Long-term follow-up in patients with homozygous familial hypercholesterolemia; 13-year experience of a university hospital lipid clinic [Homozigot ailevi hiperkolesterolemili hastalarin uzun dönem izlemi: Bir üniversite hastanesi lipit poliklini?inin 13 yillik deneyimi]

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dc.contributor.authorKayikçio?lu M.
dc.contributor.authorKismali E.
dc.contributor.authorCan L.
dc.contributor.authorPayzin S.
dc.date.accessioned2019-10-26T21:28:55Z
dc.date.available2019-10-26T21:28:55Z
dc.date.issued2014
dc.departmentEge Üniversitesien_US
dc.description.abstractObjectives: Familiar hypercholesterolemia (FH) is a genetic disease characterized with extremely high levels of cholesterol leading to premature atherosclerosis. In homozygous individuals (HoFH) cardiovascular events could develop in childhood. In this article, long-term clinical experience with adult HoFH patients who are followed in Department of Cardiology, Ege University Faculty of Medicine is presented. Study design: Seventeen HoFH patients (11 females, 6 males) who are being followed between the years 2000-2013 were included. All data including clinical characteristics, family history, lipid levels, treatment, lipid-apheresis, cardiovascular events, complications were obtained retrospectively from patient chart records. Results: Mean age was 31 ±10 years at admission to our clinic. First diagnosis age was 25±14. At diagnosis, mean cholesterol level was 625±136 mg/dl. Admission complaints were dermatologic (41 %) and ischemic symptoms (41 %). Atotal of 3 patients (18%) were diagnosed during family screening. 65% of the patients' parents had consanguineous marriage. Xantomas was present in 59%, aortic valve pathology in 59%, and carotid artery plaques in 47%. Coronary artery disease was documented in 59%. Though all patients had indication for apheresis, 10 patients received apheresis due to high refusal rate. Age at the first apheresis was 27±12 (minimum 10-maximum 42) and adherence to apheresis was 60%. With 2 years regular apheresis skin depositions were vanished, however carotid atherosclerosis and aortic pathology progressed. During the 43±42 months follow-up, 4 patients died (mean age: 25±5). Conclusion: Diagnosis is late in HoAH. Due to the delayed treatment of lipid apheresis, atherosclerosis and aortic stenosis progress in these patients. The awareness of the physicians and knowledge of the public is warranted. © 2014 Turkish Society of Cardiology.en_US
dc.identifier.doi10.5543/tkda.2014.09633en_US
dc.identifier.endpage611en_US
dc.identifier.issn1016-5169
dc.identifier.issue7en_US
dc.identifier.pmid25490294en_US
dc.identifier.scopusqualityQ4en_US
dc.identifier.startpage599en_US
dc.identifier.urihttps://doi.org/10.5543/tkda.2014.09633
dc.identifier.urihttps://hdl.handle.net/11454/17520
dc.identifier.volume42en_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isotren_US
dc.publisherTurkish Society of Cardiologyen_US
dc.relation.ispartofTurk Kardiyoloji Dernegi Arsivien_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectApolipoprotein B-100/geneticsen_US
dc.subjectCoronary artery diseaseen_US
dc.subjectFamilial hypercholesterolemiaen_US
dc.subjectGenetic predisposition to diseaseen_US
dc.subjectHyperlipoproteinemia type 11/epidemiologyen_US
dc.subjectLipid apheresisen_US
dc.subjectXanthomaen_US
dc.titleLong-term follow-up in patients with homozygous familial hypercholesterolemia; 13-year experience of a university hospital lipid clinic [Homozigot ailevi hiperkolesterolemili hastalarin uzun dönem izlemi: Bir üniversite hastanesi lipit poliklini?inin 13 yillik deneyimi]en_US
dc.typeArticleen_US

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