{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T06:17:41Z","timestamp":1788329861742,"version":"build-2803163510"},"reference-count":42,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"17","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["JAHA"],"published-print":{"date-parts":[[2026,9,1]]},"abstract":"<jats:sec xml:lang=\"en\">\n                    <jats:title>Background<\/jats:title>\n                    <jats:p xml:lang=\"en\">Homozygous familial hypercholesterolemia (HoFH), characterized by extremely elevated low\u2010density lipoprotein cholesterol (LDL\u2010C) due to severely impaired LDL\u2010C clearance from circulation, remains challenging to treat. Here, we investigated clinical benefits of monoclonal antibody, evinacumab, inhibiting angiopoietin\u2010like 3, recently approved in children with homozygous familial hypercholesterolemia.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec xml:lang=\"en\">\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p xml:lang=\"en\">This retrospective, observational study included 13 children with homozygous familial hypercholesterolemia on oral lipid\u2010lowering therapy and assessed their attainment of LDL\u2010C goal, need for lipoprotein apheresis (LA), and cardiac plaque formation at initiation of evinacumab and at follow\u2010up of 2.0 (1.3\u20133.0) years.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec xml:lang=\"en\">\n                    <jats:title>Results<\/jats:title>\n                    <jats:p xml:lang=\"en\">\n                      Evinacumab, initiated at age 11 (8.5\u201313.5) years, increased number of patients reaching LDL\u2010C goal from none before initiation to 9 of 13 (69%;\n                      <jats:italic toggle=\"no\">P<\/jats:italic>\n                      =0.004) at follow\u2010up. Mean\u00b1SD LDL\u2010C at follow\u2010up was 111 (44) mg\/dL, compared with 206 (61) mg\/dL before evinacumab (mean difference, \u221295 mg\/dL [95% CI, \u2212142 to \u221247];\n                      <jats:italic toggle=\"no\">P<\/jats:italic>\n                      &lt;0.001). Before initiation of evinacumab, 11 of 13 children received LA with average interval of 7.8\u2009days. At follow\u2010up, LA frequency decreased in 7 of 11 children with average interval of 18.9\u2009days; increase between LA sessions was 11.0\u2009days (3.5\u201318.5;\n                      <jats:italic toggle=\"no\">P<\/jats:italic>\n                      =0.008), 42% longer session interval. For 3 (15%) children, due to evinacumab initiation, LA remained dispensable over the course of treatment. Coronary computed tomography angiography showed plaque stabilization (n=5), regression (n=3), and no formation (n=2), in 10 of 13 (77%) children, and plaque progression in 3 (23%) children.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec xml:lang=\"en\">\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p xml:lang=\"en\">Add\u2010on evinacumab increased number of children with homozygous familial hypercholesterolemia reaching and maintaining LDL\u2010C goal, concomitant reduction in LA frequency, and in most patients, stabilization or improvement in coronary computed tomography angiography findings.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1161\/jaha.125.048419","type":"journal-article","created":{"date-parts":[[2026,8,20]],"date-time":"2026-08-20T11:49:01Z","timestamp":1787226541000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Clinical Benefits Associated With Evinacumab in Pediatric Patients With Homozygous Familial Hypercholesterolemia"],"prefix":"10.1161","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0971-1936","authenticated-orcid":false,"given":"M. D.","family":"Reijman","sequence":"first","affiliation":[{"name":"Amsterdam University Medical Centers","place":["Amsterdam, The Netherlands"]},{"name":"Amsterdam Cardiovascular Sciences Research Institute","place":["Amsterdam, The Netherlands"]},{"name":"Amsterdam University Medical Centers","place":["Amsterdam, The Netherlands"]},{"name":"Amsterdam Gastroenterology Endocrinology Metabolism Research Institute","place":["Amsterdam, The Netherlands"]},{"name":"Amsterdam University Medical Centers","place":["Amsterdam, The Netherlands"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3692-8754","authenticated-orcid":false,"given":"Charlotte","family":"Nigmann","sequence":"additional","affiliation":[{"name":"Medical University Vienna","place":["Vienna, Austria"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D. M.","family":"Kusters","sequence":"additional","affiliation":[{"name":"Amsterdam University Medical Centers","place":["Amsterdam, The Netherlands"]},{"name":"Amsterdam Cardiovascular Sciences Research Institute","place":["Amsterdam, The Netherlands"]},{"name":"Amsterdam University Medical Centers","place":["Amsterdam, The Netherlands"]},{"name":"Amsterdam Gastroenterology Endocrinology Metabolism Research Institute","place":["Amsterdam, The Netherlands"]},{"name":"Amsterdam University Medical Centers","place":["Amsterdam, The Netherlands"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3940-4670","authenticated-orcid":false,"given":"R. N.","family":"Planken","sequence":"additional","affiliation":[{"name":"Amsterdam Cardiovascular Sciences Research Institute","place":["Amsterdam, The Netherlands"]},{"name":"Amsterdam University Medical Centers","place":["Amsterdam, The Netherlands"]},{"name":"Amsterdam University Medical Centers","place":["Amsterdam, The Netherlands"]},{"name":"Mayo Clinic","place":["Rochester, USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2502-0676","authenticated-orcid":false,"given":"Christian","family":"Loewe","sequence":"additional","affiliation":[{"name":"Medical University Vienna and Vienna General Hospital","place":["Vienna, Austria"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8934-3995","authenticated-orcid":false,"given":"Victor A.","family":"Verpalen","sequence":"additional","affiliation":[{"name":"Amsterdam Cardiovascular Sciences Research Institute","place":["Amsterdam, The Netherlands"]},{"name":"Amsterdam University Medical Centers","place":["Amsterdam, The Netherlands"]},{"name":"Amsterdam University Medical Centers","place":["Amsterdam, The Netherlands"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8145-1676","authenticated-orcid":false,"given":"G. 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