Clinical management, psychosocial characteristics, and quality of life in patients with homozygous familial hypercholesterolemia undergoing LDL-apheresis in Turkey: Results of a nationwide survey (A-HIT1 registry)

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dc.contributor.authorKayikcioglu, Meral
dc.contributor.authorKuman-Tuncel, Oztem
dc.contributor.authorPirildar, Sebnem
dc.contributor.authorYilmaz, Mehmet
dc.contributor.authorKaynar, Leylagul
dc.contributor.authorAktan, Melih
dc.contributor.authorDurms, Rana Berru
dc.date.accessioned2024-02-23T14:12:31Z
dc.date.available2024-02-23T14:12:31Z
dc.date.issued2019
dc.departmentNEÜen_US
dc.description.abstractBACKGROUND: Homozygous familial hypercholesterolemia (HoFH) is a rare, life-threatening inherited disease leading to early-onset atherosclerosis and associated morbidity. Because of its rarity, longitudinal data on the management of HoFH in the real world are lacking, particularly on the impact the condition has on quality of life (QoL), including the impact of the extracorporeal lipid removal procedure apheresis (LA). METHODS: The A-HIT1 study included 88 patients with HoFH aged >= 12 years receiving regular LA in 19 centers in Turkey. Demographic and disease characteristics data were obtained. For patients aged >= 18 years, additional data on psychosocial status were obtained via the SF-36 score, the Hospital Anxiety and Depression Scale, and a HoFH-specific questionnaire. RESULTS: There was no standardized approach to therapy between centers. Mean (+/-SD) frequency of LA sessions was every 19.9 (+/-14) days, with only 11.6% receiving LA weekly, and 85% of patients were not willing to increase LA frequency. The most common concerns of patients were disease prognosis (31%), and physical, aesthetic, and psychological problems (27.5%, 15.9%, and 11.6%, respectively). Lower age at diagnosis was associated with better QoL, lower anxiety, improved functioning, and greater emotional well-being compared to later diagnosis. CONCLUSIONS: These findings demonstrate that adult patients with HoFH undergoing LA, experience significant impairment of QoL with an increased risk of depression. From patients' point of view, LA is time-consuming, uncomfortable, and difficult to cope with. The speed of diagnosis and referral has a considerable impact on patient well-being. (C) 2019 National Lipid Association. All rights reserved.en_US
dc.description.sponsorshipAegerion pharmaceuticals; Amryt Pharmaceuticals DACen_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 article, which was funded by Amryt Pharmaceuticals DAC.en_US
dc.identifier.doi10.1016/j.jacl.2019.02.001
dc.identifier.endpage467en_US
dc.identifier.issn1933-2874
dc.identifier.issn1876-4789
dc.identifier.issue3en_US
dc.identifier.pmid30928440en_US
dc.identifier.scopus2-s2.0-85063369912en_US
dc.identifier.scopusqualityQ1en_US
dc.identifier.startpage455en_US
dc.identifier.urihttps://doi.org/10.1016/j.jacl.2019.02.001
dc.identifier.urihttps://hdl.handle.net/20.500.12452/12088
dc.identifier.volume13en_US
dc.identifier.wosWOS:000475544900016en_US
dc.identifier.wosqualityQ1en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherElsevier Science Incen_US
dc.relation.ispartofJournal Of Clinical Lipidologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectHomozygous Familial Hypercholesterolemiaen_US
dc.subjectLdl-Apheresisen_US
dc.subjectQuality Of Lifeen_US
dc.subjectPsychosocial Impacten_US
dc.subjectTurkeyen_US
dc.titleClinical management, psychosocial characteristics, and quality of life in patients with homozygous familial hypercholesterolemia undergoing LDL-apheresis in Turkey: Results of a nationwide survey (A-HIT1 registry)en_US
dc.typeArticleen_US

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