Cited by 106Lipid metabolism and disordersLiver Disease Diagnosis and TreatmentLipoproteins and Cardiovascular Health

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Hepatic and Cardiovascular Consequences of Familial Hypobetalipoproteinemia

Sankatsing, Raaj R., Fouchier, Sigrid W., de Haan, Stefan, Hutten, Barbara A., de Groot, Eric, Kastelein, John J.P., Stroes, Erik S.G.·2005·Arteriosclerosis, Thrombosis, and Vascular Biology

DOI 10.1161/01.atv.0000176191.64314.07

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Abstract

Objective— Individuals with familial hypobetalipoproteinemia (FHBL) have been reported to be prone to fatty liver disease (FLD). Conversely, the profound reduction of low-density lipoprotein (LDL) cholesterol in this disorder might decrease cardiovascular risk. In the present study, we assessed hepatic steatosis as well as noninvasive surrogate markers for cardiovascular disease (CVD) in subjects with FHBL and in matched controls. Methods and Results— Hepatic steatosis was assessed by abdominal ultrasonography. Carotid intima-media thickness (IMT) and distal common carotid arterial wall stiffness as surrogate markers for CVD risk were measured using high-resolution B-mode ultrasonography. Whereas transaminase levels were only modestly elevated, both prevalence (54% versus 29%; P =0.01) and severity of steatosis were significantly higher in FHBL individuals compared with controls. Despite similar IMT measurements, arterial stiffness was significantly lower in FHBL ( P =0.04) compared with controls. Additionally, the increase in arterial stiffness as seen in the presence of traditional risk factors was attenuated, suggesting that very low levels of apoB-containing lipoproteins can negate the adverse effects of other risk factors on the vasculature. Conclusions— FHBL is characterized by an increased prevalence and severity of fatty liver disease. The observed decreased level of arterial wall stiffness, most pronounced in the presence of nonlipid risk factors, is indicative of cardiovascular protection in these subjects.

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Authors: Sankatsing, R. R., Fouchier, S. W., de Haan, S., Hutten, B. A., de Groot, E., Kastelein, J. J. P., & Stroes, E. S. G.
Affiliations: Amsterdam UMC Location University of Amsterdam (NL)
ORCID: Fouchier, Sigrid W.
Hutten, Barbara A.
de Groot, Eric
Kastelein, John J.P.
Stroes, Erik S.G.
Venue: Arteriosclerosis, Thrombosis, and Vascular Biology
Publisher: Ovid Technologies (Wolters Kluwer Health)
Published: 2005-09
Volume: 25
Issue: 9
Pages: 1979-1984
ISSN: 1079-5642 (ISSN-L)
1524-4636
DOI: 10.1161/01.atv.0000176191.64314.07
OpenAlex: W2110975556
Cited by: 106
References: 56
Citations by year: 2026: 3 · 2025: 1 · 2024: 5 · 2023: 2 · 2022: 4 · 2021: 6 · 2020: 2 · 2019: 4 · 2018: 1 · 2017: 2 · 2016: 4 · 2015: 8 · 2014: 8 · 2013: 13 · 2012: 8
FWCI: 3.86
Type: journal article
Language: en
Open access: closed
Locations: Arteriosclerosis Thrombosis and Vascular Biology (publishedVersion)
PubMed (publishedVersion)
Pure Amsterdam UMC (publishedVersion)
Data Archiving and Networked Services (DANS) (submittedVersion)
Topics: Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Lipid metabolism and disorders
Health Sciences › Medicine › Hepatology › Liver Disease Diagnosis and Treatment
Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Lipoproteins and Cardiovascular Health
Keywords: familial hypobetalipoproteinemia, hepatic steatosis, cardiovascular risk, carotid intima-media thickness, arterial stiffness, LDL cholesterol, apolipoprotein B, transaminases, cardioprotection
MeSH terms: Adult, Apolipoproteins B, Carotid Artery Diseases, Fatty Liver, Female, Humans, Hypobetalipoproteinemias, Cholesterol, LDL, Male, Middle Aged, Risk Factors, Severity of Illness Index, Ultrasonography, Prevalence

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Hepatic and Cardiovascular Consequences of Familial Hypobetalipoproteinemia

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