Clinical characteristics of adult and paediatric patients with familial hypercholesterolemia: A real-life cross-sectional study from the Turkish National Database.


Journal

Atherosclerosis
ISSN: 1879-1484
Titre abrégé: Atherosclerosis
Pays: Ireland
ID NLM: 0242543

Informations de publication

Date de publication:
06 2023
Historique:
received: 01 11 2022
revised: 22 04 2023
accepted: 25 04 2023
medline: 19 6 2023
pubmed: 22 5 2023
entrez: 22 5 2023
Statut: ppublish

Résumé

Familial hypercholesterolemia (FH) is the most common cause of premature atherosclerotic cardiovascular disease (ASCVD). Türkiye is among the countries with the highest rate of ASCVD. However, no population-based study has been published so far on the prevalence of FH, demographic and clinical characteristics, burden of ASCVD, treatment compliance, and attainment of low-density lipoprotein cholesterol (LDL-C) targets. We performed a study using the Turkish Ministry of Health's national electronic health records involving 83,063,515 citizens as of December 2021 dating back 2016. Adults fulfilling the diagnostic criteria of definite or probable FH according to the Dutch Lipid Network Criteria (DLNC), and children and adolescents fulfilling the criteria of probable FH according to the European Atherosclerosis Society (EAS) Consensus Panel report formed the study population (n = 157,790). The primary endpoint was the prevalence of FH. Probable or definite FH was detected in 0.63% (1 in 158) of the adults and 0.61% (1 in 164) of the total population. The proportion of adults with LDL-C levels >4.9 mmol/L (190 mg/dL) was 4.56% (1 in 22). The prevalence of FH among children and adolescents was 0.37% (1 in 270). Less than one-third of the children and adolescents, and two-thirds of young adults (aged 18-29) with FH were already diagnosed with dyslipidaemia. The proportion of adults and children and adolescents on lipid-lowering treatment (LLT) was 32.1% and 1.5%, respectively. The overall discontinuation rate of LLT was 65.8% among adults and 77.9% among children and adolescents. Almost no subjects on LLT were found to attain the target LDL-C levels. This nationwide study showed a very high prevalence of FH in Türkiye. Patients with FH are diagnosed late and treated sub-optimally. Whether these findings may explain the high rates of premature ASCVD in Türkiye needs further investigation. These results denote the urgent need for country-wide initiatives for early diagnosis and effective management of FH patients.

Sections du résumé

BACKGROUND AND AIMS
Familial hypercholesterolemia (FH) is the most common cause of premature atherosclerotic cardiovascular disease (ASCVD). Türkiye is among the countries with the highest rate of ASCVD. However, no population-based study has been published so far on the prevalence of FH, demographic and clinical characteristics, burden of ASCVD, treatment compliance, and attainment of low-density lipoprotein cholesterol (LDL-C) targets.
METHODS
We performed a study using the Turkish Ministry of Health's national electronic health records involving 83,063,515 citizens as of December 2021 dating back 2016. Adults fulfilling the diagnostic criteria of definite or probable FH according to the Dutch Lipid Network Criteria (DLNC), and children and adolescents fulfilling the criteria of probable FH according to the European Atherosclerosis Society (EAS) Consensus Panel report formed the study population (n = 157,790). The primary endpoint was the prevalence of FH.
RESULTS
Probable or definite FH was detected in 0.63% (1 in 158) of the adults and 0.61% (1 in 164) of the total population. The proportion of adults with LDL-C levels >4.9 mmol/L (190 mg/dL) was 4.56% (1 in 22). The prevalence of FH among children and adolescents was 0.37% (1 in 270). Less than one-third of the children and adolescents, and two-thirds of young adults (aged 18-29) with FH were already diagnosed with dyslipidaemia. The proportion of adults and children and adolescents on lipid-lowering treatment (LLT) was 32.1% and 1.5%, respectively. The overall discontinuation rate of LLT was 65.8% among adults and 77.9% among children and adolescents. Almost no subjects on LLT were found to attain the target LDL-C levels.
CONCLUSIONS
This nationwide study showed a very high prevalence of FH in Türkiye. Patients with FH are diagnosed late and treated sub-optimally. Whether these findings may explain the high rates of premature ASCVD in Türkiye needs further investigation. These results denote the urgent need for country-wide initiatives for early diagnosis and effective management of FH patients.

Identifiants

pubmed: 37216728
pii: S0021-9150(23)00181-8
doi: 10.1016/j.atherosclerosis.2023.04.011
pii:
doi:

Substances chimiques

Cholesterol, LDL 0

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

9-20

Informations de copyright

Copyright © 2023 Elsevier B.V. All rights reserved.

Déclaration de conflit d'intérêts

Declaration of competing interest The authors written below declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Auteurs

Alper Sonmez (A)

Ankara Guven Hospital, Department of Endocrinology and Metabolism, Ankara, Turkiye.

Ibrahim Demirci (I)

University of Health Sciences, Gulhane Training and Research Hospital, Department of Endocrinology and Metabolism, Ankara, Turkiye.

Cem Haymana (C)

University of Health Sciences, Gulhane Faculty of Medicine, Department of Endocrinology and Metabolism, Ankara, Turkiye.

Ilker Tasci (I)

University of Health Sciences, Gulhane Faculty of Medicine, Department of Internal Medicine, Ankara, Turkiye.

Mustafa Okan Ayvalı (MO)

General Directorate of the Health Information Systems, Ministry of Health, Ankara, Turkiye.

Naim Ata (N)

Department of Strategy Development, Ministry of Health, Ankara, Turkiye.

Fatih Suheyl Ezgu (FS)

Gazi University, Faculty of Medicine, Department of Pediatric Endocrinology, Ankara, Turkiye.

Fahri Bayram (F)

Erciyes University, Faculty of Medicine, Department of Endocrinology and Metabolism, Kayseri, Turkiye.

Cem Barcin (C)

University of Health Sciences, Gulhane Faculty of Medicine, Department of Cardiology, Ankara, Turkiye.

Murat Caglayan (M)

University of Health Sciences, Yildirim Beyazit Training and Research Hospital, Department of Biochemistry, Ankara, Turkiye.

Mustafa Mahir Ülgü (MM)

General Directorate of the Health Information Systems, Ministry of Health, Ankara, Turkiye.

Suayip Birinci (S)

Deputy Health Minister, Ministry of Health, Ankara, Turkiye.

Lale Tokgozoglu (L)

Hacettepe University, Faculty of Medicine, Department of Cardiology, Ankara, Turkiye.

Ilhan Satman (I)

Istanbul University, Istanbul Faculty of Medicine, Department of Internal Medicine, Division of Endocrinology and Metabolism, Istanbul, Turkiye.

Meral Kayikcioglu (M)

Ege University Faculty of Medicine, Department of Cardiology, Izmir, Turkiye. Electronic address: meral.kayikcioglu@gmail.com.

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