Children with Heterozygous Familial Hypercholesterolemia in the United States: Data from the Cascade Screening for Awareness and Detection-FH Registry
To describe enrollment characteristics of youth in the Cascade Screening for Awareness and Detection of FH Registry. This is a cross-sectional analysis of 493 participants aged
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| Veröffentlicht in: | The Journal of pediatrics 2021-02, Vol.229, p.70-77 |
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| creator | de Ferranti, Sarah D. Shrader, Peter Linton, MacRae F. Knowles, Joshua W. Hudgins, Lisa C. Benuck, Irwin Kindt, Iris O'Brien, Emily C. Peterson, Amy L. Ahmad, Zahid S. Clauss, Sarah Duell, P. Barton Shapiro, Michael D. Wilemon, Katherine Gidding, Samuel S. Neal, William |
| description | To describe enrollment characteristics of youth in the Cascade Screening for Awareness and Detection of FH Registry.
This is a cross-sectional analysis of 493 participants aged |
| doi_str_mv | 10.1016/j.jpeds.2020.09.042 |
| format | Article |
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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.
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.
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.</description><identifier>ISSN: 0022-3476</identifier><identifier>EISSN: 1097-6833</identifier><identifier>DOI: 10.1016/j.jpeds.2020.09.042</identifier><identifier>PMID: 32976895</identifier><language>eng</language><publisher>NEW YORK: Elsevier Inc</publisher><subject>Adolescent ; Anticholesteremic Agents - therapeutic use ; Child ; cholesterol ; Cholesterol, LDL - blood ; Coronary Artery Disease - prevention & control ; Cross-Sectional Studies ; Dietary Supplements ; Drug Utilization - statistics & numerical data ; Female ; Humans ; Hydroxymethylglutaryl-CoA Reductase Inhibitors - therapeutic use ; Hyperlipoproteinemia Type II - blood ; Hyperlipoproteinemia Type II - epidemiology ; Hyperlipoproteinemia Type II - therapy ; Life Sciences & Biomedicine ; Life Style ; Male ; Pediatrics ; Registries ; Science & Technology ; statin ; United States - epidemiology</subject><ispartof>The Journal of pediatrics, 2021-02, Vol.229, p.70-77</ispartof><rights>2020 Elsevier Inc.</rights><rights>Copyright © 2020 Elsevier Inc. All rights reserved.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>true</woscitedreferencessubscribed><woscitedreferencescount>12</woscitedreferencescount><woscitedreferencesoriginalsourcerecordid>wos000610242700017</woscitedreferencesoriginalsourcerecordid><citedby>FETCH-LOGICAL-c359t-4eff91ac6e39ac8f2b662a1bc7964b3aa2791f4de3cee9ccb06810e4cbd245ef3</citedby><cites>FETCH-LOGICAL-c359t-4eff91ac6e39ac8f2b662a1bc7964b3aa2791f4de3cee9ccb06810e4cbd245ef3</cites><orcidid>0000-0002-0208-5154 ; 0000-0003-1922-7240 ; 0000-0002-9071-3287</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/j.jpeds.2020.09.042$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>315,782,786,3554,27933,27934,39267,46004</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/32976895$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>de Ferranti, Sarah D.</creatorcontrib><creatorcontrib>Shrader, Peter</creatorcontrib><creatorcontrib>Linton, MacRae F.</creatorcontrib><creatorcontrib>Knowles, Joshua W.</creatorcontrib><creatorcontrib>Hudgins, Lisa C.</creatorcontrib><creatorcontrib>Benuck, Irwin</creatorcontrib><creatorcontrib>Kindt, Iris</creatorcontrib><creatorcontrib>O'Brien, Emily C.</creatorcontrib><creatorcontrib>Peterson, Amy L.</creatorcontrib><creatorcontrib>Ahmad, Zahid S.</creatorcontrib><creatorcontrib>Clauss, Sarah</creatorcontrib><creatorcontrib>Duell, P. Barton</creatorcontrib><creatorcontrib>Shapiro, Michael D.</creatorcontrib><creatorcontrib>Wilemon, Katherine</creatorcontrib><creatorcontrib>Gidding, Samuel S.</creatorcontrib><creatorcontrib>Neal, William</creatorcontrib><title>Children with Heterozygous Familial Hypercholesterolemia in the United States: Data from the Cascade Screening for Awareness and Detection-FH Registry</title><title>The Journal of pediatrics</title><addtitle>J PEDIATR-US</addtitle><addtitle>J Pediatr</addtitle><description>To describe enrollment characteristics of youth in the Cascade Screening for Awareness and Detection of FH Registry.
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.
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.
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.</description><subject>Adolescent</subject><subject>Anticholesteremic Agents - therapeutic use</subject><subject>Child</subject><subject>cholesterol</subject><subject>Cholesterol, LDL - blood</subject><subject>Coronary Artery Disease - prevention & control</subject><subject>Cross-Sectional Studies</subject><subject>Dietary Supplements</subject><subject>Drug Utilization - statistics & numerical data</subject><subject>Female</subject><subject>Humans</subject><subject>Hydroxymethylglutaryl-CoA Reductase Inhibitors - therapeutic use</subject><subject>Hyperlipoproteinemia Type II - blood</subject><subject>Hyperlipoproteinemia Type II - epidemiology</subject><subject>Hyperlipoproteinemia Type II - therapy</subject><subject>Life Sciences & Biomedicine</subject><subject>Life Style</subject><subject>Male</subject><subject>Pediatrics</subject><subject>Registries</subject><subject>Science & Technology</subject><subject>statin</subject><subject>United States - epidemiology</subject><issn>0022-3476</issn><issn>1097-6833</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2021</creationdate><recordtype>article</recordtype><sourceid>HGBXW</sourceid><sourceid>EIF</sourceid><recordid>eNqNkdGKEzEUhgdR3O7qEwiSS0FmTDJpZiJ4scxurbAguO51yCQnbcrMpCappT6Iz2u6rXspXiWQ78_h_F9RvCG4IpjwD5tqswUTK4oprrCoMKPPihnBoil5W9fPixnGlJY1a_hFcRnjBmMsGMYvi4uaioa3Yj4rfndrN5gAE9q7tEZLSBD8r8PK7yJaqNENTg1oedhC0Gs_QDw-DzA6hdyE0hrQw-QSGHSfVIL4Ed2opJANfnx87FTUygC61wFgctMKWR_Q9V7lgRAjUpNBN3mkTs5P5WKJvsHKxRQOr4oXVg0RXp_Pq-Jhcfu9W5Z3Xz9_6a7vSl3PRSoZWCuI0hxqoXRrac85VaTXjeCsr5WijSCWGag1gNC6x7wlGJjuDWVzsPVV8e707zb4H7u8nhxd1DAMaoJcgaSMcc5b3JCM1idUBx9jACu3wY0qHCTB8ihEbuSjEHkUIrGQWUhOvT0P2PUjmKfMXwMZaE_AHnpvo3YwaXjCsjJOMGW0yTfSdC7XnKvq_G5KOfr-_6OZ_nSiIff500GQ54RxIQuQxrt_bvIHHlXBkA</recordid><startdate>202102</startdate><enddate>202102</enddate><creator>de Ferranti, Sarah D.</creator><creator>Shrader, Peter</creator><creator>Linton, MacRae F.</creator><creator>Knowles, Joshua W.</creator><creator>Hudgins, Lisa C.</creator><creator>Benuck, Irwin</creator><creator>Kindt, Iris</creator><creator>O'Brien, Emily C.</creator><creator>Peterson, Amy L.</creator><creator>Ahmad, Zahid S.</creator><creator>Clauss, Sarah</creator><creator>Duell, P. Barton</creator><creator>Shapiro, Michael D.</creator><creator>Wilemon, Katherine</creator><creator>Gidding, Samuel S.</creator><creator>Neal, William</creator><general>Elsevier Inc</general><general>Elsevier</general><scope>BLEPL</scope><scope>DTL</scope><scope>HGBXW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0002-0208-5154</orcidid><orcidid>https://orcid.org/0000-0003-1922-7240</orcidid><orcidid>https://orcid.org/0000-0002-9071-3287</orcidid></search><sort><creationdate>202102</creationdate><title>Children with Heterozygous Familial Hypercholesterolemia in the United States: Data from the Cascade Screening for Awareness and Detection-FH Registry</title><author>de Ferranti, Sarah D. ; Shrader, Peter ; Linton, MacRae F. ; Knowles, Joshua W. ; Hudgins, Lisa C. ; Benuck, Irwin ; Kindt, Iris ; O'Brien, Emily C. ; Peterson, Amy L. ; Ahmad, Zahid S. ; Clauss, Sarah ; Duell, P. 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Barton</creatorcontrib><creatorcontrib>Shapiro, Michael D.</creatorcontrib><creatorcontrib>Wilemon, Katherine</creatorcontrib><creatorcontrib>Gidding, Samuel S.</creatorcontrib><creatorcontrib>Neal, William</creatorcontrib><collection>Web of Science Core Collection</collection><collection>Science Citation Index Expanded</collection><collection>Web of Science - Science Citation Index Expanded - 2021</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>The Journal of pediatrics</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>de Ferranti, Sarah D.</au><au>Shrader, Peter</au><au>Linton, MacRae F.</au><au>Knowles, Joshua W.</au><au>Hudgins, Lisa C.</au><au>Benuck, Irwin</au><au>Kindt, Iris</au><au>O'Brien, Emily C.</au><au>Peterson, Amy L.</au><au>Ahmad, Zahid S.</au><au>Clauss, Sarah</au><au>Duell, P. Barton</au><au>Shapiro, Michael D.</au><au>Wilemon, Katherine</au><au>Gidding, Samuel S.</au><au>Neal, William</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Children with Heterozygous Familial Hypercholesterolemia in the United States: Data from the Cascade Screening for Awareness and Detection-FH Registry</atitle><jtitle>The Journal of pediatrics</jtitle><stitle>J PEDIATR-US</stitle><addtitle>J Pediatr</addtitle><date>2021-02</date><risdate>2021</risdate><volume>229</volume><spage>70</spage><epage>77</epage><pages>70-77</pages><issn>0022-3476</issn><eissn>1097-6833</eissn><abstract>To describe enrollment characteristics of youth in the Cascade Screening for Awareness and Detection of FH Registry.
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.
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.
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.</abstract><cop>NEW YORK</cop><pub>Elsevier Inc</pub><pmid>32976895</pmid><doi>10.1016/j.jpeds.2020.09.042</doi><tpages>8</tpages><orcidid>https://orcid.org/0000-0002-0208-5154</orcidid><orcidid>https://orcid.org/0000-0003-1922-7240</orcidid><orcidid>https://orcid.org/0000-0002-9071-3287</orcidid></addata></record> |
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| ispartof | The Journal of pediatrics, 2021-02, Vol.229, p.70-77 |
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| subjects | Adolescent Anticholesteremic Agents - therapeutic use Child cholesterol Cholesterol, LDL - blood Coronary Artery Disease - prevention & control Cross-Sectional Studies Dietary Supplements Drug Utilization - statistics & numerical data Female Humans Hydroxymethylglutaryl-CoA Reductase Inhibitors - therapeutic use Hyperlipoproteinemia Type II - blood Hyperlipoproteinemia Type II - epidemiology Hyperlipoproteinemia Type II - therapy Life Sciences & Biomedicine Life Style Male Pediatrics Registries Science & Technology statin United States - epidemiology |
| title | Children with Heterozygous Familial Hypercholesterolemia in the United States: Data from the Cascade Screening for Awareness and Detection-FH Registry |
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