2023-06-132023-06-132019-07Kayıkcıoğlu, M. vd. (2019). ''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)''. Journal of Clinical Lipidology, 13(3), 455-467.1933-28741876-4789https://doi.org/10.1016/j.jacl.2019.02.001https://www.sciencedirect.com/science/article/pii/S1933287419300297http://hdl.handle.net/11452/33027Çalışmada 25 yazar bulunmaktadır. Bu yazarlardan sadece Bursa Uludağ Üniversitesi mensuplarının girişleri yapılmıştır.BACKGROUND: 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.eninfo:eu-repo/semantics/closedAccessPharmacology & pharmacyHomozygous familial hypercholesterolemiaLdl-ApheresisQuality of lifePsychosocial impactTurkeyLipoprotein apheresisFollow-upAortic rootInsightsExperienceSomatizationChildrenGuidanceCareAdolescentAdultBlood component removalCholesterol, ldlFemaleHomozygoteHumansHyperlipoproteinemia type IIMaleMiddle agedQuality of lifeRegistriesSurveys and questionnairesTurkeyYoung adultArticle0004755449000162-s2.0-8506336991245546713330928440Pharmacology & pharmacyBlood Component Removal; Familial Hypercholesterolemia; LipoproteinsLow density lipoprotein cholesterolAdultAgeArticleEmotionFamilial hypercholesterolemiaFemaleFunctional statusHealth surveyHomozygosityHumanLipoprotein apheresisMajor clinical studyMalePersonal appearancePhysical performancePriority journalPrognosisPsychological well-beingQuality of lifeSocial psychologyTurkey (republic)AdolescentApheresisBloodFamilial hypercholesterolemiaGeneticsHomozygoteMiddle agedQuestionnaireRegisterYoung adultTurkey (bird)