. months, LDL were still significantly lower in both groups (8/18%, p <.05 and .001), although HDL was significantly decreased only in the PKU group (15%, p <.05). Apolipoprotein A-I/A-II and B changed respectively in accordance with HDL-cholesterol and LDL-cholesterol changes. Despite similar phenylalanine levels, the PKU group invariably had lower cholesterol concentrations than the HPA group had. Conclusion: Irrespective of phenylalanine concentrations, lipoprotein synthesis in PAH-deficient children, particularly in PKU children, was suppressed in early life.

Original languageEnglish
Pages (from-to)1-4
Number of pages4
JournalClinica Chimica Acta
Volume433
DOIs
Publication statusPublished - Jun 10 2014

Fingerprint

Phenylalanine Hydroxylase
Phenylketonurias
LDL Cholesterol
HDL Cholesterol
Lipoproteins
Cholesterol
Phenylalanine
Apolipoproteins
Apolipoprotein A-I
Level control
HDL Lipoproteins
LDL Lipoproteins
Siblings
Parturition

Keywords

  • Cholesterol
  • High-density lipoprotein
  • Low-density lipoprotein
  • Phenylalanine hydroxylase
  • Phenylketonuria

ASJC Scopus subject areas

  • Biochemistry
  • Clinical Biochemistry
  • Biochemistry, medical
  • Medicine(all)

Cite this

Changes of lipoproteins in phenylalanine hydroxylase-deficient children during the first year of life. / Nagasaka, Hironori; Tsukahara, Hirokazu; Okano, Yoshiyuki; Hirano, Ken ichi; Sakurai, Toshihiro; Hui, Shu Ping; Ohura, Toshihiro; Usui, Hiromi; Yorifuji, Tohru; Hirayama, Satoshi; Ohtake, Akira; Miida, Takashi.

In: Clinica Chimica Acta, Vol. 433, 10.06.2014, p. 1-4.

Research output: Contribution to journalArticle

Nagasaka, H, Tsukahara, H, Okano, Y, Hirano, KI, Sakurai, T, Hui, SP, Ohura, T, Usui, H, Yorifuji, T, Hirayama, S, Ohtake, A & Miida, T 2014, 'Changes of lipoproteins in phenylalanine hydroxylase-deficient children during the first year of life', Clinica Chimica Acta, vol. 433, pp. 1-4. https://doi.org/10.1016/j.cca.2014.02.020
Nagasaka, Hironori ; Tsukahara, Hirokazu ; Okano, Yoshiyuki ; Hirano, Ken ichi ; Sakurai, Toshihiro ; Hui, Shu Ping ; Ohura, Toshihiro ; Usui, Hiromi ; Yorifuji, Tohru ; Hirayama, Satoshi ; Ohtake, Akira ; Miida, Takashi. / Changes of lipoproteins in phenylalanine hydroxylase-deficient children during the first year of life. In: Clinica Chimica Acta. 2014 ; Vol. 433. pp. 1-4.
@article{1aa364d714d047c389d6c2a9094c1143,
title = "Changes of lipoproteins in phenylalanine hydroxylase-deficient children during the first year of life",
abstract = "Background: Influence of hyperphenylalaninemia on lipoproteins in early life remains unclear. Methods: We enrolled 24 phenylalanine hydroxylase (PAH)-deficient children who were classified into a phenylketonuria (PKU) group (n = 12) lacking PAH activity and a benign hyperphenylalaninemia (HPA) group (n = 12) having partial PAH activity, and their 11 non-affected siblings. We measured serum total-cholesterol, low-density lipoprotein (LDL)-cholesterol, and high-density lipoprotein (HDL)-cholesterol levels together with apolipoproteins for the first year of life, and compared them with those of 30 age-matched healthy controls. Results: The affected groups invariably had lower cholesterol levels than non-affected groups. At birth, HDL-cholesterol decrease was greatest and predominated over the LDL-cholesterol decrease: total cholesterol, 28/36{\%} decrease to the control level in HPA/PKU; HDL-cholesterol, 33/51{\%}; LDL-cholesterol, 20/28{\%}. At 3. months, the opposite changes were observed: total cholesterol, 16/28{\%}; HDL-cholesterol, 13/23{\%}; LDL-cholesterol, 16/33{\%}. At 12. months, LDL were still significantly lower in both groups (8/18{\%}, p <.05 and .001), although HDL was significantly decreased only in the PKU group (15{\%}, p <.05). Apolipoprotein A-I/A-II and B changed respectively in accordance with HDL-cholesterol and LDL-cholesterol changes. Despite similar phenylalanine levels, the PKU group invariably had lower cholesterol concentrations than the HPA group had. Conclusion: Irrespective of phenylalanine concentrations, lipoprotein synthesis in PAH-deficient children, particularly in PKU children, was suppressed in early life.",
keywords = "Cholesterol, High-density lipoprotein, Low-density lipoprotein, Phenylalanine hydroxylase, Phenylketonuria",
author = "Hironori Nagasaka and Hirokazu Tsukahara and Yoshiyuki Okano and Hirano, {Ken ichi} and Toshihiro Sakurai and Hui, {Shu Ping} and Toshihiro Ohura and Hiromi Usui and Tohru Yorifuji and Satoshi Hirayama and Akira Ohtake and Takashi Miida",
year = "2014",
month = "6",
day = "10",
doi = "10.1016/j.cca.2014.02.020",
language = "English",
volume = "433",
pages = "1--4",
journal = "Clinica Chimica Acta",
issn = "0009-8981",
publisher = "Elsevier",

}

TY - JOUR

T1 - Changes of lipoproteins in phenylalanine hydroxylase-deficient children during the first year of life

AU - Nagasaka, Hironori

AU - Tsukahara, Hirokazu

AU - Okano, Yoshiyuki

AU - Hirano, Ken ichi

AU - Sakurai, Toshihiro

AU - Hui, Shu Ping

AU - Ohura, Toshihiro

AU - Usui, Hiromi

AU - Yorifuji, Tohru

AU - Hirayama, Satoshi

AU - Ohtake, Akira

AU - Miida, Takashi

PY - 2014/6/10

Y1 - 2014/6/10

N2 - Background: Influence of hyperphenylalaninemia on lipoproteins in early life remains unclear. Methods: We enrolled 24 phenylalanine hydroxylase (PAH)-deficient children who were classified into a phenylketonuria (PKU) group (n = 12) lacking PAH activity and a benign hyperphenylalaninemia (HPA) group (n = 12) having partial PAH activity, and their 11 non-affected siblings. We measured serum total-cholesterol, low-density lipoprotein (LDL)-cholesterol, and high-density lipoprotein (HDL)-cholesterol levels together with apolipoproteins for the first year of life, and compared them with those of 30 age-matched healthy controls. Results: The affected groups invariably had lower cholesterol levels than non-affected groups. At birth, HDL-cholesterol decrease was greatest and predominated over the LDL-cholesterol decrease: total cholesterol, 28/36% decrease to the control level in HPA/PKU; HDL-cholesterol, 33/51%; LDL-cholesterol, 20/28%. At 3. months, the opposite changes were observed: total cholesterol, 16/28%; HDL-cholesterol, 13/23%; LDL-cholesterol, 16/33%. At 12. months, LDL were still significantly lower in both groups (8/18%, p <.05 and .001), although HDL was significantly decreased only in the PKU group (15%, p <.05). Apolipoprotein A-I/A-II and B changed respectively in accordance with HDL-cholesterol and LDL-cholesterol changes. Despite similar phenylalanine levels, the PKU group invariably had lower cholesterol concentrations than the HPA group had. Conclusion: Irrespective of phenylalanine concentrations, lipoprotein synthesis in PAH-deficient children, particularly in PKU children, was suppressed in early life.

AB - Background: Influence of hyperphenylalaninemia on lipoproteins in early life remains unclear. Methods: We enrolled 24 phenylalanine hydroxylase (PAH)-deficient children who were classified into a phenylketonuria (PKU) group (n = 12) lacking PAH activity and a benign hyperphenylalaninemia (HPA) group (n = 12) having partial PAH activity, and their 11 non-affected siblings. We measured serum total-cholesterol, low-density lipoprotein (LDL)-cholesterol, and high-density lipoprotein (HDL)-cholesterol levels together with apolipoproteins for the first year of life, and compared them with those of 30 age-matched healthy controls. Results: The affected groups invariably had lower cholesterol levels than non-affected groups. At birth, HDL-cholesterol decrease was greatest and predominated over the LDL-cholesterol decrease: total cholesterol, 28/36% decrease to the control level in HPA/PKU; HDL-cholesterol, 33/51%; LDL-cholesterol, 20/28%. At 3. months, the opposite changes were observed: total cholesterol, 16/28%; HDL-cholesterol, 13/23%; LDL-cholesterol, 16/33%. At 12. months, LDL were still significantly lower in both groups (8/18%, p <.05 and .001), although HDL was significantly decreased only in the PKU group (15%, p <.05). Apolipoprotein A-I/A-II and B changed respectively in accordance with HDL-cholesterol and LDL-cholesterol changes. Despite similar phenylalanine levels, the PKU group invariably had lower cholesterol concentrations than the HPA group had. Conclusion: Irrespective of phenylalanine concentrations, lipoprotein synthesis in PAH-deficient children, particularly in PKU children, was suppressed in early life.

KW - Cholesterol

KW - High-density lipoprotein

KW - Low-density lipoprotein

KW - Phenylalanine hydroxylase

KW - Phenylketonuria

UR - http://www.scopus.com/inward/record.url?scp=84896124849&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84896124849&partnerID=8YFLogxK

U2 - 10.1016/j.cca.2014.02.020

DO - 10.1016/j.cca.2014.02.020

M3 - Article

VL - 433

SP - 1

EP - 4

JO - Clinica Chimica Acta

JF - Clinica Chimica Acta

SN - 0009-8981

ER -