RESUMO
BACKGROUND: Cardiovascular disease and stroke are common and costly, and their prevalence is rising. Forecasts on the prevalence of risk factors and clinical events are crucial. METHODS: Using the 2015 to March 2020 National Health and Nutrition Examination Survey and 2015 to 2019 Medical Expenditure Panel Survey, we estimated trends in prevalence for cardiovascular risk factors based on adverse levels of Life's Essential 8 and clinical cardiovascular disease and stroke. We projected through 2050, overall and by age and race and ethnicity, accounting for changes in disease prevalence and demographics. RESULTS: We estimate that among adults, prevalence of hypertension will increase from 51.2% in 2020 to 61.0% in 2050. Diabetes (16.3% to 26.8%) and obesity (43.1% to 60.6%) will increase, whereas hypercholesterolemia will decline (45.8% to 24.0%). The prevalences of poor diet, inadequate physical activity, and smoking are estimated to improve over time, whereas inadequate sleep will worsen. Prevalences of coronary disease (7.8% to 9.2%), heart failure (2.7% to 3.8%), stroke (3.9% to 6.4%), atrial fibrillation (1.7% to 2.4%), and total cardiovascular disease (11.3% to 15.0%) will rise. Clinical CVD will affect 45 million adults, and CVD including hypertension will affect more than 184 million adults by 2050 (>61%). Similar trends are projected in children. Most adverse trends are projected to be worse among people identifying as American Indian/Alaska Native or multiracial, Black, or Hispanic. CONCLUSIONS: The prevalence of many cardiovascular risk factors and most established diseases will increase over the next 30 years. Clinical and public health interventions are needed to effectively manage, stem, and even reverse these adverse trends.
Assuntos
American Heart Association , Doenças Cardiovasculares , Previsões , Acidente Vascular Cerebral , Humanos , Estados Unidos/epidemiologia , Prevalência , Acidente Vascular Cerebral/epidemiologia , Doenças Cardiovasculares/epidemiologia , Fatores de Risco , Adulto , Feminino , Masculino , Pessoa de Meia-Idade , Idoso , Efeitos Psicossociais da Doença , Adulto JovemRESUMO
BACKGROUND: Food insecurity, a social and economic condition of limited availability of healthy food, is a risk factor for adverse cardiovascular health outcomes among adults; few studies have been conducted in adolescents. This study explores the association between food insecurity and cardiovascular health risk factors among a nationally representative sample of US adolescents, adopting the American Heart Association's Life's Essential 8 metric. METHODS AND RESULTS: We analyzed data from 2534 adolescents aged 12 to 19 years from the 2013 to 2018 National Health and Nutrition Examination Surveys. In the sample, 24.8% of adolescents lived in food-insecure households. After multivariable adjustment, food insecurity was associated with a 3.23-unit lower total Life's Essential 8 score (95% CI, -6.32, -0.15) and lower scores on diet quality (ß=-5.39 [95% CI, -8.91, -1.87]) and nicotine exposure (ß=-4.85 [95% CI, -9.24, -0.45]). Regarding diet, food insecurity was associated with 5% lower Healthy Eating Index-2015 scores [95% CI, -7%, -2%], particularly lower intakes of whole grains and seafood/plant proteins and marginally higher intake of added sugar. Regarding nicotine exposure, food insecurity was associated with ever use of a tobacco product among m (odds ratio, 1.74 [95% CI, 1.20-2.53]). Compared with their food-secure counterparts, food-insecure male (odds ratio, 1.98 [95% CI, 1.07-3.65]) and female (odds ratio, 3.22 [95% CI, 1.60-6.45]) adolescents had higher odds of living with a current indoor smoker. CONCLUSIONS: In this nationally representative sample of adolescents, food insecurity was associated with multiple indicators of cardiovascular health risk. These findings underscore the need for public health interventions and policies to reduce food insecurity and improve cardioprotective behaviors during adolescence, with particular efforts targeting diet quality and nicotine exposure.
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Abastecimento de Alimentos , Nicotina , Adulto , Humanos , Adolescente , Estados Unidos/epidemiologia , Dieta , Fatores de Risco , Inquéritos Nutricionais , Insegurança AlimentarRESUMO
Homozygous familial hypercholesterolaemia is a life-threatening genetic condition, which causes extremely elevated LDL-C levels and atherosclerotic cardiovascular disease very early in life. It is vital to start effective lipid-lowering treatment from diagnosis onwards. Even with dietary and current multimodal pharmaceutical lipid-lowering therapies, LDL-C treatment goals cannot be achieved in many children. Lipoprotein apheresis is an extracorporeal lipid-lowering treatment, which is used for decades, lowering serum LDL-C levels by more than 70% directly after the treatment. Data on the use of lipoprotein apheresis in children with homozygous familial hypercholesterolaemia mainly consists of case-reports and case-series, precluding strong evidence-based guidelines. We present a consensus statement on lipoprotein apheresis in children based on the current available evidence and opinions from experts in lipoprotein apheresis from over the world. It comprises practical statements regarding the indication, methods, treatment goals and follow-up of lipoprotein apheresis in children with homozygous familial hypercholesterolaemia and on the role of lipoprotein(a) and liver transplantation.
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Remoção de Componentes Sanguíneos , Consenso , Homozigoto , Humanos , Remoção de Componentes Sanguíneos/métodos , Criança , Resultado do Tratamento , Lipoproteína(a)/sangue , LDL-Colesterol/sangue , Adolescente , Transplante de Fígado , Biomarcadores/sangue , Hiperlipoproteinemia Tipo I/diagnóstico , Hiperlipoproteinemia Tipo I/terapia , Hiperlipoproteinemia Tipo I/sangue , Hiperlipoproteinemia Tipo I/genética , Fenótipo , Hiperlipoproteinemia Tipo II/terapia , Hiperlipoproteinemia Tipo II/sangue , Hiperlipoproteinemia Tipo II/genética , Hiperlipoproteinemia Tipo II/diagnóstico , Pré-Escolar , Lipoproteínas/sangue , Predisposição Genética para DoençaRESUMO
PURPOSE OF REVIEW: Despite familial hypercholesterolemia (FH) being the most common genetic cause of cardiovascular disease (CVD), genetic testing is rarely utilized in the US. This review summarizes what is known about the clinical utility of genetic testing and its role in the diagnosis and screening of FH. RECENT FINDINGS: The presence of an FH-causative variant is associated with a substantially higher risk of CVD, even when low-density lipoprotein cholesterol (LDL-C) levels are only modestly elevated. Genetic testing can facilitate the identification of FH cases who may be missed by clinical diagnostic criteria, improve risk stratification beyond LDL-C and family history, guide treatment decisions, and improve treatment initiation and adherence. Genetic testing can be incorporated into FH screening and diagnosis algorithms, including cascade, targeted, and universal screening. Integrating genetic testing into cascade screening can enhance the effectiveness of the process. Several models of universal FH screening with coordinated genetic and lipid testing are feasible and effective. SUMMARY: More systematic integration of genetic testing into FH diagnosis and screening can significantly reduce the burden of this condition through early detection and treatment. Further pragmatic implementation studies are needed to determine how to more effectively and affordably integrate genetic testing into clinical lipid screening programs.
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Doenças Cardiovasculares , Hiperlipoproteinemia Tipo II , Humanos , LDL-Colesterol/genética , Testes Genéticos , Hiperlipoproteinemia Tipo II/diagnóstico , Hiperlipoproteinemia Tipo II/genética , Hiperlipoproteinemia Tipo II/tratamento farmacológico , Doenças Cardiovasculares/diagnóstico , Doenças Cardiovasculares/genética , Programas de RastreamentoRESUMO
Importance: Familial hypercholesterolemia (FH) is a genetic disorder that often results in severely high low-density lipoprotein cholesterol (LDL-C) and high risk of premature coronary heart disease (CHD). However, the impact of FH variants on CHD risk among individuals with moderately elevated LDL-C is not well quantified. Objective: To assess CHD risk associated with FH variants among individuals with moderately (130-189 mg/dL) and severely (≥190 mg/dL) elevated LDL-C and to quantify excess CHD deaths attributable to FH variants in US adults. Design, Setting, and Participants: A total of 21â¯426 individuals without preexisting CHD from 6 US cohort studies (Atherosclerosis Risk in Communities study, Coronary Artery Risk Development in Young Adults study, Cardiovascular Health Study, Framingham Heart Study Offspring cohort, Jackson Heart Study, and Multi-Ethnic Study of Atherosclerosis) were included, 63 of whom had an FH variant. Data were collected from 1971 to 2018, and the median (IQR) follow-up was 18 (13-28) years. Data were analyzed from March to May 2023. Exposures: LDL-C, cumulative past LDL-C, FH variant status. Main Outcomes and Measures: Cox proportional hazards models estimated associations between FH variants and incident CHD. The Cardiovascular Disease Policy Model projected excess CHD deaths associated with FH variants in US adults. Results: Of the 21â¯426 individuals without preexisting CHD (mean [SD] age 52.1 [15.5] years; 12â¯041 [56.2%] female), an FH variant was found in 22 individuals with moderately elevated LDL-C (0.3%) and in 33 individuals with severely elevated LDL-C (2.5%). The adjusted hazard ratios for incident CHD comparing those with and without FH variants were 2.9 (95% CI, 1.4-6.0) and 2.6 (95% CI, 1.4-4.9) among individuals with moderately and severely elevated LDL-C, respectively. The association between FH variants and CHD was slightly attenuated when further adjusting for baseline LDL-C level, whereas the association was no longer statistically significant after adjusting for cumulative past LDL-C exposure. Among US adults 20 years and older with no history of CHD and LDL-C 130 mg/dL or higher, more than 417â¯000 carry an FH variant and were projected to experience more than 12â¯000 excess CHD deaths in those with moderately elevated LDL-C and 15â¯000 in those with severely elevated LDL-C compared with individuals without an FH variant. Conclusions and Relevance: In this pooled cohort study, the presence of FH variants was associated with a 2-fold higher CHD risk, even when LDL-C was only moderately elevated. The increased CHD risk appeared to be largely explained by the higher cumulative LDL-C exposure in individuals with an FH variant compared to those without. Further research is needed to assess the value of adding genetic testing to traditional phenotypic FH screening.
Assuntos
Aterosclerose , Doenças Cardiovasculares , Doença da Artéria Coronariana , Hipercolesterolemia , Hiperlipoproteinemia Tipo II , Adulto Jovem , Humanos , Feminino , Pessoa de Meia-Idade , Masculino , Hipercolesterolemia/complicações , LDL-Colesterol/genética , Doenças Cardiovasculares/prevenção & controle , Estudos de Coortes , Fatores de Risco , Hiperlipoproteinemia Tipo II/diagnóstico , Doença da Artéria Coronariana/complicações , Aterosclerose/complicações , Fatores de Risco de Doenças CardíacasRESUMO
This contemporary, international, evidence-informed guidance aims to achieve the greatest good for the greatest number of people with familial hypercholesterolaemia (FH) across different countries. FH, a family of monogenic defects in the hepatic LDL clearance pathway, is a preventable cause of premature coronary artery disease and death. Worldwide, 35 million people have FH, but most remain undiagnosed or undertreated. Current FH care is guided by a useful and diverse group of evidence-based guidelines, with some primarily directed at cholesterol management and some that are country-specific. However, none of these guidelines provides a comprehensive overview of FH care that includes both the lifelong components of clinical practice and strategies for implementation. Therefore, a group of international experts systematically developed this guidance to compile clinical strategies from existing evidence-based guidelines for the detection (screening, diagnosis, genetic testing and counselling) and management (risk stratification, treatment of adults or children with heterozygous or homozygous FH, therapy during pregnancy and use of apheresis) of patients with FH, update evidence-informed clinical recommendations, and develop and integrate consensus-based implementation strategies at the patient, provider and health-care system levels, with the aim of maximizing the potential benefit for at-risk patients and their families worldwide.
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Aterosclerose , Hiperlipoproteinemia Tipo II , Adulto , Criança , Feminino , Gravidez , Humanos , Hiperlipoproteinemia Tipo II/diagnóstico , Hiperlipoproteinemia Tipo II/genética , Hiperlipoproteinemia Tipo II/terapia , Aterosclerose/diagnóstico , Aterosclerose/genética , Aterosclerose/terapia , Testes Genéticos , ColesterolAssuntos
Doença das Coronárias , Hipercolesterolemia , Hiperlipoproteinemia Tipo II , Humanos , Hipercolesterolemia/epidemiologia , Hipercolesterolemia/genética , Hiperlipoproteinemia Tipo II/complicações , Hiperlipoproteinemia Tipo II/diagnóstico , Hiperlipoproteinemia Tipo II/epidemiologia , Genótipo , Doença das Coronárias/epidemiologia , Doença das Coronárias/genéticaRESUMO
Background Homozygous familial hypercholesterolemia (HoFH) is a rare, treatment-resistant disorder characterized by early-onset atherosclerotic and aortic valvular cardiovascular disease if left untreated. Contemporary information on HoFH in the United States is lacking, and the extent of underdiagnosis and undertreatment is uncertain. Methods and Results Data were analyzed from 67 children and adults with clinically diagnosed HoFH from the CASCADE (Cascade Screening for Awareness and Detection) FH Registry. Genetic diagnosis was confirmed in 43 patients. We used the clinical characteristics of genetically confirmed patients with HoFH to query the Family Heart Database, a US anonymized payer health database, to estimate the number of patients with similar lipid profiles in a "real-world" setting. Untreated low-density lipoprotein cholesterol levels were lower in adults than children (533 versus 776 mg/dL; P=0.001). At enrollment, atherosclerotic cardiovascular disease and supravalvular and aortic valve stenosis were present in 78.4% and 43.8% and 25.5% and 18.8% of adults and children, respectively. At most recent follow-up, despite multiple lipid-lowering treatment, low-density lipoprotein cholesterol goals were achieved in only a minority of adults and children. Query of the Family Heart Database identified 277 individuals with profiles similar to patients with genetically confirmed HoFH. Advanced lipid-lowering treatments were prescribed for 18%; 40% were on no lipid-lowering treatment; atherosclerotic cardiovascular disease was reported in 20%; familial hypercholesterolemia diagnosis was uncommon. Conclusions Only patients with the most severe HoFH phenotypes are diagnosed early. HoFH remains challenging to treat. Results from the Family Heart Database indicate HoFH is systemically underdiagnosed and undertreated. Earlier screening, aggressive lipid-lowering treatments, and guideline implementation are required to reduce disease burden in HoFH.
Assuntos
Anticolesterolemiantes , Aterosclerose , Doenças Cardiovasculares , Hipercolesterolemia Familiar Homozigota , Hiperlipoproteinemia Tipo II , Estados Unidos/epidemiologia , Humanos , Doenças Cardiovasculares/tratamento farmacológico , Hiperlipoproteinemia Tipo II/diagnóstico , Hiperlipoproteinemia Tipo II/epidemiologia , Hiperlipoproteinemia Tipo II/genética , LDL-Colesterol , Aterosclerose/diagnóstico , Aterosclerose/epidemiologia , Aterosclerose/genética , Sistema de Registros , Anticolesterolemiantes/uso terapêutico , HomozigotoRESUMO
Background Heterozygous familial hypercholesterolemia (FH) is a common genetic disorder causing premature cardiovascular disease. Despite this, there is no national screening program in the United States to identify individuals with FH or likely pathogenic FH genetic variants. Methods and Results The clinical characteristics and FH variant status of 49 738 UK Biobank participants were used to develop a regression model to predict the probability of having any FH variants. The regression model and modified Dutch Lipid Clinic Network criteria were applied to 39 790 adult participants (aged ≥20 years) in the National Health and Nutrition Examination Survey to estimate the yield of FH screening programs using Dutch Lipid Clinic Network clinical criteria alone (excluding genetic variant status), genetic testing alone, or combining clinical criteria with genetic testing. The regression model accurately predicted FH variant status in UK Biobank participants (observed prevalence, 0.27%; predicted, 0.26%; area under the receiver-operator characteristic curve, 0.88). In the National Health and Nutrition Examination Survey, the estimated yield per 1000 individuals screened (95% CI) was 3.7 (3.0-4.6) FH cases with the Dutch Lipid Clinic Network clinical criteria alone, 3.8 (2.7-5.1) cases with genetic testing alone, and 6.6 (5.3-8.0) cases by combining clinical criteria with genetic testing. In young adults aged 20 to 39 years, using clinical criteria alone was estimated to yield 1.3 (95% CI, 0.6-2.5) FH cases per 1000 individuals screened, which was estimated to increase to 4.2 (95% CI, 2.6-6.4) FH cases when combining clinical criteria with genetic testing. Conclusions Screening for FH using a combination of clinical criteria with genetic testing may increase identification and the opportunity for early treatment of individuals with FH.
Assuntos
Hiperlipoproteinemia Tipo II , Testes Genéticos , Humanos , Hiperlipoproteinemia Tipo II/diagnóstico , Hiperlipoproteinemia Tipo II/epidemiologia , Hiperlipoproteinemia Tipo II/genética , Lipídeos , Programas de Rastreamento/métodos , Inquéritos Nutricionais , Adulto JovemRESUMO
At a population level, engagement in healthy lifestyle behaviors is suboptimal in the United States. Moreover, marked disparities exist in healthy lifestyle behaviors and cardiovascular risk factors as a result of social determinants of health. In addition, there are specific challenges to engaging in healthy lifestyle behaviors related to age, developmental stage, or major life circumstances. Key components of a healthy lifestyle are consuming a healthy dietary pattern, engaging in regular physical activity, avoiding use of tobacco products, habitually attaining adequate sleep, and managing stress. For these health behaviors, there are guidelines and recommendations; however, promotion in clinical settings can be challenging, particularly in certain population groups. These challenges must be overcome to facilitate greater promotion of healthy lifestyle practices in clinical settings. The 5A Model (assess, advise, agree, assist, and arrange) was developed to provide a framework for clinical counseling with consideration for the demands of clinical settings. In this science advisory, we summarize specific considerations for lifestyle-related behavior change counseling using the 5A Model for patients across the life span. In all life stages, social determinants of health and unmet social-related health needs, as well as overweight and obesity, are associated with increased risk of cardiovascular disease, and there is the potential to modify this risk with lifestyle-related behavior changes. In addition, specific considerations for lifestyle-related behavior change counseling in life stages in which lifestyle behaviors significantly affect cardiovascular disease risk are outlined. Greater attention to healthy lifestyle behaviors during every clinician visit will contribute to improved cardiovascular health.
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Doenças Cardiovasculares , Comportamentos Relacionados com a Saúde , Promoção da Saúde , Estilo de Vida Saudável , Motivação , American Heart Association , Doenças Cardiovasculares/epidemiologia , Doenças Cardiovasculares/prevenção & controle , Humanos , Estados Unidos/epidemiologiaRESUMO
OBJECTIVE: To describe enrollment characteristics of youth in the Cascade Screening for Awareness and Detection of FH Registry. STUDY DESIGN: This is a cross-sectional analysis of 493 participants aged <18 years with heterozygous familial hypercholesterolemia recruited from US lipid clinics (n = 20) between April 1, 2014, and January 12, 2018. At enrollment, some were new patients and some were already in care. Clinical characteristics are described, including lipid levels and lipid-lowering treatments. RESULTS: Mean age at diagnosis was 9.4 (4.0) years; 47% female, 68% white and 12% Hispanic. Average (SD) highest Low-density lipoprotein cholesterol (LDL-C) was 238 (61) mg/dL before treatment. Lipid-lowering therapy was used by 64% of participants; 56% were treated with statin. LDL-C declined 84 mg/dL (33%) among those treated with lipid-lowering therapy; statins produced the greatest decline, 100 mg/dL (39% reduction). At enrollment, 39% had reached an LDL-C goal, either <130 mg/dL or ≥50% decrease from pre-treatment; 20% of those on lipid-lowering therapy reached both goals. CONCLUSIONS: Among youth enrolled in the Cascade Screening for Awareness and Detection of FH Registry, diagnosis occurred relatively late, only 77% of children eligible for lipid-lowering therapy were receiving treatment, and only 39% of those treated met their LDL-C goal. Opportunities exist for earlier diagnosis, broader use of lipid-lowering therapy, and greater reduction of LDL-C levels.
Assuntos
Hiperlipoproteinemia Tipo II/epidemiologia , Hiperlipoproteinemia Tipo II/terapia , Adolescente , Anticolesterolemiantes/uso terapêutico , Criança , LDL-Colesterol/sangue , Doença da Artéria Coronariana/prevenção & controle , Estudos Transversais , Suplementos Nutricionais , Uso de Medicamentos/estatística & dados numéricos , Feminino , Humanos , Inibidores de Hidroximetilglutaril-CoA Redutases/uso terapêutico , Hiperlipoproteinemia Tipo II/sangue , Estilo de Vida , Masculino , Sistema de Registros , Estados Unidos/epidemiologiaRESUMO
Although progress had been made in reducing cardiovascular disease (CVD) mortality, the positive trend has reversed in recent years, and CVD remains the most common cause of mortality in US women and men. Youth represent the future of CVD prevention; emerging evidence suggests exposure to risk factors in children contributes to atherosclerosis and results in vascular changes and increased CVD events. The contributors to CVD include those commonly seen in adults. This article reviews hypercholesterolemia, hypertension, obesity, diabetes, and smoking. It discusses the prevalence of each disease, diagnosis, treatment, and cardiovascular complications.
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Cardiologia/métodos , Doenças Cardiovasculares/prevenção & controle , Gerenciamento Clínico , Medicina Preventiva/métodos , Criança , HumanosRESUMO
BACKGROUND: The substantial risk of thrombosis in large coronary artery aneurysms (CAAs) (maximum z-score ≥ 10) after Kawasaki disease (KD) mandates effective thromboprophylaxis. We sought to determine the effectiveness of anticoagulation (low-molecular-weight heparin [LMWH] or warfarin) for thromboprophylaxis in large CAAs. METHODS: Data from 383 patients enrolled in the International KD Registry (IKDR) were used. Time-to-event analysis was used to account for differences in treatment duration and follow-up. RESULTS: From diagnosis onward (96% received acetylsalicylic acid concomitantly), 114 patients received LMWH (median duration 6.2 months, interquartile range [IQR] 2.5-12.7), 80 warfarin (median duration 2.2 years, IQR 0.9-7.1), and 189 no anticoagulation. Cumulative incidence of coronary artery thrombosis with LMWH was 5.7 ± 3.0%, with warfarin 6.7 ± 3.7%, and with no anticoagulation 20.6 ± 3.0% (P < 0.001) at 2.5 years after the start of thromboprophylaxis (LMWH vs warfarin HR 1.5, 95% confidence interval [CI] 0.4-5.1; P = 0.56). A total of 51/63 patients with coronary artery thrombosis received secondary thromboprophylaxis (ie, thromboprophylaxis after a previous thrombus): 27 LMWH, 24 warfarin. There were no differences in incidence of further coronary artery thrombosis between strategies (HR 2.9, 95% CI 0.6-13.5; P = 0.19). Severe bleeding complications were generally rare (1.6 events per 100 patient-years) and were noted equally for patients on LMWH and warfarin (HR 2.3, 95% CI 0.6-8.9; P = 0.25). CONCLUSIONS: LMWH and warfarin appear to have equivalent effectiveness for preventing thrombosis in large CAAs after KD, although event rates for secondary thromboprophylaxis and safety outcomes were low. Based on our findings, all patients with CAA z-score ≥ 10 should receive anticoagulation, but the choice of agent might be informed by secondary risk factors and patient preferences.
Assuntos
Quimioprevenção , Aneurisma Coronário , Heparina de Baixo Peso Molecular , Síndrome de Linfonodos Mucocutâneos , Trombose , Varfarina , Anticoagulantes/administração & dosagem , Anticoagulantes/efeitos adversos , Canadá/epidemiologia , Quimioprevenção/métodos , Quimioprevenção/estatística & dados numéricos , Pré-Escolar , Aneurisma Coronário/complicações , Aneurisma Coronário/tratamento farmacológico , Feminino , Hemorragia/induzido quimicamente , Hemorragia/epidemiologia , Heparina de Baixo Peso Molecular/administração & dosagem , Heparina de Baixo Peso Molecular/efeitos adversos , Humanos , Incidência , Masculino , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/epidemiologia , Sistema de Registros/estatística & dados numéricos , Risco Ajustado , Trombose/epidemiologia , Trombose/etiologia , Trombose/prevenção & controle , Estados Unidos/epidemiologia , Varfarina/administração & dosagem , Varfarina/efeitos adversosRESUMO
BACKGROUNDPediatric SARS-CoV-2 infection can be complicated by a dangerous hyperinflammatory condition termed multisystem inflammatory syndrome in children (MIS-C). The clinical and immunologic spectrum of MIS-C and its relationship to other inflammatory conditions of childhood have not been studied in detail.METHODSWe retrospectively studied confirmed cases of MIS-C at our institution from March to June 2020. The clinical characteristics, laboratory studies, and treatment response were collected. Data were compared with historic cohorts of Kawasaki disease (KD) and macrophage activation syndrome (MAS).RESULTSTwenty-eight patients fulfilled the case definition of MIS-C. Median age at presentation was 9 years (range: 1 month to 17 years); 50% of patients had preexisting conditions. All patients had laboratory confirmation of SARS-CoV-2 infection. Seventeen patients (61%) required intensive care, including 7 patients (25%) who required inotrope support. Seven patients (25%) met criteria for complete or incomplete KD, and coronary abnormalities were found in 6 cases. Lymphopenia, thrombocytopenia, and elevation in inflammatory markers, D-dimer, B-type natriuretic peptide, IL-6, and IL-10 levels were common but not ubiquitous. Cytopenias distinguished MIS-C from KD and the degree of hyperferritinemia and pattern of cytokine production differed between MIS-C and MAS. Immunomodulatory therapy given to patients with MIS-C included intravenous immune globulin (IVIG) (71%), corticosteroids (61%), and anakinra (18%). Clinical and laboratory improvement were observed in all cases, including 6 cases that did not require immunomodulatory therapy. No mortality was recorded in this cohort.CONCLUSIONMIS-C encompasses a broad phenotypic spectrum with clinical and laboratory features distinct from KD and MAS.FUNDINGThis work was supported by the National Institutes of Health, National Institute of Arthritis and Musculoskeletal and Skin Diseases; the National Institute of Allergy and Infectious Diseases; Rheumatology Research Foundation Investigator Awards and Medical Education Award; Boston Children's Hospital Faculty Career Development Awards; the McCance Family Foundation; and the Samara Jan Turkel Center.
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Corticosteroides/administração & dosagem , Betacoronavirus/metabolismo , Imunoglobulinas Intravenosas/administração & dosagem , Imunomodulação , Proteína Antagonista do Receptor de Interleucina 1/administração & dosagem , Síndrome de Resposta Inflamatória Sistêmica , Adolescente , Biomarcadores/sangue , COVID-19 , Criança , Pré-Escolar , Feminino , Produtos de Degradação da Fibrina e do Fibrinogênio/metabolismo , Humanos , Lactente , Interleucina-10/sangue , Interleucina-6/sangue , Síndrome de Ativação Macrofágica/sangue , Síndrome de Ativação Macrofágica/diagnóstico , Síndrome de Ativação Macrofágica/tratamento farmacológico , Síndrome de Ativação Macrofágica/imunologia , Masculino , Síndrome de Linfonodos Mucocutâneos/sangue , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Síndrome de Linfonodos Mucocutâneos/tratamento farmacológico , Síndrome de Linfonodos Mucocutâneos/imunologia , Peptídeo Natriurético Encefálico/sangue , Estudos Retrospectivos , SARS-CoV-2 , Síndrome de Resposta Inflamatória Sistêmica/sangue , Síndrome de Resposta Inflamatória Sistêmica/diagnóstico , Síndrome de Resposta Inflamatória Sistêmica/tratamento farmacológico , Síndrome de Resposta Inflamatória Sistêmica/imunologiaRESUMO
The prevention and management of cardiovascular risk factors during the transition from childhood to adulthood is critically important in defining cardiovascular health trajectories. Unfortunately, many young people fall out of clinical care during this important time, leading to worsening cardiovascular risk and missed opportunities to modify future outcomes. The field of health care transition has evolved to support young people with complex health needs in developing self-management and self-advocacy skills to promote positive health outcomes despite changes in health care providers and resources. While transitional care efforts are largely focused on childhood-onset chronic illnesses such as sickle cell disease and cystic fibrosis, young people with cardiovascular risk factors such as hypertension, obesity, and dyslipidemia also stand to benefit from structured supports to ensure continuity in care and positive health behaviours. On the backdrop of the broader health care transition literature, we offer practical insights and suggestions for ensuring that young people with cardiovascular risk factors experience uninterrupted high-quality care and support as they enter the adult health care system. Starting transition preparation in early adolescence, actively engaging all key stakeholders throughout the process, and remaining mindful of the developmental underpinnings and social context of transition are keys to success.
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Doenças Cardiovasculares/prevenção & controle , Comportamentos Relacionados com a Saúde , Transferência de Pacientes/métodos , Transição para Assistência do Adulto/organização & administração , Adulto , Doenças Cardiovasculares/psicologia , Criança , Humanos , Fatores de RiscoRESUMO
The pediatric lipid screening and treatment practices, attitudes, and perceived barriers of Canadian pediatricians are not known. We sought to evaluate this in a survey of pediatricians through the Canadian Pediatric Surveillance Program (CPSP) in March 2019. The survey included an assessment of lipid screening of 9- to 11-year-old youth and a hypothetical case of persistent severe dyslipidemia to ascertain management practices. There were 759 respondents (28% response rate, 759 of 2742), of whom 236 provided outpatient primary care to 9- to 11-year-old youth as part of their routine clinical practice. Among primary care-providing pediatricians, universal lipid screening of healthy 9- to 11-year-old youth most or all of the time was reported by 3% (8 of 230). Reported screening practices most or all of the time were more common for youth with risk factors such as overweight and obesity (54%, 127 of 235) and a family history of premature cardiovascular disease (39%, 85 of 217). Most respondents would refer a child with severe persistent dyslipidemia to dieticians (69%, 152 of 220) and a lipid specialist (64%, 144 of 220) most or all of the time, whereas 7% (16 of 220) would start statin therapy themselves. A lack of Canadian pediatric lipid guidelines was reported as a major barrier for 49% (114 of 233) and minor barrier for 40% (93 of 213). The rate of routine lipid screening of healthy 9- to 11-year-old youth among Canadian primary care-providing pediatricians is low and at odds with current US guidelines. This discrepancy may be due at least in part to a lack of Canadian guidelines on pediatric dyslipidemia, the development of which may address certain perceived barriers and influence future attitudes.