Document Detail

Evaluation of risk for atherosclerosis in Alagille syndrome and progressive familial intrahepatic cholestasis: two congenital cholestatic diseases with different lipoprotein metabolisms.
MedLine Citation:
PMID:  15756213     Owner:  NLM     Status:  MEDLINE    
OBJECTIVES: To evaluate the risk for atherosclerosis in Alagille syndrome (AGS) and progressive familial intrahepatic cholestasis (PFIC) on the basis of lipoprotein metabolism and by ultrasonography. STUDY DESIGN: Five patients with AGS and 5 with PFIC, ages 3 to 4 years, were enrolled. Intimal-medial thickness and wall stiffness of the common carotid artery were examined by ultrasonography. Serum levels of lipids and lipoproteins were determined. Further, the chemical composition of LDL and its ability to transform macrophages into foam cells were determined. RESULTS: Intimal-medial thickness and wall stiffness were increased in patients with PFIC but not in patients with AGS. Total cholesterol, LDL cholesterol, HDL cholesterol, and lipoprotein X were remarkably increased in patients with AGS, whereas in patients with PFIC, an increase in triglyceride and a decrease in HDL cholesterol were the prominent findings. However, despite the normal LDL cholesterol level, oxidized LDL level was strikingly high in patients with PFIC. LDLs from patients with PFIC had high TG contents and exhibited high abilities to transform macrophages into foam cells. CONCLUSIONS: These findings suggest that patients with PFIC are at high risk for cardiovascular disorders involving atherosclerosis.
Hironori Nagasaka; Tohru Yorifuji; Hiroto Egawa; Hidekatsu Yanai; Tomoo Fujisawa; Kiyotaka Kosugiyama; Akira Matsui; Makoto Hasegawa; Tomoo Okada; Masaki Takayanagi; Hitoshi Chiba; Kunihiko Kobayashi
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The Journal of pediatrics     Volume:  146     ISSN:  0022-3476     ISO Abbreviation:  J. Pediatr.     Publication Date:  2005 Mar 
Date Detail:
Created Date:  2005-03-09     Completed Date:  2005-04-15     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0375410     Medline TA:  J Pediatr     Country:  United States    
Other Details:
Languages:  eng     Pagination:  329-35     Citation Subset:  AIM; IM    
Division of Metabolism, Chiba Children's Hospital, Japan.
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MeSH Terms
Alagille Syndrome / epidemiology*
Arteriosclerosis / epidemiology*,  ultrasonography
Carotid Artery, Common / ultrasonography
Child, Preschool
Cholestasis, Intrahepatic / congenital*,  epidemiology
Cholesterol, LDL / metabolism
Foam Cells / metabolism
Lipid Metabolism
Lipoproteins / metabolism*
Risk Assessment
Reg. No./Substance:
0/Cholesterol, LDL; 0/Lipoproteins
Comment In:
J Pediatr. 2005 Mar;146(3):306-7   [PMID:  15756207 ]

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