Journal of clinical lipidology最新文献

{"title":"Extreme lipoprotein(a) levels and the risk of acute myocardial infarction by standard modifiable cardiovascular risk factors among US adults","authors":"Joana Tome MPH,&nbsp;Monica Silver PhD,&nbsp;Maria Weck MPH,&nbsp;Cory Pack BS,&nbsp;Maryam Ajose MPH,&nbsp;Janna Manjelievskaia PhD,&nbsp;Elizabeth Marchlewicz PhD","doi":"10.1016/j.jacl.2025.04.006","DOIUrl":"10.1016/j.jacl.2025.04.006","url":null,"abstract":"<div><h3>Background/Synopsis</h3><div>Elevated lipoprotein(a) (Lp[a]) levels have been associated with increased acute myocardial infarction (AMI) risk, but no approved treatment options directly targeting Lp(a) have been approved yet. In addition, risk modification by standard modifiable cardiovascular risk factors (SMuRFs) (hypertension, dyslipidemia, diabetes, chronic kidney disease, alcohol use disorder, smoking [former/current], and BMI [underweight/overweight/obese]) is not well described. Consequently, there is a need to characterize patients with extremely high (XHI) vs low (LO) Lp(a) levels by number of SMuRFs in the real-world setting.</div></div><div><h3>Objective/Purpose</h3><div>To describe patients with XHI vs LO Lp(a) levels by number of SMuRFs and evaluate their risk of AMI.</div></div><div><h3>Methods</h3><div>Using natural language processing-enhanced data from the Veradigm Network EHR linked to Komodo Health closed claims, we identified patients with ≥ 1 valid Lp(a) lab test between 01/01/2016 - 01/31/2023 (earliest lab +30 days=index date). Inclusion criteria consisted of EHR/claims activity ≥ 13 months pre- and ≥ 12 months post-index with no severe kidney/liver dysfunction or malignant neoplasm during the study period, and no ischemic stroke, MI, or coronary revascularization at baseline. Selected patients were categorized into LO (&lt;50th percentile) and XHI (&gt;90th percentile) Lp(a) levels and stratified by baseline number of SMuRFs (0, 1, 2, 3, 4+). Inverse probability treatment weighting was used to balance baseline characteristics between Lp(a) cohorts.</div></div><div><h3>Results</h3><div>Of 22,289 included patients, final weighted cohorts had 2,233 XHI and 11,023 LO patients with a mean (SD) Lp(a) (nmol/L) of 303.9 (78.0) vs 21.1 (10.6) (p&lt;0.0001), respectively. At baseline, total cholesterol, LDL-C, HDL-C, and triglycerides significantly differed between the Lp(a) cohorts (all p&lt;0.0001) (Table 1). During a mean (SD) overall follow-up of 1,239 (642) days, AMI was rare and did not vary by Lp(a) cohorts (XHI: 1.6% vs LO: 1.5%). However, AMI incidence (0: 0.1%, 1: 0.2%, 2: 1.0%, 3: 2.0%, 4+: 4.4%) and proportion of patients with certain risk factors increased with the number of SMuRFs: (hypertension [1: 5.3%, 2: 44.7%, 3: 82.6%, 4+: 97.1%], dyslipidemia [1: 72.2%, 2: 91.2%, 3: 96.2%, 4+: 99.2%], and diabetes [1: 1.1%, 2: 7.8%, 3: 33.3%, 4+: 74.6%]) (Table 2).</div></div><div><h3>Conclusions</h3><div>Despite the lack of significant difference in incident AMI between the XHI and LO Lp(a) cohorts, the positive association between AMI incidence and baseline number of SMuRFs suggests that XHI Lp(a) patients have other risk factors for AMI and should be followed as a high-risk population to identify candidates for new Lp(a) targeted therapies.</div></div>","PeriodicalId":15392,"journal":{"name":"Journal of clinical lipidology","volume":"19 3","pages":"Pages e3-e4"},"PeriodicalIF":3.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144167918","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Impact of PCSK9 monoclonal antibody inhibitors on Lp(a) reduction in African Americans with elevated Lp(a)","authors":"Cindy Nee PharmD,&nbsp;Mary Katherine Cheeley PharmD,&nbsp;Salman Hasham PharmD","doi":"10.1016/j.jacl.2025.04.073","DOIUrl":"10.1016/j.jacl.2025.04.073","url":null,"abstract":"<div><h3>Background/Synopsis</h3><div>Elevated Lipoprotein(a) [Lp(a)] levels are associated with a higher risk of atherosclerotic cardiovascular disease (ASCVD) such as myocardial infarction, ischemic stroke, coronary artery disease, and aortic stenosis, notably in the African American population. Proprotein convertase subtilisin/kexin type 9 monoclonal antibody inhibitors [PCSK9mab] have been shown to reduce Lp(a) by a small percentage. The FOURIER Trial demonstrated that evolocumab reduced Lp(a) by 6.2% to 46.7% with a median reduction of 26.9%. Other studies reported similar reductions, with a mean decrease of 20 to 25%. African Americans tend to have higher baseline Lp(a) and there are a limited number of studies highlighting the Lp(a) reduction in this population.</div></div><div><h3>Objective/Purpose</h3><div>To assess the effects of PCSK9mab inhibitor on Lp(a) in the African American population with elevated baseline Lp(a)</div></div><div><h3>Methods</h3><div>Single center, retrospective review of electronic medical record for all patients prescribed PCSK9mab inhibitor with baseline Lp(a) levels from January 1, 2023 to December 31, 2024. Chart review was used to further identify patients who had serial Lp(a) measurements. For the purposes of this study, Lp(a) levels were measured in nmol/L, with elevated Lp(a) defined as &gt; 125 nmol/L.</div></div><div><h3>Results</h3><div>A total of 50 patients were analyzed. The average age was 59.2 years old. 54% (n=27) of patients were female, 92% (n=46) of patients were secondary prevention patients. 92% (n=46) patients were Black. Average baseline LDL was 129 mg/dL. The highest Lp(a) &gt; 600 nmol/L. 10 patients had serial Lp(a). All 10 of the patients with serial Lp(a) were African American. The median Lp(a) decrease was 41.2%. The mean Lp(a) decrease was 38%.</div></div><div><h3>Conclusions</h3><div>While previous studies have demonstrated that PCSK9mab inhibitors decrease Lp(a) levels by a median of 26.9% and a mean of 20 to 25%. The results of this evaluation showed a greater reduction in median and mean Lp(a) levels compared to prior studies. They also suggest futher evaluation is warranted to determine if there is a real-world racial difference in Lp(a) reduction.</div></div>","PeriodicalId":15392,"journal":{"name":"Journal of clinical lipidology","volume":"19 3","pages":"Page e54"},"PeriodicalIF":3.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144168254","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Intubation rates and risk factors in SLE patients with protein-calorie malnutrition","authors":"M Kenan Rahima MD,&nbsp;Amin Eshghabadi MD,&nbsp;Nirys Mateo Faxas MD,&nbsp;Kim Nguyen MD,&nbsp;Sila Mateo Faxas MD","doi":"10.1016/j.jacl.2025.04.074","DOIUrl":"10.1016/j.jacl.2025.04.074","url":null,"abstract":"<div><h3>Background/Synopsis</h3><div>Respiratory failure requiring intubation is a serious complication in SLE patients, but the impact of protein-calorie malnutrition on intubation rates remains unclear.</div></div><div><h3>Objective/Purpose</h3><div>To determine the association between protein-calorie malnutrition and intubation requirements in hospitalized SLE patients.</div></div><div><h3>Methods</h3><div>We conducted a retrospective analysis using the National Inpatient Sample (NIS) database from 2017-2021. Cases were identified using International Classification of Diseases, 10th Revision, Clinical Modification (ICD-10-CM) codes for SLE (M32.10, M32.11, M32.12, M32.13, M32.19) and PCM (E40, E41, E42, E43, E44.0, E44.1, E46). Statistical analyses were performed using STATA/MP version 16.0 (StataCorp, College Station, TX) with survey-weighted commands to account for the complex sampling design. The primary outcome was mechanical ventilation requirement during hospitalization. Secondary outcomes included length of stay and total inflation-adjusted costs. Multivariable logistic regression models adjusted for demographics (age, sex, race) and year of admission were used to assess the association between PCM and intubation. Poisson regression was used for cost and length of stay analyses.</div></div><div><h3>Results</h3><div>Among hospitalized SLE patients, those with PCM had significantly higher intubation rates compared to those without PCM (7.74% vs 2.86%, p &lt; 0.001). After adjustment for confounders, SLE patients with PCM had 2.78-fold higher odds of requiring intubation (adjusted OR 2.78, 95% CI 2.59-2.98, p &lt; 0.001). Female sex was associated with lower intubation risk (aOR 0.79, 95% CI 0.73-0.85, p &lt; 0.001), while increasing age showed a modest positive association (aOR 1.004 per year, 95% CI 1.003-1.006, p &lt; 0.001). Racial disparities were evident, with Black patients showing higher odds of intubation compared to White patients (aOR 1.30, 95% CI 1.22-1.38, p &lt; 0.001). The PCM group demonstrated significantly longer hospital stays (mean difference 5.3 days, p &lt; 0.001) and nearly doubled hospital costs compared to non-PCM patients (adjusted cost ratio 1.87, 95% CI 1.81-1.94, p &lt; 0.001).</div></div><div><h3>Conclusions</h3><div>Protein-calorie malnutrition is independently associated with substantially increased intubation risk in hospitalized SLE patients. These findings suggest the need for early nutritional intervention and closer monitoring of respiratory status in SLE patients with PCM.</div></div>","PeriodicalId":15392,"journal":{"name":"Journal of clinical lipidology","volume":"19 3","pages":"Page e55"},"PeriodicalIF":3.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144168255","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Sex differences in cardiovascular outcomes among patients with elevated Lipoprotein(a).","authors":"Ahmed Mazen Amin MB ChB,&nbsp;Ahmed Mansour MB ChB,&nbsp;Mohamed Abdelwahab Mohamed Yassin MB ChB,&nbsp;James Mills MD,&nbsp;Basel Abdelazeem MD","doi":"10.1016/j.jacl.2025.04.081","DOIUrl":"10.1016/j.jacl.2025.04.081","url":null,"abstract":"<div><h3>Background/Synopsis</h3><div>Lipoprotein(a) [Lp(a)] has a prothrombotic and proatherogenic nature, and its elevation has been associated with an elevated risk of various atherosclerotic cardiovascular disease (ASCVD) conditions, such as peripheral arterial disease, stroke, and acute myocardial infarction (AMI). However, the studies that evaluated the risk of elevated Lp(a) levels in different genders and its relation with long-term cardiovascular events are limited.</div></div><div><h3>Objective/Purpose</h3><div>This study aims to assess long-term cardiovascular outcomes in male versus female patients with elevated Lp(a) through a comprehensive analysis of a retrospective cohort.</div></div><div><h3>Methods</h3><div>This study utilized data from the TriNetX Research Network. From a cohort of over 120 million records (spanning January 2016 to May 2021) 15,654 adult participants (≥ 18 years) with elevated Lp(a) levels were identified. We excluded individuals with unspecified sex; the remaining were divided into male and female groups. Propensity score matching (PSM) (1:1) based on baseline characteristics was performed. Our primary outcome was new-onset atrial fibrillation (NOAF).</div></div><div><h3>Results</h3><div>Our study included 6,484 male and 7,201 female patients from the TriNetX database. After PSM, there were 5,094 in each group. Male patients were associated with a higher risk of NOAF (Hazard Ratio (HR): 1.678, 95% CI [1.356, 2.075], p &lt; 0.001), ASCVD (HR: 1.158, 95% CI [1.083, 1.239], p &lt; 0.001), new-onset ischemic heart disease (NO-IHD) (HR: 1.282, 95% CI [1.106, 1.486], p = 0.001), new-onset aortic valve stenosis (NO-AVS) (HR: 1.499, 95% CI [1.031, 2.179], p = 0.033). However, no significant differences were observed in ischemic stroke, all-cause hospitalization, AMI, new-onset heart failure, systemic embolism, thromboembolism, and all-cause mortality.</div></div><div><h3>Conclusions</h3><div>Male patients with elevated Lp(a) levels were associated with an increased risk of adverse cardiovascular outcomes, including NOAF, ASCVD, NO-IHD, and NO-AVS. This gender-specific risk should be considered when evaluating cardiovascular risk and planning treatment.</div></div>","PeriodicalId":15392,"journal":{"name":"Journal of clinical lipidology","volume":"19 3","pages":"Pages e59-e60"},"PeriodicalIF":3.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144168363","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"† Omega-3 index improves upon the pooled cohort equation in predicting risk for CVD","authors":"William Franco MD,&nbsp;James O'Keefe MD,&nbsp;Nathan Tintle PhD,&nbsp;Evan O'Keefe MD,&nbsp;Roberto Marchioli MD,&nbsp;William Harris PhD","doi":"10.1016/j.jacl.2025.04.030","DOIUrl":"10.1016/j.jacl.2025.04.030","url":null,"abstract":"<div><h3>Background/Synopsis</h3><div>Accurate predictive tools are crucial for identifying patients at increased risk for atherosclerotic cardiovascular disease (ASCVD). The Pooled Cohort Equation (PCE) is commonly used to predict 10-year risk for ASCVD, but the extent to which it can be improved upon by the inclusion of a measure of omega-3 fatty acid status is unknown.</div></div><div><h3>Objective/Purpose</h3><div>This study examined the extent to which the omega-3 Index (O3I; the proportion of EPA+DHA in erythrocyte membranes) improved the predictive capability of the PCE and each of its modifiable components.</div></div><div><h3>Methods</h3><div>The O3I was determined in 2,550 participants without ASCVD at baseline from Framingham Offspring Cohort. The extent to which the O3I added to the PCE score was assessed using the area under the curve (AUC) metric. Changes in the AUC were also used to determine the extent to which the O3I added predictive power to each standard risk factor (blood pressure, diabetes, smoking, total and HDL cholesterol) individually when added to the basic (age+sex+race) model. Median follow-up was 10 years.</div></div><div><h3>Results</h3><div>The AUC predicting 10-year ASCVD events using PCE was 0.689. It increased to 0.698 upon addition of the O3I (p &lt; 0.05). When added to the basic model, the O3I increased the AUC by 0.012 units; this compared with 0.028 for blood pressure and for HDL-C; 0.020 for diabetes, 0.006 for cholesterol, and 0.004 for smoking [all but smoking were significant (p &lt; 0.05)]. Thus, the predictive power provided by the O3I was roughly equivalent to that provided by each of the 5 standard risk factors. Also, the O3I significantly (p &lt; 0.05) improved the predictive ability of each of these risk factors when analyzed separately (Figure). This suggests that the mechanism(s) by which a higher O3I operates to lower risk is, at least in part, independent of effects on these other risk factors. Interestingly, the 6 additional factors together (beyond age, sex and race) increased the AUC by only 0.068 units (<em>i.e.,</em> from 0.672 to 0.740), indicating that most of the predictive power of the PCE is derived from fixed, unmodifiable risk factors.</div></div><div><h3>Conclusions</h3><div>The O3I improved the PCE prediction, suggesting that it captures risk beyond standard factors. Thus, the O3I may help in ASCVD risk stratification. Further research is needed to extend these findings into more diverse cohorts and to explore the integration of O3I into other existing ASCVD risk assessment tools.</div><div><strong>Previously Published:</strong> Yes, Currently accepted (Dec 13, 20024) at <em>Journal of Clinical Lipidology</em>.</div></div>","PeriodicalId":15392,"journal":{"name":"Journal of clinical lipidology","volume":"19 3","pages":"Pages e22-e23"},"PeriodicalIF":3.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144168570","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Promoting lipoprotein(a) awareness and testing for risk identification through outreach and teaching (PATRIOT-QI)","authors":"Julie Ponn DO,&nbsp;Harsh Singh MD,&nbsp;Emaad Siddiqui MD,&nbsp;Meet Shah DO,&nbsp;Matthew Fanous MD,&nbsp;Renjit Thomas MD,&nbsp;Soniya Bhate MD,&nbsp;Navid Radfar DO","doi":"10.1016/j.jacl.2025.04.010","DOIUrl":"10.1016/j.jacl.2025.04.010","url":null,"abstract":"&lt;div&gt;&lt;h3&gt;Background/Synopsis&lt;/h3&gt;&lt;div&gt;Elevated Lipoprotein(a) [Lp(a)], is a genetically determined, highly heritable form of dyslipidemia, independently associated with coronary heart disease, peripheral artery disease, and calcific aortic stenosis. Despite being a well-established cardiovascular (CV) risk factor supported by existing guidelines, screening remains limited, particularly within the Veterans Affairs (VA) hospitals. While targeted Lp(a) lowering therapies are under investigation, addressing other modifiable CV risk factors remains critical for reducing overall CV disease burden.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Objective/Purpose&lt;/h3&gt;&lt;div&gt;The National Lipid Association (NLA) recommends measuring Lp(a) levels at least once in all adults for CV risk assessment. This Quality Improvement (QI) initiative aimed to evaluate and improve adherence to the NLA screening guidelines by implementing targeted educational interventions in an outpatient setting.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Methods&lt;/h3&gt;&lt;div&gt;This single-center QI project employed the Plan-Do-Study-Act framework. Baseline Lp(a) screening data were collected retrospectively from electronic medical records (EMRs) of health profession trainee (HPT) driven and attending practices at the VA between April 2024 to July 2024. To increase provider awareness and testing, a series of educational interventions were implemented. These included educational flyers distributed via email and displayed at HPT workstations; biweekly educational emails sent during the first two months of intervention, focusing on the clinical relevance of Lp(a) testing; and in-person presentations consisting of two educational sessions (October 2024 and December 2024) with HPTs to review Lp(a)’s clinical significance and the NLA guidelines.&lt;/div&gt;&lt;div&gt;Following these interventions, EMRs of HPT and attending clinic patients between August 2024 to December 2024 were reviewed to evaluate their impact.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Results&lt;/h3&gt;&lt;div&gt;A total of 198 patients were included in this study. In the pre-intervention phase, 8 Lp(a) tests were ordered by 5 providers: 4 attending physicians, 1 Nurse Practitioner (NP). No tests were ordered by HPTs (Figure 1). Among those tested, 2 patients (25%) had elevated Lp(a) levels. In the post-intervention phase, 190 tests were ordered by 30 providers: 7 attending physicians, 3 NPs, 20 HPTs (Figure 1). Among those tested, 82 (43.16%) had elevated Lp(a) levels. Screening rates demonstrated a notable upward trend post-intervention (Figure 2).&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Conclusions&lt;/h3&gt;&lt;div&gt;Educational interventions yielded an approximate 24-fold increase in Lp(a) screening. This expanded testing detected a larger proportion of patients who would have otherwise remained undetected as high risk, offering additional opportunities to optimize treatment and address modifiable risk factors. These findings demonstrate the efficacy of targeted education in improving early detection, risk stratification, and management of elevated ","PeriodicalId":15392,"journal":{"name":"Journal of clinical lipidology","volume":"19 3","pages":"Pages e6-e7"},"PeriodicalIF":3.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144167927","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"*A unique case of xanthoma tendinosum","authors":"Bart Duell MD,&nbsp;Bani Azari MD,&nbsp;Gisette Reyes-Soffer MD,&nbsp;Antonios Gasparis MD,&nbsp;Eugenia Gianos MD,&nbsp;Dorota Gruber DHSc,&nbsp;Thomas Dayspring MD,&nbsp;Natasha Vartak DO","doi":"10.1016/j.jacl.2025.04.049","DOIUrl":"10.1016/j.jacl.2025.04.049","url":null,"abstract":"<div><h3>Background/Synopsis</h3><div>Tendon xanthomas are a rare physical finding most commonly associated with familial hypercholesterolemia (FH), but can be associated with other genetic diagnoses.</div></div><div><h3>Objective/Purpose</h3><div>We describe a patient with prominent xanthomas and the diagnostic approach.</div></div><div><h3>Methods</h3><div>Electronic medical records of the patient were reviewed.</div></div><div><h3>Results</h3><div>A 54-year-old man, non-smoker, of Greek ethnicity presented with large xanthomas on his Achilles tendons, knees, and metacarpal joints (Figure 1). He first noticed xanthomas in his thirties and was started on rosuvastatin due to paternal family history of early heart disease. The xanthomas progressed despite statin therapy. He did not participate in sports as a child due to associated fatigue. Besides mild memory issues, he had no complaints.</div><div>Although FH was the leading diagnosis in the differential for xanthomas, his untreated LDL-C was never higher than 120 mg/dL and was 58mg/dL on rosuvastatin 20mg daily. His lipoprotein(a) (Lp(a)) was in normal range and Lp(a) elevation is not known to be associated with xanthomas. Based on exertional dyspnea with aerobic exercise, a coronary CT angiography was performed showing mild non-obstructive coronary artery disease, and his echocardiogram was normal.</div><div>Phytosterolemia was excluded by normal plasma sitosterol concentration and absence of pathogenic variants in ABCG5 or ABCG8. Cerebrotendinous xanthomatosis (CTX) was also considered, however, he had no typical symptoms, normal cholestanol levels, a normal neurological evaluation and normal MRI of his brain, making CTX less likely. A 25 gene genetic panel revealed pathogenic homozygous variants in the CYP27A1 gene. Subsequent levels of plasma 7-alpha, 12-alpha-dihydroxy-4-cholesten-3-one and urine bile alcohols were consistent with CTX. He was started on chenodeoxycholic acid and aspirin in the setting of coronary disease.</div></div><div><h3>Conclusions</h3><div>CTX is a rare, recessive genetic lipid storage disorder. Mutations in the CYP27A1 gene cause sterol 27-hydroxylase deficiency which blocks bile acid synthesis and leads to accumulation of bile acid metabolites in tissues. Cholestanol is commonly elevated, but not always. CTX can affect many organ systems and diagnosis is challenging due to varied phenotypes. Early onset cataracts, diarrhea, and progressive neurological decline are common. Some patients develop atherosclerosis. Early diagnosis is key to preventing complications. To date, there are few known cases of CTX with normal cholestanol as seen in our patient, but almost all have large xanthomas. Screening for CTX in patients with xanthomas is recommended, with a normal cholestanol level being insufficient to exclude the diagnosis. Further studies are needed to better understand phenotypic variations in patients with genetically confirmed CTX.</div></div>","PeriodicalId":15392,"journal":{"name":"Journal of clinical lipidology","volume":"19 3","pages":"Pages e35-e36"},"PeriodicalIF":3.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144168312","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"† Cholesterol treatment trends from 2014 - 2023 in 3 Million US individuals with ASCVD: a retrospective cohort analysis using the Family Heart DatabaseTM","authors":"Diane MacDougall MS,&nbsp;Hilly Paige BS,&nbsp;Seth Baum MD,&nbsp;Katherine Wilemon BS,&nbsp;Keith Ferdinand MD","doi":"10.1016/j.jacl.2025.04.015","DOIUrl":"10.1016/j.jacl.2025.04.015","url":null,"abstract":"<div><h3>Background/Synopsis</h3><div>Over the last decade, the preponderance of clinical trial data and the availability of innovative therapies have supported attainment of lower LDL-C levels, while the 2018 US guideline and quality measures have primarily emphasized use of statins. How these and other factors have impacted cholesterol treatment over time is unknown.</div></div><div><h3>Objective/Purpose</h3><div>This real-world data analysis aims to characterize annual use of LDL-C lowering therapies and attainment of LDL-C goals in US individuals with atherosclerotic cardiovascular disease (ASCVD).</div></div><div><h3>Methods</h3><div>A retrospective cohort analysis using medical claims and lab data from the Family Heart DatabaseTM that was created by the Family Heart Foundation. Individuals with an ASCVD diagnosis prior to 2014 and ≥ 1 medication claim every year from 2014 to 2023 were included. Cholesterol lowering therapies included statins, proprotein convertase subtilisin/kexin type 9 inhibitors (PCSK9is), ezetimibe and bempedoic acid. Percentages were adjusted for age and sex using logistic regression.</div></div><div><h3>Results</h3><div>Individuals (n=3,042,530) with mean (SD) age 63.7 (9.2) years were 45% female. From 2014 to 2023, annual use of low/moderate intensity statins consistently decreased from 34% to 27%, while corresponding use of high intensity statins increased from 17% to 29%. Use of cholesterol lowering therapies and attainment of LDL-C goals increased over time; however, in 2023 just 55% of individuals were receiving LDL-C lowering therapy and only 34% had attained an LDL-C level &lt;70 mg/dL (see table).</div></div><div><h3>Conclusions</h3><div>In the largest analysis of cholesterol treatment trends in US individuals with ASCVD to date, use of LDL-C lowering therapies and attainment of LDL-C goals gradually improved from 2014 to 2023. However, by 2023, 66% of these individuals failed to attain an LDL-C level &lt; 70 mg/dL and 45% received no LDL-C lowering therapy. These findings highlight suboptimal cholesterol treatment and slow improvement over time despite increasing availability of multiple cost-effective and safe therapies.</div><div><strong>Previously Published:</strong> Yes, Accepted for oral presentation at EAS May 2025 in Glasgow</div></div>","PeriodicalId":15392,"journal":{"name":"Journal of clinical lipidology","volume":"19 3","pages":"Page e11"},"PeriodicalIF":3.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144168313","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Elevated lipoprotein(a) levels attenuate LDL-C lowering in acute coronary syndrome patients treated with triple combination lipid-lowering therapy","authors":"Jaibharat Sharma MD,&nbsp;Surender Himral MD,&nbsp;Roshan Thakur MD,&nbsp;Raman Puri MD,&nbsp;Pankaj Chandel,&nbsp;Komal Mohite,&nbsp;Aziz Khan MD,&nbsp;Rajeev Agarwala MD,&nbsp;Vinod Vijan MD,&nbsp;Kunal Mahajan MD","doi":"10.1016/j.jacl.2025.04.018","DOIUrl":"10.1016/j.jacl.2025.04.018","url":null,"abstract":"<div><h3>Background/Synopsis</h3><div>Elevated lipoprotein(a) [Lp(a)] is recognized as an independent risk factor for adverse outcomes in coronary artery disease patients. Research on Lp(a) reduction in acute coronary syndrome (ACS) is ongoing. The standard of care for ACS includes high-intensity statins (HIS), which can paradoxically increase Lp(a) levels. While ezetimibe and PCSK9 inhibitors reduce Lp(a), bempedoic acid (BA) shows minimal effect. Limited data exist regarding the impact of elevated Lp(a) (50 mg/dL or higher) on LDL-C lowering in ACS patients undergoing aggressive lipid-lowering therapy (LLT).</div></div><div><h3>Objective/Purpose</h3><div>To evaluate changes in Lp(a) levels and their association with LDL-C lowering response in ACS patients treated with a triple combination of 40 mg rosuvastatin, ezetimibe, and BA (REB regimen).</div></div><div><h3>Methods</h3><div>We retrospectively analyzed data from statin-naive ACS patients at our center who received the REB regimen between March 2023 and December 2024. Only patients with consistent LLT for one month and available Lp(a) and LDL-C levels at index ACS admission and one-month post-ACS were included. We assessed the impact of changes in Lp(a) levels, particularly those exceeding 50 mg/dL, on mean LDL-C reduction and the percentage of patients achieving the LDL-C target of &lt; 50 mg/dL.</div></div><div><h3>Results</h3><div>Data from 303 patients were analyzed. Mean LDL-C at admission was 111.8 ± 34.6 mg/dL, decreasing to 44.7 ± 15.9 mg/dL at one month(60% reduction). 70.3% (213/303) of patients achieved LDL-C &lt; 50 mg/dL at one month. Median Lp(a) increased from 28.1 mg/dL at admission to 64.6 mg/dL at one month. The prevalence of Lp(a) 50 mg/dL or higher was 28.7% at admission and 60.4% at one month. Logistic regression analysis indicated that an increase in Lp(a) was associated with a decreased probability of achieving LDL-C &lt; 50 mg/dL at one month [Odds ratio=0.98(0.97-0.99), p&lt;0.01]. The mean LDL-C achieved at one month was significantly higher in patients with elevated Lp(a) 50 mg/dL or higher, both at admission (54.3 ± 16.7 vs 40.9 ± 13.8, p &lt; 0.001) and at one month (48.7 ± 16.1 vs 38.5 ± 13.3, p &lt; 0.001).</div></div><div><h3>Conclusions</h3><div>In ACS patients treated with aggressive LDL-C lowering therapy, elevated Lp(a) levels are associated with a blunted LDL-C response. The increase in Lp(a) during aggressive LDL-C lowering may hinder optimal LDL-C targets. These findings highlight the need for Lp(a) monitoring in ACS patients and suggest that Lp(a)-lowering therapies may optimize cardiovascular risk reduction. Further research is needed to evaluate the clinical impact of Lp(a)-lowering strategies in patients undergoing aggressive LDL-C management post-ACS.</div></div>","PeriodicalId":15392,"journal":{"name":"Journal of clinical lipidology","volume":"19 3","pages":"Page e13"},"PeriodicalIF":3.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144168316","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Care of youth with lipid disorders: A brief provider survey","authors":"David Farbo PhD,&nbsp;Donnie Wilson MD,&nbsp;Maya Rashad BS","doi":"10.1016/j.jacl.2025.04.025","DOIUrl":"10.1016/j.jacl.2025.04.025","url":null,"abstract":"<div><h3>Background/Synopsis</h3><div>Although CVD-related symptoms and events rarely occur in childhood, risk factors are present at an early age. Lifelong exposure to elevated cholesterol, particularly in those with familial hypercholesterolemia, significantly increases risk of premature CVD-related events such as MI and CVA at a later age. Early identification and treatment of all risk factors, including elevated cholesterol, have proven to be effective in reducing morbidity and mortality. In the past 30 years, there has been increasing interest in early intervention, as atherosclerosis begins in childhood. Professional organizations have increasingly begun to focus on risk in the pediatric population. As a result, there is a need for experienced pediatric healthcare professionals with unique knowledge, as well as dedicated lipid clinics, to evaluate and properly treat youth (&lt;18 years-of-age) with lipid disorders.</div></div><div><h3>Objective/Purpose</h3><div>The aim of this project was to describe the number and scope of services in the U.S. and Canada available to youth (&lt; 18 years-of-age) with lipid disorders.</div></div><div><h3>Methods</h3><div>We designed an internet survey (Qualtrics®) to collect clinic-specific data, including age range of patients, percentage of time spent in care of children with lipid disorders and available clinical resources. Additional data included years of experience and type of practice. A list of over 150 lipidologists and clinical staff, representing a variety of pediatric and combined adult and pediatric clinics, obtained from the Pediatric Atherosclerosis Prevention and Lipidology (PeDAL) working group, was used as the target population. Completion of the survey was voluntary and all responses anonymous.</div></div><div><h3>Results</h3><div>There were 39 MD/DO respondents. Almost 80% of all respondents practice in an academic setting. Additional staff include a dietitian/nutritionist (81.4%), pediatric nurse (65%), and advanced practice practitioners (NP/PA) (37.2%). For non-English speaking families 51% of respondents had access to an in-person translator and 74.4% reported having a virtual translator service with audio and visual capabilities. A wide range of educational materials are available to patients/families ranging from handouts, internet links and pamphlets/booklets. In addition to English, educational materials are available in additional languages, such as Spanish (58.1%), Arabic (4.6%) and Chinese (2.3%).</div></div><div><h3>Conclusions</h3><div>This brief internet survey provides insight into the scope of services offered to children and adolescents with lipid disorders. Further studies should focus on best practices in pediatric lipid clinics to help inform clinical care and improve outcomes for this vulnerable population.</div></div>","PeriodicalId":15392,"journal":{"name":"Journal of clinical lipidology","volume":"19 3","pages":"Pages e17-e19"},"PeriodicalIF":3.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144168731","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}