The Diagnostic Criteria 2020 for Triglyceride Deposit Cardiomyovasculopathy.
Adipose triglyceride lipase
BMIPP
Diagnostic criteria
Rare and intractable diseases
Tricaprin
Triglyceride deposit cardiomyovasculopathy
Journal
Annals of nuclear cardiology
ISSN: 2424-1741
Titre abrégé: Ann Nucl Cardiol
Pays: Japan
ID NLM: 101747118
Informations de publication
Date de publication:
2020
2020
Historique:
received:
13
07
2020
revised:
22
07
2020
accepted:
22
07
2020
medline:
1
1
2020
pubmed:
1
1
2020
entrez:
1
5
2023
Statut:
ppublish
Résumé
Triglyceride deposit cardiomyovasculopathy (TGCV) is a newly identified disease that was discovered in individuals who required cardiac transplantation in Japan in 2008. Defective intracellular lipolysis causes triglyceride (TG) accumulation in the myocardium and coronary artery vascular smooth muscle cells, which results in severe heart failure and coronary artery disease with poor prognosis. A known cause of TGCV is a genetic deficiency of adipose triglyceride lipase (ATGL), a rate-limiting enzyme in the intracellular hydrolysis of TG. TGCV is classified into primary TGCV with ATGL mutations and idiopathic TGCV without ATGL mutations. Since its discovery, the Japan TGCV Study Group has attempted to elucidate its pathophysiology, develop diagnostic procedures, and specific treatment. Myocardial scintigraphy with iodine-123-
Identifiants
pubmed: 37123492
doi: 10.17996/anc.20-00131
pmc: PMC10133930
doi:
Types de publication
Journal Article
Review
Langues
eng
Pagination
99-104Informations de copyright
© The Japanese Society of Nuclear Cardiology 2020.
Déclaration de conflit d'intérêts
KH received grants from Nihon Medi-Physics Co. Ltd. KN received grants from Nihon Medi-Physics Co. Ltd. HY received honoraria for speaking activities from Bayer, Kowa, and Takeda Co. Ltd.
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