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 language | English |
|---|---|
| Pages (from-to) | 1-4 |
| Number of pages | 4 |
| Journal | Clinica Chimica Acta |
| Volume | 433 |
| DOIs | |
| Publication status | Published - Jun 10 2014 |
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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 journal › Article
}
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
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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 -