Effect of evolocumab on fasting and post fat load lipoprotein distribution and composition in familial dysbetalipoproteinemia
Approximately 0.7% of the general population is homozygous for the ɛ2 allele in the APOE gene.1 Since about 10-18% of these ɛ2ɛ2 individuals develop the specific dysbetalipoproteinemia phenotype,2,3 the estimated prevalence of familial dysbetalipoproteinemia (FD) is 1 in 850 to 3500 individuals, making FD the second most common monogenic lipid disorder after fam ilial hypercholesterolemia.4 FD is associated with a very high risk of cardiovascular disease (CVD), due to accumulation of atherogenic cholesterol-enriched triglyceride-rich lipoproteins (TRLs) and generally displays a (very) low concentration of low-density li...
Source: Journal of Clinical Lipidology - July 21, 2023 Category: Lipidology Authors: Britt E Heidemann, A David Marais, Monique T Mulder, Frank LJ Visseren, Jeanine E Roeters van Lennep, Erik S Stroes, Niels P Riksen, Leonie C van Vark – van der Zee, Dee M Blackhurst, Charlotte Koopal Tags: Original Research Source Type: research

Treatment of Cerebrotendinous Xanthomatosis in Pregnancy: Patient and Physician Perspectives
Cerebrotendinous xanthomatosis (CTX) is a rare, autosomal recessive disorder of bile acid synthesis characterized by accumulation of abnormal sterols and bile acid metabolites in various tissues.1-3 Individuals with CTX have biallelic pathogenic variants in CYP27A1, the gene that encodes for sterol 27-hydroxylase, a key enzyme involved in bile acid synthesis.1-3 Disruption of this biochemical pathway leads to bile acid deficiency and elevated levels of cholestanol and other metabolites in blood and other tissues, causing a constellation of hallmark CTX symptoms that may include neonatal cholestatic jaundice, chronic diarrh...
Source: Journal of Clinical Lipidology - July 17, 2023 Category: Lipidology Authors: P. Barton Duell, Rana Dutta, Ashley Wolf, Hollisa Rosengrant Tags: Brief Communication Source Type: research

† Evaluating the Residual Risk of Recurrent Cardiovascular Events in Contemporary Cardiovascular Outcomes Trials
Patients with coronary artery disease (CAD) remain at high risk of recurrent cardiovascular (CV) events despite secondary preventative measures. This risk is further elevated among those who develop acute coronary syndrome (ACS), where recurrent events are significantly higher compared with the general population. In addition to traditional risk factors, this residual risk results, at least in part, from residual pro-inflammatory and metabolic risk factors. (Source: Journal of Clinical Lipidology)
Source: Journal of Clinical Lipidology - July 1, 2023 Category: Lipidology Authors: Korjian Serge, Yazan Daaboul, Deepak Bhatt, Paul M Ridker, Robert W Yeh, Roxana Mehran, Suzanne Baron, Peter Libby, Gerald Chi, Arzu Kalayci, Paul Nara, Alka Shaunik, C Michael Gibson Tags: Best Practices in Lipid Management Source Type: research

⁎ Closing The Gap: A Quality Improvement Initiative to Educate House Staff and Faculty on Guideline Directed Lipid Lowering Therapy
The use of lipid lowering therapies (LLT) has consistently demonstrated reduction in cardiovascular risk. However, studies have shown that patients are undertreated, and recommended low density lipoprotein levels are infrequently achieved. (Source: Journal of Clinical Lipidology)
Source: Journal of Clinical Lipidology - July 1, 2023 Category: Lipidology Authors: Spencer Frederick Weintraub, Zohaib Bagha, Danielle Ezzo, Jenny Wahl, Moqadam Rhamatzada, Benjamin Hirsh, Jiaqi Cai Tags: Best Practices in Lipid Management Source Type: research

Addressing Gaps in Management of Homozygous Familial Hypercholesterolemia: The Impacts of Continuing Medical Education that Includes Patients
Despite the availability of a high number of educational opportunities, and an increasing number of treatments options, remaining barriers prevent clinicians (HCPs) from early management of people with Homozygous Familial Hypercholesterolemia (HoFH). Barriers include poor ability to identify probable cases, lack of knowledge of new treatment options, and lack of confidence referring patients for infusion therapy. (Source: Journal of Clinical Lipidology)
Source: Journal of Clinical Lipidology - July 1, 2023 Category: Lipidology Authors: Carole Drexel, Christie M Ballantyne, Emily Bixler Tags: Best Practices in Lipid Management Source Type: research

Trends in Lipoprotein(a) Testing at the University of Pennsylvania Health Systems from 2012-2021
Lipoprotein(a) [Lp(a)] is an independent and causal risk factor for atherosclerotic cardiovascular disease (ASCVD). Elevated Lp(a) levels are associated with a higher risk for myocardial infarction, coronary artery disease, aortic stenosis, and stroke. Lp(a) levels are primarily genetically driven and minimally impacted by diet and lifestyle, so a single measurement can identify individuals with elevated levels. However, Lp(a) screening has not been widely integrated into clinical practice in the United States. (Source: Journal of Clinical Lipidology)
Source: Journal of Clinical Lipidology - July 1, 2023 Category: Lipidology Authors: Archna Bajaj, Lakshmi Parvathinathan, Marjorie Risman, Ahmad Alsattari, Daniel J. Rader Tags: Best Practices in Lipid Management Source Type: research

Treatment Target Achievement in Patients with Familial Hypercholesterolemia: A Real-World Descriptive Study
Statin and ezetimibe are the first line of lipid-lowering treatment (LLT) in patients with familial hypercholesterolemia (FH). However, despite being treated with maximally tolerated statin dose, the achieved LDL-C remains above the treatment target for a substantial proportion of those patients who could benefit from add-on therapy. This proportion in a real-world context has never been investigated in the Province of Quebec (Canada). (Source: Journal of Clinical Lipidology)
Source: Journal of Clinical Lipidology - July 1, 2023 Category: Lipidology Authors: Alexis Wilfrid Baass, Simon Faubert, Martine Paquette, Sophie Bernard Tags: Best Practices in Lipid Management Source Type: research

† The Vast Majority of High- and Very-High Risk Hypercholesterolemia Patients Never Reach below LDL-C Thresholds in the 2018 ACC/AHA Guidelines
Based on extensive clinical trial data demonstrating lower LDL-C reduces heart attacks, strokes, and need for interventional surgery, the 2018 Multidisciplinary Guideline on the Management of Blood Cholesterol calls for initiation and intensification of lipid-lowering therapies (LLT) if LDL-C exceeds defined thresholds in high and very high (H/VH) risk patients. (Source: Journal of Clinical Lipidology)
Source: Journal of Clinical Lipidology - July 1, 2023 Category: Lipidology Authors: Katherine A Wilemon, Diane Elaine MacDougall, Mary P McGowan, William Howard, Kelly D Myers Tags: Best Practices in Lipid Management Source Type: research

† Higher Cardiovascular Event Rates for High-Risk Americans Who Did Not Meet 2018 Multidisciplinary Guideline on the Management of Blood Cholesterol Thresholds
The 2018 Multidisciplinary Guideline on the Management of Blood Cholesterol calls for initiation and intensification of lipid-lowering therapy (LLT) if low density lipoprotein cholesterol (LDL-C) exceeds thresholds in high and very high (H/VH) risk patients. Despite LDL-C being a major modifiable cardiovascular risk factor, most patients fail to meet guideline thresholds. (Source: Journal of Clinical Lipidology)
Source: Journal of Clinical Lipidology - July 1, 2023 Category: Lipidology Authors: Kelly D Myers, Diane Elaine MacDougall, Katherine A Wilemon, Catherine D Ahmed, Hilliard Paige, William Howard, Mary P McGowan Tags: Best Practices in Lipid Management Source Type: research

Genetic and Metabolic Determinants of Lipoprotein(a)
Lipoprotein(a) [Lp(a)] levels are a well-known independent risk factor for atherosclerotic cardiovascular disease. 70-90% of the variation in Lp(a) levels is genetically determined by the apolipoprotein(a) gene (LPA) locus, but relatively little is known about other regulators. Lp(a) levels and subsequent risk of CVD are associated with SNPs near other genes such as APOE and LDLR but there are currently few FDA approved treatments for elevated Lp(a) beyond PCSK9 inhibitors and plasmapheresis. (Source: Journal of Clinical Lipidology)
Source: Journal of Clinical Lipidology - July 1, 2023 Category: Lipidology Authors: Sarah L Stewart, Oleksandr Savenkov, Maurice A Hurd, Amanda Halstrom, John Falcone, Katerine Claudio, Jyothi Manohar, Fana Dealla, Sonal Kumar, Jessica M Pe ña, Michele Yeung, Judy Tung, Greg Dakin, Esther Wei, Lisa C Hudgins, Laura C Alonso, Shuibing Ch Tags: Clinical Application of Biomarkers Source Type: research

Effect of Dexamethasone on Modulating Lp(a) Levels in COVID-19 Pneumonia
It has been hypothesized that patients with COVID-19 and elevated Lipoprotein(a) have increased Interleukin-6 (IL-6) and an exaggerated inflammatory response. Moriarty, et al, hypothesized that elevated Lp(a) during COVID-19 could be a predictor of the severity of infection. Nurmohamed, et al. observed that Lp(a) increased by 16.9 mg/dL over 3 weeks in patients with COVID-19 and is strongly correlated with an increase in IL-6. They also observed a trend between the delta of IL-6 and ICU admission. (Source: Journal of Clinical Lipidology)
Source: Journal of Clinical Lipidology - July 1, 2023 Category: Lipidology Authors: Laura Grace Kukuc, Matan Khanian, Robert Fishberg, Tiffany Haynes Tags: Clinical Application of Biomarkers Source Type: research

Clinical Vignette - Keto Diet-Induced Dyslipidemia and Lean Mass Hyper-Responders
Some patients who start a very low-carbohydrate/ketogenic diet either for weight loss or to treat certain inflammatory diseases often drive their LDL-cholesterol levels quite high. It is unclear why there is heterogeneity in this LDL-response. (Source: Journal of Clinical Lipidology)
Source: Journal of Clinical Lipidology - July 1, 2023 Category: Lipidology Authors: Deepak Vedamurthy, Frances Burke, Krithika Suri, Daniel Soffer, Douglas Jacoby Tags: Complex or Unusual Cases in Lipid Management Source Type: research

Pegylated-Asparaginase Induced Severe Hypertriglyceridemia in an Adult with Acute B-cell Lymphoblastic Leukemia
We review a case of a young woman with hypertriglyceridemia after Pegylated-Asparaginase (PEG-ASP) treatment for B-cell LL. (Source: Journal of Clinical Lipidology)
Source: Journal of Clinical Lipidology - July 1, 2023 Category: Lipidology Authors: Bernardo Andres Acevedo-Mendez, Yuting Ye, Eugenia Gianos, Guy Mintz Tags: Complex or Unusual Cases in Lipid Management Source Type: research

Severe Heterozygous Familial Hypercholesterolemia and Elevated Lipoprotein(a): A Double Whammy
A 47-year-old African American female presented to the Preventive Cardiology program with heterozygous familial hypercholesterolemia (HeFH), with a pathogenic variant in LDLR (c.582C>T(p.Glu228*)) and an LDL-C level of 262 mg/dL on alirocumab 150 mg biweekly and ezetimibe 10 mg daily. She was intolerant to statins due to myalgias. Family history was notable for her mother who died from an MI at age 50, her maternal aunt who had CABG in her 60s, and a maternal cousin who died from an MI at age 52. (Source: Journal of Clinical Lipidology)
Source: Journal of Clinical Lipidology - July 1, 2023 Category: Lipidology Authors: Lakshmi Parvathinathan, Isaac Elysee, Archna Bajaj Tags: Complex or Unusual Cases in Lipid Management Source Type: research

Statin Use After ICH; Is It Safe? A Common but Complex Clinical Scenario under Current Investigation
Statin therapy and lower LDL-c levels have been observed to potentially increase risk of intracerebral hemorrhage (ICH). However observational studies and randomized clinical trials have yielded conflicting results. Patients with ASCVD risk factors who suffer an ICH pose a clinical challenge as statin association with ICH is unclear. (Source: Journal of Clinical Lipidology)
Source: Journal of Clinical Lipidology - July 1, 2023 Category: Lipidology Authors: Maxwell Ambrosino, Deepak Vedamurthy, Daniel Soffer, Douglas Jacoby Tags: Complex or Unusual Cases in Lipid Management Source Type: research