A case of severe acquired hypertriglyceridemia in a 7-year-old girl

Lilley, Jessica S.; Linton, MacRae F.; Kelley, Jennifer C.; Graham, T. Brent; Fazio, Sergio; Tavori, Hagai

doi:10.1016/j.jacl.2017.08.003

Cited by 7 publications

(2 citation statements)

References 13 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In accordance with the above, previous mechanistic work links disruptions in triglyceride metabolism (and thus subsequent atherosclerosis) to mutations in certain genes such as APOA5, APOC2, GPIHBP1, or LMF1, as well as autoimmune antibodies to lipoprotein lipase. Lipase is the enzyme that catalyses the hydrolysis of the triacylglycerol component of circulating chylomicrons and vLDL, which supplies non-esterified fatty acids and 2-monoacylglycerol for tissue utilisation [ 22 ].…”

Section: Introductionmentioning

confidence: 99%

Impact of Circulating Triglycerides Concentration on Atherosclerotic Disease Status in Middle-Aged Saudi Arabian Dwellers

et al. 2018

View full text Add to dashboard Cite

A number of food micronutrients are reported to influence markers of cardio-metabolic health. There is an expectation that there may be an optimal endocrine profile, with triglycerides as a key factor, which may help minimise atherosclerotic disease and associated risk factors. This study involved 84 participants aged (mean ± SD) 48.2 ± 8.3 years from both sexes segregated into n = 30 controls, n = 25 at-risk, and n = 29 diagnosed with atherosclerosis, including 20 participants using statins. Atherosclerosis status and risk factors were assessed using a combination of clinical records, C-reactive protein (CRP), blood glucose (FBG), lipids profiles, vascular structural and functional characteristics (including carotid-radial pulse wave velocity (CR-PWV), central systolic blood pressure (C-SBP), peripheral systolic blood pressure (P-SBP), peripheral diastolic blood pressure (P-DBP), carotid intima-media thickness (IMT), and carotid artery inter-adventitial diameter (IAD)). There was a significant difference in triglycerides (TG) levels between the clinical groups (p < 0.05) and between the users and non-users of statin (p < 0.001). Significant associations were distinguished between TG and CRP, FBG, high-density lipoprotein (HDL), C-SBP, P-SBP, P-DBP, CR-PWV, heart rate (HR), and body weight in the pooled sample (p < 0.05). In non-users of statin, TG was associated with C-SBP, P-SBP, P-DBP, and HR. In sub-clinical groups, TG was also associated with most of the blood markers. After controlling for statin use, composite z-score analysis revealed 48%, 2%, and 0% differences in in vivo vascular phenotype between high and low TG subgroups in controls, at-risk, and diagnosed atherosclerosis groups, respectively. Thus, TG levels seem to be good indicators for incidence and risk factors of atherosclerosis.

show abstract

Section: Introductionmentioning

confidence: 99%

Impact of Circulating Triglycerides Concentration on Atherosclerotic Disease Status in Middle-Aged Saudi Arabian Dwellers

et al. 2018

View full text Add to dashboard Cite

show abstract

“…Several groups have reported cases of chylomicronemia from LPL autoantibodies (87)(88)(89)(90)(91)(92)(93)(94)(95)(96)(97). The evidence for LPL autoantibodies has included the ability of the patient's serum to block LPL catalytic activity, the ability of the immunoglobulins in the serum to bind to LPL on ELISA plates, the ability of immunoglobulins in the serum to bind (in western blots) to a protein in postheparin plasma with a molecular weight similar to LPL (~52 kDa), or the ability of immunoglobulins in the serum to bind to LPL purchased from a commercial source.…”

Section: Downloaded Frommentioning

confidence: 99%

Chylomicronemia from GPIHBP1 autoantibodies

Miyashita

Lutz²,

Hudgins

et al. 2020

Journal of Lipid Research

View full text Add to dashboard Cite

Some cases of chylomicronemia are caused by autoantibodies against GPIHBP1, an endothelial cell protein that shuttles lipoprotein lipase (LPL) to the capillary lumen. GPIHBP1 autoantibodies prevent binding and transport of LPL by GPIHBP1, thereby disrupting the lipolytic processing of triglyceride-rich lipoproteins. Here, we review the “GPIHBP1 autoantibody syndrome” and summarize clinical and laboratory findings in 22 patients. All patients had GPIHBP1 autoantibodies and chylomicronemia, but we did not find a correlation between triglyceride levels and autoantibody levels. Many of the patients had a history of pancreatitis, and most had clinical and/or serological evidence of autoimmune disease. IgA autoantibodies were present in all patients, and IgG4 autoantibodies were present in 19 of 22 patients. Patients with GPIHBP1 autoantibodies had low plasma LPL levels, consistent with impaired delivery of LPL into capillaries. Plasma levels of GPIHBP1, measured with a monoclonal antibody–based ELISA, were very low in 17 patients, reflecting the inability of the ELISA to detect GPIHBP1 in the presence of autoantibodies (immunoassay interference). However, GPIHBP1 levels were very high in 5 patients, indicating little capacity of their autoantibodies to interfere with the ELISA. Recently, several GPIHBP1 autoantibody syndrome patients were treated successfully with rituximab, resulting in the disappearance of GPIHBP1 autoantibodies and normalization of both plasma triglyceride and LPL levels. The GPIHBP1 autoantibody syndrome should be considered in any patient with newly acquired and unexplained chylomicronemia.

show abstract

Approach to children with Sjögren’s syndrome

Stern

Basiaga

2020

Sjogren's Syndrome

View full text Add to dashboard Cite

A case of severe acquired hypertriglyceridemia in a 7-year-old girl

Cited by 7 publications

References 13 publications

Impact of Circulating Triglycerides Concentration on Atherosclerotic Disease Status in Middle-Aged Saudi Arabian Dwellers

Impact of Circulating Triglycerides Concentration on Atherosclerotic Disease Status in Middle-Aged Saudi Arabian Dwellers

Chylomicronemia from GPIHBP1 autoantibodies

Approach to children with Sjögren’s syndrome

Contact Info

Product

Resources

About