What have we learned from Turkish familial hypercholesterolemia registries (A-HIT1 and A-HIT2)?

Autor: Kayikcioglu M; Ege Univ Faculty of Med, Izmir, Turkey. Electronic address: meral.kayikcioglu@gmail.com., Tokgozoglu L; Hacettepe Univ Faculty of Med, Ankara, Turkey., Dogan V; Mugla Sitki Kocman Univ Faculty of Med, Mugla, Turkey., Ceyhan C; Adnan Menderes Univ Faculty of Med, Aydin, Turkey., Tuncez A; Selcuk Univ Faculty of Med, Konya, Turkey., Kutlu M; Karadeniz Teknik Univ Faculty of Med, Trabzon, Turkey., Onrat E; Kocatepe Univ Faculty of Med, Afyon, Turkey., Alici G; Kartal Kosuyolu Res & Training Hosp, Istanbul, Turkey., Akbulut M; Firat Univ Faculty of Med, Elazig, Turkey., Celik A; Mersin Univ Faculty of Med, Mersin, Turkey., Yesilbursa D; Uludag Univ Faculty of Med, Bursa, Turkey., Sahin T; Kocaeli Univ Faculty of Med, Kocaeli, Turkey., Sonmez A; Gulhane Res & Training Hosp, Ankara, Turkey., Ozdogan O; Tepecik Res & Training Hosp, Izmir, Turkey., Temizhan A; Yuksek Ihtisas Res & Training Hosp, Ankara, Turkey., Kilic S; Nizip State Hosp, Gaziantep, Turkey., Bayram F; Erciyes Univ Faculty of Med, Kayseri, Turkey., Sabuncu T; Harran Univ Faculty of Med, Sanliurfa, Turkey., Coskun FY; Gaziantep Univ Faculty of Med, Gaziantep, Turkey., Ildizli M; Sultanbeyli State Hosp, Istanbul, Turkey., Durakoglugil E; Recep Tayyip Erdogan Univ Faculty of Med, Rize, Turkey., Kirilmaz B; 18 Mart Univ Faculty of Med, Canakkale, Turkey., Yilmaz MB; Cumhuriyet Univ Faculty of Med, Sivas, Turkey., Yigit Z; Istanbul University Cardiology Institute, Istanbul, Turkey., Yildirim AB; Akdeniz Univ Faculty of Med, Antalya, Turkey., Gedikli O; 19 Mayis Univ Faculty of Med, Samsun, Turkey., Topcu S; Ataturk Univ Faculty of Med, Erzurum, Turkey., Oğuz A; Medeniyet Univ Faculty of Med, Istanbul, Turkey., Demir M; Cukurova Univ Faculty of Med, Adana, Turkey., Yenerçağ M; Samsun Res & Training Hosp, Samsun, Turkey., Yıldırır A; Baskent Univ Faculty of Med, Ankara, Turkey., Demircan S; İstanbul Bilim Univ Faculty of Med, Istanbul, Turkey., Yilmaz M; Gaziantep Univ Faculty of Med, Gaziantep, Turkey., Kaynar LG; Erciyes Univ Faculty of Med, Kayseri, Turkey., Aktan M; Istanbul University Istanbul Univ Faculty of Med, Istanbul, Turkey., Durmus RB; Istanbul University Istanbul Univ Faculty of Med, Istanbul, Turkey., Gokce C; Mustafa Kemal Univ Faculty of Med Hatay, Turkey., Ozcebe Oİ; Hacettepe Univ Faculty of Med, Ankara, Turkey., Akyol TK; Hacettepe Univ Faculty of Med, Ankara, Turkey., Okutan H; Diskapi Yildirim Beyazit Res & Training Hosp, Ankara, Turkey., Sag S; Uludag Univ Faculty of Med, Bursa, Turkey., Gul OO; Uludag Univ Faculty of Med, Bursa, Turkey., Salcioglu Z; Kanuni Sultan Suleyman Res & Training Hosp, Istanbul, Turkey., Altunkeser BB; Selcuk Univ Faculty of Med, Konya, Turkey., Kuku I; Inonu Univ Faculty of Med, Malatya, Turkey., Yasar HY; Tepecik Res & Training Hosp, Izmir, Turkey., Kurtoglu E; Antalya Res & Training Hosp, Antalya, Turkey., Kose MD; Behcet Uz Children Res & Training Hosp, Izmir, Turkey., Demircioglu S; Necmettin Erbakan Univ Faculty of Med, Konya, Turkey., Pekkolay Z; Dicle Univ Faculty of Med, Diyarbakir, Turkey., Ilhan O; Ankara Univ Faculty of Med, Ankara, Turkey., Can LH; Ege Univ Faculty of Med, Izmir, Turkey.
Jazyk: angličtina
Zdroj: Atherosclerosis [Atherosclerosis] 2018 Oct; Vol. 277, pp. 341-346.
DOI: 10.1016/j.atherosclerosis.2018.08.012
Abstrakt: Background and Aims: Familial hypercholesterolemia (FH) is a common genetic disease of high-level cholesterol leading to premature atherosclerosis. One of the key aspects to overcome FH burden is the generation of large-scale reliable data in terms of registries. This manuscript underlines the important results of nation-wide Turkish FH registries (A-HIT1 and A-HIT2).
Methods: A-HIT1 is a survey of homozygous FH patients undergoing low density lipoprotein (LDL) apheresis (LA). A-HIT2 is a registry of adult FH patients (homozygous and heterozygous) admitted to outpatient clinics. Both registries used clinical diagnosis of FH.
Results: A-HIT1 evaluated 88 patients (27 ± 11 years, 41 women) in 19 centers. All patients were receiving regular LA. There was a 7.37 ± 7.1-year delay between diagnosis and initiation of LA. LDL-cholesterol levels reached the target only in 5 cases. Mean frequency of apheresis sessions was 19 ± 13 days. None of the centers had a standardized approach for LA. Mean frequency of apheresis sessions was every 19 ± 13 (7-90) days. Only 2 centers were aware of the target LDL levels. A-HIT2 enrolled 1071 FH patients (53 ± 8 years, 606 women) from 31 outpatients clinics specialized in cardiology (27), internal medicine (1), and endocrinology (3); 96.4% were heterozygous. 459 patients were on statin treatment. LDL targets were attained in 23 patients (2.1% of the whole population, 5% receiving statin) on treatment. However, 66% of statin-receiving patients were on intense doses of statins. Awareness of FH was 9.5% in the whole patient population.
Conclusions: The first nationwide FH registries revealed that FH is still undertreated even in specialized centers in Turkey. Additional effective treatment regiments are urgently needed.
(Copyright © 2018 Elsevier B.V. All rights reserved.)
Databáze: MEDLINE
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