ArticleJournal of the Endocrine Society2019
Differentiating Familial Chylomicronemia Syndrome From Multifactorial Severe Hypertriglyceridemia by Clinical Profiles.
Article in Journal of the Endocrine Society, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
23 citing papers in PubMed, 62 citations in OpenAlex.
- Dietary Interventions for Hypertriglyceridemia.Current atherosclerosis reports · 2026Review
- From Phenotype to Genotype and Beyond: Insights into Familial Hypercholesterolemia and Familial Hypertriglyceridemia.Medicina (Kaunas, Lithuania) · 2026Review
- Beyond triglyceride clearance: rethinking hypertriglyceridemia-associated acute pancreatitis in the apoC-III era.Gastroenterology report · 2026Review
- Content validity of the Familial Chylomicronemia Syndrome Symptoms and Impacts Scale (FCS-SIS) for severe hypertriglyceridemia (sHTG).Patient related outcome measures · 2026Article
- The Ongoing Utility of lipoprotein lipase activity in diagnosing familial Chylomicronemia Syndrome.Biochemistry and biophysics reports · 2025Article
- Clinical considerations for the treatment of patients with familial chylomicronemia syndrome using a hepatic-targetedAmerican journal of preventive cardiology · 2025Review
- Experience with apheretic treatment in the chronic management of severe hypertriglyceridemia: case series.Endocrine · 2025Article
- Novel Therapeutics for Familial Chylomicronemia Syndrome.Current atherosclerosis reports · 2025Review
- What is the phenotype of heterozygous lipoprotein lipase deficiency?Current opinion in lipidology · 2025Review
- An overview of persistent chylomicronemia: much more than meets the eye.Current opinion in endocrinology, diabetes, and obesity · 2025Review
- Comparison of Patients With Familial Chylomicronemia Syndrome and Multifactorial Chylomicronemia Syndrome.The Journal of clinical endocrinology and metabolism · 2025Article
- Symptoms and impacts of familial chylomicronemia syndrome: a qualitative study of the patient experience.Orphanet journal of rare diseases · 2023Article
- Severe Hypertriglyceridaemia and Chylomicronaemia Syndrome-Causes, Clinical Presentation, and Therapeutic Options.Metabolites · 2023Review
- Analyses of familial chylomicronemia syndrome in Pereira, Colombia 2010-2020: a cross-sectional study.Lipids in health and disease · 2023Article
- Magic bubbles: utilizing histotripsy to modulate the tumor microenvironment and improve systemic anti-tumor immune responses.International journal of hyperthermia : the official journal of European Society for Hyperthermic Oncology, North American Hyperthermia Group · 2023Review
- Low circulating PCSK9 levels inFrontiers in genetics · 2022Article
- The Evolving Story of Multifactorial Chylomicronemia Syndrome.Frontiers in cardiovascular medicine · 2022Review
- Familial hypertriglyceridemia: an entity with distinguishable features from other causes of hypertriglyceridemia.Lipids in health and disease · 2021Article
- Non-Alcoholic Fatty Liver in Patients with Chylomicronemia.Journal of clinical medicine · 2021Article
- Familial Chylomicronemia Syndrome (FCS): Recent Data on Diagnosis and Treatment.Current atherosclerosis reports · 2020Review
Corrections and comments
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Authors and funding
13 authors at 11 institutions in 6 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
contextDifferentiation between familial chylomicronemia syndrome (FCS, type 1 hyperlipoproteinemia), a rare metabolic disorder, and the more common multifactorial severe hypertriglyceridemia (sHTG, type 5 hyperlipoproteinemia) is challenging because of their overlapping symptoms but important in patient management.
objectiveTo assess whether readily obtainable clinical information beyond triglycerides can effectively diagnose and differentiate patients with FCS from those with sHTG, based on well-curated data from two intervention studies of these conditions.
methodsThe analysis included 154 patients from two phase 3 clinical trials of patients with sHTG, one cohort with genetically confirmed FCS (n = 49) and one with multifactorial sHTG (n = 105). Logistic regression analyses were performed to determine the ability of variables (patient demographics, medical history, and baseline lipids, individually or in sets) to differentiate the patient populations. Receiver operating characteristics were used to determine the variable sets with the highest accuracy (percentage of times actual values matched predicted) and optimal sensitivity and specificity.
resultsThe primary model diagnosed 45 of 49 patients with FCS and 99 of 105 patients with sHTG correctly. Optimal sensitivity for all available parameters (n = 17) was 91.8%, optimal specificity was 94.3%, and accuracy was 93.5%. Fasting low-density lipoprotein cholesterol (LDL-C) provided the highest individual predictability. However, a three-variable set of ultracentrifugally measured LDL-C, body mass index, and pancreatitis history differentiated the diseases with a near similar accuracy of 91.0%, and adding high-density lipoprotein cholesterol and very low-density lipoprotein cholesterol for a five-variable set provided a small incremental increase in accuracy (92.2%).
conclusionsIn the absence of genetic testing, hypertriglyceridemic patients with FCS and sHTG can be differentiated with a high degree of accuracy by analyzing readily obtainable clinical information.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.