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  1. Article ; Online: Contemporary Homozygous Familial Hypercholesterolemia in the United States: Insights From the CASCADE FH Registry.

    Cuchel, Marina / Lee, Paul C / Hudgins, Lisa C / Duell, P Barton / Ahmad, Zahid / Baum, Seth J / Linton, MacRae F / de Ferranti, Sarah D / Ballantyne, Christie M / Larry, John A / Hemphill, Linda C / Kindt, Iris / Gidding, Samuel S / Martin, Seth S / Moriarty, Patrick M / Thompson, Paul P / Underberg, James A / Guyton, John R / Andersen, Rolf L /
    Whellan, David J / Benuck, Irwin / Kane, John P / Myers, Kelly / Howard, William / Staszak, David / Jamison, Allison / Card, Mary C / Bourbon, Mafalda / Chora, Joana R / Rader, Daniel J / Knowles, Joshua W / Wilemon, Katherine / McGowan, Mary P

    Journal of the American Heart Association

    2023  Volume 12, Issue 9, Page(s) e029175

    Abstract: 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 ... ...

    Abstract 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;
    MeSH term(s) United States/epidemiology ; Humans ; Homozygous Familial Hypercholesterolemia ; Cardiovascular Diseases/drug therapy ; Hyperlipoproteinemia Type II/diagnosis ; Hyperlipoproteinemia Type II/epidemiology ; Hyperlipoproteinemia Type II/genetics ; Cholesterol, LDL ; Atherosclerosis/diagnosis ; Atherosclerosis/epidemiology ; Atherosclerosis/genetics ; Registries ; Anticholesteremic Agents/therapeutic use ; Homozygote
    Chemical Substances Cholesterol, LDL ; Anticholesteremic Agents
    Language English
    Publishing date 2023-04-29
    Publishing country England
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
    ZDB-ID 2653953-6
    ISSN 2047-9980 ; 2047-9980
    ISSN (online) 2047-9980
    ISSN 2047-9980
    DOI 10.1161/JAHA.122.029175
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Longitudinal low density lipoprotein cholesterol goal achievement and cardiovascular outcomes among adult patients with familial hypercholesterolemia: The CASCADE FH registry.

    Duell, P Barton / Gidding, Samuel S / Andersen, Rolf L / Knickelbine, Thomas / Anderson, Lars / Gianos, Eugenia / Shrader, Peter / Kindt, Iris / O'Brien, Emily C / McCann, Dervilla / Hemphill, Linda C / Ahmed, Catherine D / Martin, Seth S / Larry, John A / Ahmad, Zahid S / Kullo, Iftikhar J / Underberg, James A / Guyton, John / Thompson, Paul /
    Wilemon, Katherine / Roe, Matthew T / Rader, Daniel J / Cuchel, Marina / Linton, MacRae F / Shapiro, Michael D / Moriarty, Patrick M / Knowles, Joshua W

    Atherosclerosis

    2019  Volume 289, Page(s) 85–93

    Abstract: Background and aims: There are limited data from the US on outcomes of patients in specialty care for familial hypercholesterolemia (FH).: Methods: CASCADE FH Registry data were analyzed to assess longitudinal changes in medication usage, in low ... ...

    Abstract Background and aims: There are limited data from the US on outcomes of patients in specialty care for familial hypercholesterolemia (FH).
    Methods: CASCADE FH Registry data were analyzed to assess longitudinal changes in medication usage, in low density lipoprotein cholesterol (LDL-C) levels, and the rate of major adverse cardiovascular events (MACE (myocardial infarction, coronary revascularization, stroke or transient ischemic attack) in adults with FH followed in US specialty clinics.
    Results: The cohort consisted of 1900 individuals (61% women, 87% Caucasian), with mean age of 56 ± 15 years, 37% prevalence of ASCVD at enrollment, mean pretreatment LDL-C 249 ± 68 mg/dl, mean enrollment LDL-C 145 mg/dl and 93% taking lipid lowering therapy. Over follow up of 20 ± 11 months, lipid lowering therapy use increased (mean decrease in LDL-C of 32 mg/dl (p < 0.001)). Only 48% of participants achieved LDL-C < 100 mg/dl and 22% achieved LDL-C < 70 mg/dl; ASCVD at enrollment was associated with greater likelihood of goal achievement. MACE event rates were almost 6 times higher among patients with prior ASCVD compared to those without (4.6 vs 0.8/100 patient years). Also associated with incident MACE were markers of FH severity and conventional ASCVD risk factors.
    Conclusions: With care in FH specialized clinics, LDL-C decreased, but LDL-C persisted >100 mg/dl in 52% of patients. High ASCVD event rates suggest that adults with FH warrant designation as having an ASCVD risk equivalent. Earlier and more aggressive therapy of FH is needed to prevent ASCVD events.
    MeSH term(s) Adult ; Aged ; Atherosclerosis/blood ; Atherosclerosis/prevention & control ; Cardiology/standards ; Cardiovascular Diseases/blood ; Cardiovascular Diseases/prevention & control ; Cholesterol, LDL/blood ; Female ; Follow-Up Studies ; Heterozygote ; Humans ; Hyperlipoproteinemia Type II/blood ; Hyperlipoproteinemia Type II/genetics ; Hyperlipoproteinemia Type II/therapy ; Longitudinal Studies ; Male ; Middle Aged ; Registries ; Risk Factors ; Treatment Outcome
    Chemical Substances Cholesterol, LDL
    Language English
    Publishing date 2019-08-19
    Publishing country Ireland
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 80061-2
    ISSN 1879-1484 ; 0021-9150
    ISSN (online) 1879-1484
    ISSN 0021-9150
    DOI 10.1016/j.atherosclerosis.2019.08.007
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 226: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)

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  3. Article ; Online: Health disparities among adult patients with a phenotypic diagnosis of familial hypercholesterolemia in the CASCADE-FH™ patient registry.

    Amrock, Stephen M / Duell, P Barton / Knickelbine, Thomas / Martin, Seth S / O'Brien, Emily C / Watson, Karol E / Mitri, Joanna / Kindt, Iris / Shrader, Peter / Baum, Seth J / Hemphill, Linda C / Ahmed, Catherine D / Andersen, Rolf L / Kullo, Iftikhar J / McCann, Dervilla / Larry, John A / Murray, Michael F / Fishberg, Robert / Guyton, John R /
    Wilemon, Katherine / Roe, Matthew T / Rader, Daniel J / Ballantyne, Christie M / Underberg, James A / Thompson, Paul / Duffy, Dannielle / Linton, MacRae F / Shapiro, Michael D / Moriarty, Patrick M / Knowles, Joshua W / Ahmad, Zahid S

    Atherosclerosis

    2017  Volume 267, Page(s) 19–26

    Abstract: Background and aims: Most familial hypercholesterolemia (FH) patients remain undertreated, and it is unclear what role health disparities may play for FH patients in the US. We sought to describe sex and racial/ethnic disparities in a national registry ... ...

    Abstract Background and aims: Most familial hypercholesterolemia (FH) patients remain undertreated, and it is unclear what role health disparities may play for FH patients in the US. We sought to describe sex and racial/ethnic disparities in a national registry of US FH patients.
    Methods: We analyzed data from 3167 adults enrolled in the CAscade SCreening for Awareness and DEtection of Familial Hypercholesterolemia (CASCADE-FH) registry. Logistic regression was used to evaluate for disparities in LDL-C goals and statin use, with adjustments for covariates including age, cardiovascular risk factors, and statin intolerance.
    Results: In adjusted analyses, women were less likely than men to achieve treated LDL-C of <100 mg/dL (OR 0.68, 95% CI, 0.57-0.82) or ≥50% reduction from pretreatment LDL-C (OR 0.79, 95% CI, 0.65-0.96). Women were less likely than men to receive statin therapy (OR, 0.60, 95% CI, 0.50-0.73) and less likely to receive a high-intensity statin (OR, 0.60, 95% CI, 0.49-0.72). LDL-C goal achievement also varied by race/ethnicity: compared with whites, Asians and blacks were less likely to achieve LDL-C levels <100 mg/dL (Asians, OR, 0.47, 95% CI, 0.24-0.94; blacks, OR, 0.49, 95% CI, 0.32-0.74) or ≥50% reduction from pretreatment LDL-C (Asians, OR 0.56, 95% CI, 0.32-0.98; blacks, OR 0.62, 95% CI, 0.43-0.90).
    Conclusions: In a contemporary US population of FH patients, we identified differences in LDL-C goal attainment and statin usage after stratifying the population by either sex or race/ethnicity. Our findings suggest that health disparities contribute to the undertreatment of US FH patients. Increased efforts are warranted to raise awareness of these disparities.
    MeSH term(s) Adult ; African Americans ; Aged ; Asian Americans ; Cardiovascular Diseases/metabolism ; Cholesterol, HDL/metabolism ; Cholesterol, LDL/blood ; Ethnic Groups ; Female ; Health Status Disparities ; Healthcare Disparities ; Humans ; Hydroxymethylglutaryl-CoA Reductase Inhibitors/therapeutic use ; Hyperlipoproteinemia Type II/blood ; Hyperlipoproteinemia Type II/diagnosis ; Hyperlipoproteinemia Type II/ethnology ; Male ; Middle Aged ; Multicenter Studies as Topic ; Odds Ratio ; Phenotype ; Prospective Studies ; Registries ; Retrospective Studies ; Risk Factors ; Sex Factors
    Chemical Substances Cholesterol, HDL ; Cholesterol, LDL ; Hydroxymethylglutaryl-CoA Reductase Inhibitors
    Language English
    Publishing date 2017-10-06
    Publishing country Ireland
    Document type Journal Article
    ZDB-ID 80061-2
    ISSN 1879-1484 ; 0021-9150
    ISSN (online) 1879-1484
    ISSN 0021-9150
    DOI 10.1016/j.atherosclerosis.2017.10.006
    Database MEDical Literature Analysis and Retrieval System OnLINE

    Full text online

    More links

    Kategorien

    In stock of ZB MED Cologne/Königswinter

    Zs.A 226: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

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