Guidance for Pediatric Familial Hypercholesterolemia 2017
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- Harada-Shiba Mariko
- Department of Molecular Innovation in Lipidology, National Cerebral and Cardiovascular Center Research Institute
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- Ohta Takao
- Department of Child Health and Welfare (Pediatrics), Faculty of Medicine, University of the Ryukyu
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- Ohtake Akira
- Department of Pediatrics, Faculty of Medicine, Saitama Medical University
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- Ogura Masatsune
- Department of Molecular Innovation in Lipidology, National Cerebral and Cardiovascular Center Research Institute
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- Dobashi Kazushige
- Department of Pediatrics, Showa University School of Medicine
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- Nohara Atsushi
- Kanazawa University Health Service Center
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- Yamashita Shizuya
- Department of Community Medicine & Department of Cardiovascular Medicine, Osaka University Graduate School of Medicine Rinku General Medical Center
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- Yokote Koutaro
- Department of Clinical Cell Biology and Medicine, Chiba University Graduate School of Medicine
Bibliographic Information
- Other Title
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- Yokote K; joint working group by Japan pediatric society and Japan atherosclerosis society for making guidance of pediatric familial hypercholesterolemia. Guidance for pediatric familial hypercholesterolemia 2017
- Joint working group by Japan pediatric society and Japan atherosclerosis society for making guidance of pediatric familial hypercholesterolemia. Guidance for pediatric familial hypercholesterolemia 2017
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Abstract
<p>This paper describes consensus statement by Joint Working Group by Japan Pediatric Society and Japan Atherosclerosis Society for Making Guidance of Pediatric Familial Hypercholesterolemia (FH) in order to improve prognosis of FH.</p><p>FH is a common genetic disease caused by mutations in genes related to low density lipoprotein (LDL) receptor pathway. Because patients with FH have high LDL cholesterol (LDL-C) levels from the birth, atherosclerosis begins and develops during childhood which determines the prognosis. Therefore, in order to reduce their lifetime risk for cardiovascular disease, patients with FH need to be diagnosed as early as possible and appropriate treatment should be started.</p><p>Diagnosis of pediatric heterozygous FH patients is made by LDL-C ≥140 mg/dL, and family history of FH or premature CAD. When the diagnosis is made, they need to improve their lifestyle including diet and exercise which sometimes are not enough to reduce LDL-C levels. For pediatric FH aged ≥10 years, pharmacotherapy needs to be considered if the LDL-C level is persistently above 180 mg/dL. Statins are the first line drugs starting from the lowest dose and are increased if necessary. The target LDL-C level should ideally be <140 mg/dL. Assessment of atherosclerosis is mainly performed by noninvasive methods such as ultrasound.</p><p>For homozygous FH patients, the diagnosis is made by existence of skin xanthomas or tendon xanthomas from infancy, and untreated LDL-C levels are approximately twice those of heterozygous FH parents. The responsiveness to pharmacotherapy should be ascertained promptly and if the effect of treatment is not enough, LDL apheresis needs to be immediately initiated.</p>
Journal
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- Journal of Atherosclerosis and Thrombosis
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Journal of Atherosclerosis and Thrombosis 25 (6), 539-553, 2018-06-01
Japan Atherosclerosis Society
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Details
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- CRID
- 1390564237989015040
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- NII Article ID
- 130007382567
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- ISSN
- 18803873
- 13403478
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- PubMed
- 29415907
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- Text Lang
- en
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- Data Source
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- JaLC
- Crossref
- PubMed
- CiNii Articles
- KAKEN
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- Abstract License Flag
- Disallowed