Lp8 is potentially associated with partial lecithin:cholesterol acyltransferase deficiency in a patient with primary biliary cirrhosis.

Abstract

BACKGROUND:

The significance of Lp8, that is, abnormal lipoprotein(s) detected in fraction 8 by combined high-performance liquid chromatography/gel filtration column in patients with familial lecithin:cholesterol acyltransferase (LCAT) syndrome, in relation to the severity of LCAT deficiency has not been analyzed.

OBJECTIVE:

We have studied Lp8 in a patient with primary biliary cirrhosis.

METHODS:

Plasma lipoproteins were analyzed using high-performance liquid chromatography/gel filtration column in the course of treatment of a 47-year-old female patient with primary biliary cirrhosis.

RESULTS:

Electrophoretic lipoprotein analyses showed massive accumulation of abnormal ß- and preß-lipoproteins with a minor lipoprotein fraction at a position near the cathode corresponding to Lp-X, on day A (status: hypercholesterolemia, LCAT activity undetectable). Chromatographic lipoprotein subfraction analysis revealed free cholesterol- and phospholipid-rich lipoproteins in fractions 1-6, corresponding to chylomicrons and very low-density lipoprotein, and phospholipid- and triglyceride-rich lipoproteins with increased free cholesterol, that is, Lp8, in fractions 7-9 (corresponding to low-density lipoprotein). On day B, after additional treatment for 7 months (status: almost normolipidemia, decreased LCAT activity), although the abnormal lipoprotein and the lipoproteins in fractions 1-6, were drastically decreased, the presence of Lp8 persisted.

CONCLUSIONS:

Lp8 likely is a minor abnormal lipoprotein fraction in patients with mildly decreased secondary LCAT activity, as well as with severely reduced primary LCAT activity.

• Authors: Toshiyuki Watanabe, Toshirou Nema, Nobuyuki Hiruta, Takeyoshi Murano, Wolfgang J Schneider, Hideaki Bujo
• Journal: Journal of clinical lipidology (J Clin Lipidol) 2018 Sep - Oct Vol. 12 Issue 5 Pg. 1157-1163 ISSN: 1933-2874 [Print] United States
• PMID: 30055975 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2018 National Lipid Association. Published by Elsevier Inc. All rights reserved.

Chemical References

• Lipoproteins

Topics

• Dyslipidemias (complications)
• Female
• Humans
• Lecithin Cholesterol Acyltransferase Deficiency (complications, metabolism)
• Lipoproteins (metabolism)
• Liver Cirrhosis, Biliary (complications, metabolism)
• Middle Aged