Highlights
- •Almost 10% of children with NAFLD have extremely low LDL-C levels.
- •Patients with low LDL-C levels have more severe steatosis.
- •These patients are younger and less likely to have metabolic comorbidities.
Background
Low-density lipoprotein cholesterol (LDL-C) levels below 50 mg/dL may suggest familial
hypobetalipoproteinemia, particularly in patients with hepatic steatosis. The prevalence
of hypobetalipoproteinemia in cohorts with nonalcoholic fatty liver disease (NAFLD)
is not known, and it is not clear whether the severity of liver disease of these patients
is different. The objective of this study was to address these questions in a large
pediatric NAFLD cohort.
Methods
Retrospective study of children followed at the Steatohepatitis Center of a tertiary
care center from August 2010 to October 2017. Patients with secondary causes of hepatic
steatosis and those on statins were excluded.
Results
Of the 740 patients included, 58 (8%) had hypobetalipoproteinemia. These patients
were younger (P = .04), had a lower body mass index (P < .01) and waist circumference (P = .01), and were less likely to be on metformin (P = .01). In spite of that, serum aminotransferase levels were not different between
those with low, normal, and high LDL-C levels. Of the 222 patients who had both lipid
and histology data available, the steatosis score was higher in those with low LDL-C
compared to those with normal or elevated LDL-C, a result that trended toward significance
(P = .06). The severity of inflammation and fibrosis did not differ between the groups.
When all patients with hypertriglyceridemia were excluded, steatosis severity was
higher in those with low LDL-C (P = .04).
Conclusion
Hypobetalipoproteinemia is common among patients with NAFLD and is associated with
similar liver disease severity in spite of a leaner phenotype and a more favorable
metabolic profile.
Keywords
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Article info
Publication history
Published online: February 14, 2019
Accepted:
February 7,
2019
Received:
October 3,
2018
Footnotes
Conflict of interest: The authors have no conflicts of interest to disclose. Dr Mouzaki is acting as the submission's guarantor.
Lay summary: Approximately 1-in-10 patients with fatty liver disease have extremely low levels of LDL cholesterol (LDL-C), otherwise known as the bad cholesterol. This protects them from a heart disease standpoint, but it is not known if their liver disease is any different from that of patients with normal or high LDL-C levels. Here, we show that the liver disease of patients with extremely low LDL-C levels is as serious as that of patients with normal or high LDL-C levels, even though they tend to be younger and thinner.
Identification
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