A nation-wide survey of patients with homozygous familial hypercholesterolemia phenotype undergoing LDL-apheresis in Turkey (A-HIT 1 registry)

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dc.contributor.authorKayikcioglu, Meral
dc.contributor.authorTokgozoglu, Lale
dc.contributor.authorYilmaz, Mehmet
dc.contributor.authorKaynar, Leylagul
dc.contributor.authorAktan, Melih
dc.contributor.authorDurmus, Rana Berru
dc.contributor.authorGokce, Cumali
dc.date.accessioned2024-02-23T14:02:12Z
dc.date.available2024-02-23T14:02:12Z
dc.date.issued2018
dc.departmentNEÜen_US
dc.description.abstractBackground and aims: Homozygous familial hypercholesterolemia (HoFH) is a genetic condition characterized by lethally high levels of low-density lipoprotein cholesterol (LDL-C) from birth, and requires rapid and aggressive intervention to prevent death due to coronary heart disease and/or atherosclerosis. Where available, lipoprotein apheresis (LA) is the mainstay of treatment to promote survival. Methods: A-HIT1 registry was conducted with the aim of providing insight to the real-life management of HoFH patients undergoing LA in Turkey, where LA procedures are fully reimbursed and widely available. Participating centers provided patient information, including family history, treatment patterns and relevant laboratory values, via a standard questionnaire. Results: The study evaluated 88 patients (mean age: 27 +/- 11 years, 41 women) in 19 centers. All patients were receiving regular LA with a clinical diagnosis of HoFH. Mean age at first symptom disease was 10 +/- 10 years, and at diagnosis it was 12 +/- 11 years; 74.7% were diagnosed before age 15 years; and only 31% before the age of 7. First referral of most patients was to pediatricians. Early onset coronary artery disease was present in 57.8% of patients. Mean age at first LA was 21 +/- 12 years. Only 11 (12.5%) patients were undergoing LA weekly. Mean frequency of apheresis sessions was 19 +/- 13 days. For the last four LA sessions, LDL-C levels reached the target in only in 5.7% of patients. Conclusions: Diagnosis of HoFH is delayed, and LDL targets are not reached. LA frequencies are not optimal. Urgent attention is needed to support the survival of patients with HoFH. (c) 2018 Elsevier B.V. All rights reserved.en_US
dc.description.sponsorshipAegerion pharmaceuticals; Amryt Pharmaceuticalsen_US
dc.description.sponsorshipThis investigator initiated study was funded by Aegerion pharmaceuticals, and authors wish to thank Nigel Eastmond of Eastmond Medicomm Ltd for assistance in editing the final manuscript, which was funded by Amryt Pharmaceuticals.en_US
dc.identifier.doi10.1016/j.atherosclerosis.2018.01.034
dc.identifier.endpage48en_US
dc.identifier.issn0021-9150
dc.identifier.issn1879-1484
dc.identifier.pmid29407887en_US
dc.identifier.scopus2-s2.0-85041631684en_US
dc.identifier.scopusqualityQ1en_US
dc.identifier.startpage42en_US
dc.identifier.urihttps://doi.org/10.1016/j.atherosclerosis.2018.01.034
dc.identifier.urihttps://hdl.handle.net/20.500.12452/11627
dc.identifier.volume270en_US
dc.identifier.wosWOS:000427639800007en_US
dc.identifier.wosqualityQ1en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherElsevier Ireland Ltden_US
dc.relation.ispartofAtherosclerosisen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectLipoprotein Apheresisen_US
dc.subjectHomozygous Familial Hypercholesterolemiaen_US
dc.subjectTurkeyen_US
dc.subjectRegistryen_US
dc.titleA nation-wide survey of patients with homozygous familial hypercholesterolemia phenotype undergoing LDL-apheresis in Turkey (A-HIT 1 registry)en_US
dc.typeArticleen_US

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