Document Detail

Changes of lipoproteins in phenylalanine hydroxylase-deficient children during the first year of life.
MedLine Citation:
PMID:  24607329     Owner:  NLM     Status:  Publisher    
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 3months, the opposite changes were observed: total cholesterol, 16/28%; HDL-cholesterol, 13/23%; LDL-cholesterol, 16/33%. At 12months, 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.
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
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2014-3-4
Journal Detail:
Title:  Clinica chimica acta; international journal of clinical chemistry     Volume:  -     ISSN:  1873-3492     ISO Abbreviation:  Clin. Chim. Acta     Publication Date:  2014 Mar 
Date Detail:
Created Date:  2014-3-10     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  1302422     Medline TA:  Clin Chim Acta     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2014. Published by Elsevier B.V.
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