Dataset Information

Hokuriku-plus familial hypercholesterolaemia registry study: rationale and study design.

ABSTRACT: INTRODUCTION:Familial hypercholesterolaemia (FH) is an autosomal-dominant inherited genetic disease. It carries an extremely high cardiovascular risk associated with significantly elevated low-density lipoprotein (LDL) cholesterol. The diagnostic rate of this disease in some European nations is quite high, due to the presence of multiple prospective registries. On the other hand, few data-and in particular multicentre data-exist regarding this issue among Japanese subjects. Therefore, this study intends to assemble a multicentre registry that aims to comprehensively assess cardiovascular risk among Japanese FH patients while taking into account their genetic backgrounds. METHODS AND ANALYSIS:The Hokuriku-plus FH registry is a prospective, observational, multicentre cohort study, enrolling consecutive FH patients who fulfil the clinical criteria of FH in Japan from 37 participating hospitals mostly in Hokuriku region of Japan from April 2020 to March 2024. A total of 1000 patients will be enrolled into the study, and we plan to follow-up participants over 5 years. We will collect clinical parameters, including lipids, physical findings, genetic backgrounds and clinical events covering atherosclerotic and other important events, such as malignancies. The primary endpoint of this study is new atherosclerotic cardiovascular disease (ASCVD) events. The secondary endpoints are as follows: LDL cholesterol, secondary ASCVD events and the occurrence of other diseases including hypertension, diabetes and malignancies. ETHICS AND DISSEMINATION:This study is being conducted in compliance with the Declaration of Helsinki, the Ethical Guidelines for Medical and Health Research Involving Human Subjects, and all other applicable laws and guidelines in Japan. This study protocol has been approved by the Institutional Review Board at Kanazawa University. We will disseminate the final results at international conferences and in a peer-reviewed journal. TRIAL REGISTRATION NUMBER:UMIN000038210.

SUBMITTER: Tada H

PROVIDER: S-EPMC7485236 | biostudies-literature | 2020 Sep

REPOSITORIES: biostudies-literature

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Hokuriku-plus familial hypercholesterolaemia registry study: rationale and study design.

Tada Hayato H Okada Hirofumi H Yoshida Shohei S Shimojima Masaya M Nomura Akihiro A Tsuda Toyonobu T Mori Mika M Takashima Shin-Ichiro SI Kato Takeshi T Usui Soichiro S Sakata Kenji K Hayashi Kenshi K Fujino Noboru N Inazu Akihiro A Takahara Shizuko S Imai Yasuhito Y Matsubara Takao T Nohara Atsushi A Miwa Kenji K Namura Masanobu M Terai Hidenobu H Yoshida Taiji T Araki Tsutomu T Minamoto Masahiro M Aburao Toru T Ito Yuji Y Nakanishi Chiaki C Kawasaki Suguru S Todo Yasuhiro Y Koizumi Junji J Kita Yoshihito Y Matsumoto Hiroshi H Shintaku Hiroaki H Hodatsu Akihiko A Ino Hidekazu H Higashikata Toshinori T Takata Mutsuko M Misawa Katsushi K Yamaguchi Masato M Noji Yoshihiro Y Osato Kazuo K Mabuchi Tomohito T Ichise Taro T Kaku Bunji B Katsuda Shoji S Fujimoto Manabu M Uchiyama Katsuharu K Fujioka Kensuke K Nakahashi Takuya T Nozue Tsuyoshi T Michishita Ichiro I Usuda Kazuo K Otowa Kanichi K Okeie Kazuyasu K Hirota Satoshi S Aburadani Isao I Kurokawa Keisuke K Takatori Osamu O Hondo Shunichiro S Oda Hiroyuki H Takata Shigeo S Murai Hisayoshi H Kinoshita Masaki M Nagai Hideo H Sekiguchi Yoshiteru Y Sakagami Satoru S Omi Wataru W Fujita Chikara C Katsuki Tatsuo T Ootsuji Hiroshi H Igarashi Atsushi A Nakano Manabu M Okura Seiichiro S Maeno Koji K Mitamura Yasuhito Y Sugimoto Naoki N Yamamoto Masakazu M Akao Hironobu H Kajinami Kouji K Takamura Masayuki M Kawashiri Masa-Aki MA

BMJ open 20200910 9

<h4>Introduction</h4>Familial hypercholesterolaemia (FH) is an autosomal-dominant inherited genetic disease. It carries an extremely high cardiovascular risk associated with significantly elevated low-density lipoprotein (LDL) cholesterol. The diagnostic rate of this disease in some European nations is quite high, due to the presence of multiple prospective registries. On the other hand, few data-and in particular multicentre data-exist regarding this issue among Japanese subjects. Therefore, th ...[more]

PMID: 32912992

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Project description:BackgroundThe frequency of heart failure (HF) in Tunisia is on the rise and has now become a public health concern. This is mainly due to an aging Tunisian population (Tunisia has one of the oldest populations in Africa as well as the highest life expectancy in the continent) and an increase in coronary artery disease and hypertension. However, no extensive data are available on demographic characteristics, prognosis, and quality of care of patients with HF in Tunisia (nor in North Africa).ObjectiveThe aim of this study was to analyze, follow, and evaluate patients with HF in a large nation-wide multicenter trial.MethodsA total of 1700 patients with HF diagnosed by the investigator will be included in the National Tunisian Registry of Heart Failure study (NATURE-HF). Patients must visit the cardiology clinic 1, 3, and 12 months after study inclusion. This follow-up is provided by the investigator. All data are collected via the DACIMA Clinical Suite web interface.ResultsAt the end of the study, we will note the occurrence of cardiovascular death (sudden death, coronary artery disease, refractory HF, stroke), death from any cause (cardiovascular and noncardiovascular), and the occurrence of a rehospitalization episode for an HF relapse during the follow-up period. Based on these data, we will evaluate the demographic characteristics of the study patients, the characteristics of pathological antecedents, and symptomatic and clinical features of HF. In addition, we will report the paraclinical examination findings such as the laboratory standard parameters and brain natriuretic peptides, electrocardiogram or 24-hour Holter monitoring, echocardiography, and coronarography. We will also provide a description of the therapeutic environment and therapeutic changes that occur during the 1-year follow-up of patients, adverse events following medical treatment and intervention during the 3- and 12-month follow-up, the evaluation of left ventricular ejection fraction during the 3- and 12-month follow-up, the overall rate of rehospitalization over the 1-year follow-up for an HF relapse, and the rate of rehospitalization during the first 3 months after inclusion into the study.ConclusionsThe NATURE-HF study will fill a significant gap in the dynamic landscape of HF care and research. It will provide unique and necessary data on the management and outcomes of patients with HF. This study will yield the largest contemporary longitudinal cohort of patients with HF in Tunisia.Trial registrationClinicalTrials.gov NCT03262675; https://clinicaltrials.gov/ct2/show/NCT03262675.International registered report identifier (irrid)DERR1-10.2196/12262.

Dataset Information

Hokuriku-plus familial hypercholesterolaemia registry study: rationale and study design.

Publications

Hokuriku-plus familial hypercholesterolaemia registry study: rationale and study design.

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