Abstract | OBJECTIVE: PATIENTS AND METHODS: RESULTS:
Hypercholesterolemia and lipoprotein X accumulation were never observed throughout this study. When the cholestasis was severe, triglyceride-rich low-density lipoprotein ( LDL) accounted for most of the plasma lipoproteins whereas HDL was hardly detectable. Concurrently, activities of all regulators were decreased, together with decreases of the serum parameter for liver protein synthesis. In particular, suppressions of LCAT and HTGL activities were severe and greatly contributed to the appearance of triglyceride-rich LDL. As the cholestasis improved, this LDL gradually transformed into normal LDL with the recoveries of LCAT and HTGL activities. The activities of all regulators for the last 1 to 2 months were normal but HDL remained depleted. His liver showed low FXR expression compared with control livers. CONCLUSIONS: The present study showed an appearance of triglyceride-rich LDL due to suppressions of LCAT and HTGL activities and a depletion of HDL that is not able to be explained by lipoprotein regulators or FXR in our patient.
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Authors | Hironori Nagasaka, Hitoshi Chiba, Shu-Ping Hui, Hajime Takikawa, Takashi Miida, Masaki Takayanagi, Tohru Yorifuji, Makoto Hasegawa, Akemi Ota, Ken-ichi Hirano, Hideaki Kikuchi, Hirokazu Tsukahara, Kunihiko Kobayashi |
Journal | Journal of pediatric gastroenterology and nutrition
(J Pediatr Gastroenterol Nutr)
Vol. 45
Issue 1
Pg. 96-105
(Jul 2007)
ISSN: 1536-4801 [Electronic] United States |
PMID | 17592371
(Publication Type: Case Reports, Journal Article)
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Chemical References |
- Apolipoproteins E
- Cholesterol, HDL
- Lipoproteins, HDL
- Lipoproteins, LDL
- Triglycerides
- Adenosine Triphosphatases
- ATP8B1 protein, human
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Topics |
- Adenosine Triphosphatases
(deficiency)
- Adult
- Apolipoproteins E
(blood)
- Cholestasis, Intrahepatic
(blood, etiology)
- Cholesterol, HDL
(blood)
- Humans
- Hyperbilirubinemia
(etiology)
- Japan
- Lipoproteins, HDL
(blood, chemistry)
- Lipoproteins, LDL
(blood, chemistry)
- Liver
(metabolism)
- Male
- Pancreatitis
(blood, etiology)
- Protein Biosynthesis
- Recurrence
- Triglycerides
(blood)
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