In 1975, a 22-year-old male was admitted to a hospital because of palpitation. He was diagnosed as cardiomyopathy and ventricular tachycardia with multiple lipid droplets in his peripheral leucocytes called “Jordans’ anomaly”. A couple of years later, he unfortunately died of progressive heart failure. A pathologist observed massive triglyceride accumulation in his autopsied heart and stored formalin-fixed specimens for future research.(please see the photo above.)
In 2008, we reported a cardiac transplant recipient with massive TG accumulation in his myocardium as well as unusual coronary atherosclerotic lesions. We named this phenotype triglyceride deposit cardiomyovasculopathy (TGCV). In 2009, we launched the Japan TGCV study group supported by the Japanese Government. The known primary cause of TGCV so far is the genetic mutation in the adipose triglyceride lipase (ATGL), an essential molecule for intracellular hydrolysis of TG, which was discovered by Zimmermann et al. We have identified seven Japanese patients with ATGL deficiency. Half of them had already died of severe heart diseases by their middle age.
Recently, the patient whom I described at the beginning turned out to be ATGL deficiency more than thirty years after his premature death. To our knowledge, he is the oldest ATGL deficiency ever identified. Therefore, we have decided to use photos of his pathological sections as the motif of our symposium, which was kindly provided by Dr. Michio Tanaka, Tokyo, Japan.
Since this kind of disorder including TGCV and neutral lipid storage disease (NLSD) is rare, I strongly believe that it is essential for researchers and physicians to get together, exchange information and discuss how we diagnose and treat patients in order to overcome this intractable disease as soon as possible.
I hope that this symposium will provide such an opportunity.
Thank you very much.
Ken-ichi Hirano, MD, PhD
The First International Symposium on TGCV and NLSD
The 1st International Symposium on Triglyceride Deposit Cardiomyovasculopathy
& Neutral Lipid Strage Disease
26th Nov. 2011
at Kyoto University Clock Tower Centennial Hall
Robert Zimmermann, PhD
(University of Graz, Austria)
Rita Horvath, MD
(University of Newcastle, UK)
Daniela Tavian, MD
(Catholic University, Milan. Italy)
Yun Yuan, MD
(Peking University First Hospital, Beijing, China)
Ichizo Nishino, MD
(National Center of Neurology and Psychiatry, Japan)
Kunihisa Kobayashi, MD
(Fukuoka University, Chikushi Hospital, Japan)
Call for Abstracts
Thank you for your interest in submitting an abstract for the Symposium on TGCV and NLSD.
Please download the abstract form below and return it by e-mail.
DOWNLOAD : 2011 Call for Abstract
E-mail adress : jim%cnt-osaka.com (% = @)
abstract deadline for submissions is: 2 November 2011