Homozygous familial hypercholesterolemia (hoFH) may cause life-threatening atherosclerotic cardiovascular disease in childhood. Lipoprotein apheresis (LA) is considered a pivotal treatment option, ...but data on its efficacy, safety and optimal performance are limited. We therefore established an international registry on the execution and outcomes of LA in HoFH children. Here we report LA policies and short-term outcomes.
We approached centers worldwide, involved in LA in children with hoFH for participation. We collected information on clinical and treatment characteristics on patients aged 0–19 years between November 2016 and November 2018.
We included 50 children, treated at 15 sites. Median (IQR) LDL-C levels at diagnosis, on medication and on LA were 19.2 (16.2–22.1), 14.4 (10.8–16.7) mmol/L and 4.6 mmol/L, respectively. Median (IQR) time between diagnosis and start of LA was 2.8 (1.0–4.7) years. Six (12%) patients developed cardiovascular disease during that period. Most children received LA either weekly (43%) or biweekly (37%). Seven (17%) patients reached mean LDL-C levels <3.5 mmol/L, all of them treated at least weekly. Xanthomas were present in 42 (84%) patients at diagnosis and disappeared completely in 19 (45%) on LA. Side effects of LA were minor. There were significant differences in LA conduction between sites in terms of frequency, responsible medical specialities and vascular access.
LA is a safe treatment and may effectively lower LDL-C in children with HoFH. However, there is room for improvement with respect to time of onset and optimization of LA therapy in terms of frequency and execution.
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•Lipoprotein apheresis (LA) is safe in children with hoFH with a paucity of side effects.•LA leads to an important reduction of LDL-C and xanthomas in children with hoFH.•Only a small number of children with hoFH reach treatment goals on LA.•There are important differences in LA conduction strategies between different sites.
Abstract Cardiovascular disease (CVD) is multifactorial in etiology. Traditional cardiovascular risk factors, such as increased cholesterol concentrations and blood pressure, are used to assess CVD ...risk. Recently, better understanding of the role of inflammation in atherosclerosis has prompted many to propose the measurement of various inflammatory markers to better identify those who are at increased risk. C-reactive protein (CRP) is found in endothelial atherosclerotic lesions, and evidence suggests that it may play a role in atherogenesis. Of candidate serum markers that might add information to clinical risk assessment, high-sensitivity C-reactive protein (hsCRP) measurement has the most potential for clinical use for multiple reasons: (a) high hsCRP is associated with a twofold to a threefold increase in the prevalence of myocardial infarction, stroke, and peripheral vascular disease, and it predicts incident cardiovascular events in those with and without preexisting CVD; (b) the increased risk associated with high hsCRP is independent of other established risk factors; (c) hsCRP augments the predictive capacity of the Framingham Risk Score; (d) hsCRP assays are standardized, and this analyte is biologically stable over time; (e) various risk-reducing interventions also reduce hsCRP, and research is underway to assess whether specifically targeting hsCRP reduces CVD risk. National guidelines regarding the clinical utility of hsCRP in primary and secondary prevention settings have been recently issued.
This study shows that only 12.5% of laboratory reports (2/16) included age-appropriate pediatric reference ranges for all lipid and lipoproteins. The use of erroneous reference range(s) could lead to ...missed alerts of dyslipidemia in up to 97.3% (total cholesterol), 93.6% (high-density lipoprotein cholesterol), 94.8% (low-density lipoprotein cholesterol), and 87.8% (triglycerides) of youth in the population-based National Health and Nutrition Examination Survey cohort. These findings highlight the potential missed opportunities for reinforcing lifestyle counseling for dyslipidemia in addition to obesity in youth.
Obesity may pose additional cardiovascular risk to children with acquired and congenital heart disease. Many children with heart disease are sedentary as a result of physician-, parent-, and/or ...self-imposed restrictions. The aim of this study was to evaluate the impact of the epidemic of obesity on children with heart disease.
A cross-sectional review was performed of children evaluated in 2004 at 2 cardiology outpatient clinics. Differences in the prevalence of obese (BMI > or = 95%) and overweight (BMI 85%-95%) children were compared with national data and healthy control subjects. Dictated letters were reviewed to determine whether obesity was discussed with referring practitioners.
Of 2921 patients assessed, 1523 had heart disease. Diagnostic subgroups included "mild" heart disease (n = 401), arrhythmia (n = 447), biventricular repair (n = 511), univentricular palliation (Fontan; n = 108), and heart transplantation (n = 56). More than 25% of the patients with heart disease were obese or overweight; the prevalence of obese and overweight children was significantly lower only in the Fontan group (15.9%). Pediatric cardiologists failed to document obesity or weight counseling in the majority of clinic letters.
Obesity is common in children with congenital and acquired heart disease. Pediatric cardiologists demonstrate inadequate communication regarding this problem to referring practitioners. Healthy-lifestyle counseling and routine exercise in children with heart disease may be underemphasized.
Aim
This study aimed to describe the current management practices for Kawasaki disease (KD) in Australia and New Zealand.
Methods
We performed a secondary analysis on the Australian and New Zealand ...responses to a large international survey of clinicians' perspectives on KD diagnosis and management.
Results
There was general consensus among Australian and New Zealand clinicians regarding the indications for intravenous immunoglobulin and aspirin in the management of acute KD. There was less consensus on the dose of these agents, the definition and management of treatment‐resistant KD and the approach to long‐term thromboprophylaxis.
Conclusion
Most clinicians use intravenous immunoglobulin for primary treatment of KD. There is variation regarding other aspects of KD diagnosis and important management issues. Future studies should confirm whether this reported variation occurs in real‐world practice and assess potential impacts on patient outcome.
•Less than half of young women feel informed about heart disease and stroke.•Young women exposed to heart disease information are more likely to feel informed.•Family and friends are common ...motivating factors to become more informed.•Preferences for heart disease information include medical providers or social media.
Cardiovascular disease (CVD) is the leading cause of death for American women, yet young women are rarely the target population of CVD prevention campaigns. This study investigated young women’s exposure to CVD information.
We surveyed 331 females ages 15–24 years to determine 1) whether participants felt informed about heart disease or stroke, 2) their exposure to heart disease information sources over the past year, and 3) whether they had ever discussed CVD-related topics with healthcare providers.
Over half of participants reported feeling not informed about heart disease (52%) or stroke (59%). Participants were more likely to report feeling informed if they were exposed to information from websites or social media, or if they had ever discussed family history of heart disease, personal risk for heart disease, or high blood pressure with their healthcare provider.
Most young women did not feel informed about CVD. Exposure to specific information sources and discussions with healthcare providers may help improve this.
Public health campaigns should promote cardiovascular health through websites and social media popular amongst young women. Healthcare providers should discuss CVD risk factor modification with young patients in order to promote cardiovascular health across the life course.
Ideal cardiovascular health is present in <50% of children and <1% of adults, yet its prevalence from adolescence through adulthood has not been fully evaluated. This study characterizes the ...association of age with ideal cardiovascular health and compares these associations across sex, race/ethnicity, and SES subgroups.
This study, conducted in 2020, analyzed adolescents and adults aged 12–79 years from the cross-sectional National Health and Nutrition Examination Survey 2005–2016 (N=38,706). Polynomial models were used to model the association of age with ideal cardiovascular health, defined using the American Heart Association's Life's Simple 7 criteria (scales 0–14, with higher values indicating better cardiovascular health).
Mean cardiovascular health was lower with increasing age, starting in early adolescence and dropping to a nadir by age 60 years before stabilizing. At age 20 years, only 45% of adults had ideal cardiovascular health (≥5 ideal cardiovascular health metrics), and >50% of adults had poor cardiovascular health (≤2 ideal cardiovascular health metrics) at age 53 years. Women had higher mean cardiovascular health than men in early life but lower mean cardiovascular health from age 60 years onward. Mean cardiovascular health scores were highest for non-Hispanic White and higher-income adults and lowest for non-Hispanic Black and low-income adults across all ages. Mean cardiovascular health scores fell from intermediate to poor levels approximately 30 years earlier for non-Hispanic Black than for non-Hispanic White adults and approximately 35 years earlier for low-income adults than in higher-income adults.
Cardiovascular health scores are lower with increasing age from early adolescence through adulthood. Race/ethnicity and income disparities in cardiovascular health are observed at young ages and are more profound at older ages.
Health care practitioners who care for adolescents transitioning to adulthood often face incongruent recommendations from pediatric and adult guidelines for treatment of lipid levels.
To compare the ...proportion of young people aged 17 to 21 years who meet criteria for pharmacologic treatment of elevated low-density lipoprotein cholesterol (LDL-C) levels under pediatric vs adult guidelines.
We performed a cross-sectional analysis of the National Health and Nutrition Examination Survey (NHANES) population. Surveys were administered from January 1, 1999, through December 31, 2012, and the analysis was performed from June through December 2014. Participants included 6338 individuals aged 17 to 21 years in the United States.
To estimate the number and proportion of individuals aged 17 to 21 years in the NHANES population who were eligible for statin therapy, we applied treatment algorithms from the 2011 Integrated Guidelines for Cardiovascular Health and Risk Reduction in Children and Adolescents of the National Heart, Lung, and Blood Institute and the 2013 Guideline on the Treatment of Blood Cholesterol to Reduce Atherosclerotic Cardiovascular Risk in Adults from the American College of Cardiology and American Heart Association. After imputing missing data and applying NHANES sampling weights, we extrapolated the results to 20.4 million noninstitutionalized young people aged 17 to 21 years living in the United States.
Of the 6338 young people aged 17 to 21 years in the NHANES population, 2.5% (95% CI, 1.8%-3.2%) would qualify for statin treatment under the pediatric guidelines compared with 0.4% (95% CI, 0.1%-0.8%) under the adult guidelines. Participants who met pediatric criteria had lower mean (SD) LDL-C levels (167.3 3.8 vs 210.0 7.1 mg/dL) but higher proportions of other cardiovascular risk factors, including hypertension (10.8% vs 8.4%), smoking (55.0% vs 23.9%), and obesity (67.7% vs 18.2%) compared with those who met the adult guidelines. Extrapolating to the US population of individuals aged 17 to 21 years represented by the NHANES sample, 483 500 (95% CI, 482 100-484 800) young people would be eligible for treatment of LDL-C levels if the pediatric guidelines were applied compared with only 78 200 (95% CI, 77 600-78 700) if the adult guidelines were applied.
Application of pediatric vs adult guidelines for lipid levels, which consider additional cardiovascular risk factors beyond age and LDL-C concentration, might result in statin treatment for more than 400 000 additional adolescents and young adults.
Abstract Familial hypercholesterolemia (FH) is an autosomal dominant disorder of low-density lipoprotein (LDL) metabolism leading to high LDL cholesterol (LDL-C) and accelerated atherosclerosis. The ...rare homozygous form is associated with physical examination findings and coronary heart disease during childhood. The more common heterozygous form (hetFH) is asymptomatic until adulthood, when those affected develop premature cardiovascular disease (CVD) events, often in early adulthood. Identification of hetFH is key because of the relatively high prevalence, 1 in 200 to 500, and the opportunity to lower LDL-C and reduce CVD outcomes. Selective screening based on family history can identify affected individuals, but many with hetFH are missed by relying on this strategy and go undiagnosed during childhood, leading to the recommendation by the National Heart, Lung, and Blood Institute Expert Panel for universal lipid screening between ages 9 and 11 y and again at ages 17 to 21 y. Diagnosis should lead to treatment with lifestyle modification and pharmacotherapy when appropriate because lowering LDL-C in youth has beneficial effects on subclinical atherosclerosis and likely reduces premature CVD events. This article reviews what is known about the epidemiology and pathophysiology of FH as it relates to the care of children and adolescents. Approaches to identification and treatment of FH during childhood are presented, including both recommendations from published guidelines and clinical experience. A clinical case is used to illustrate various points.
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