Study of atherosclerosis in CINCA sydrome

• Project/Area Number: 24791072
• Research Category: Grant-in-Aid for Young Scientists (B)
• Allocation Type: Multi-year Fund
• Research Field: Pediatrics
• Research Institution: Kyushu University
• Principal Investigator: YAMAMURA Kenichiro 九州大学, 大学病院, 助教 (30532858)
• Project Period (FY): 2012-04-01 – 2014-03-31
• Project Status: Completed (Fiscal Year 2013)
• Budget Amount: ¥3,510,000 (Direct Cost: ¥2,700,000、Indirect Cost: ¥810,000); Fiscal Year 2013: ¥2,340,000 (Direct Cost: ¥1,800,000、Indirect Cost: ¥540,000); Fiscal Year 2012: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
• Keywords: CINCA症候群 / 動脈硬化 / 慢性炎症

Research Abstract

Chronic inflammation plays a key role in the development of atherosclerosis. Chronic infantile neurologic cutaneous and articular (CINCA) syndrome is a severe autoinflammatory disease which displays signs of inflammation from their early childhood. The objective of this study is to assess the development of atherosclerosis in patients with CINCA syndrome. Intima-media thickness (IMT) of the carotid arteries, stiffness parameter beta, ankle brachial index (ABI) and pressure wave velocity (PWV) were evaluated by ultrasonography in 3 patients with CINCA syndrome (5, 17, 15 years old) and 19 age-matched healthy controls.
The levels of Carotid IMT, stiffness parameter beta and PWV in CINCA syndrome patients were significantly higher than those in healthy controls. Patients with CINCA syndrome showed signs of atherosclerosis from their early childhood. The result of this study emphasizes the importance of chronic inflammation in the development of atherosclerosis.

Research Products

• [Journal Article] Early progression of atherosclerosis in children with CINCA syndrome (2014)
  • Author(s): Yamamura K, Takada H, Uike K, Nakashima Y, Hirata Y, Nagata H, Takimoto T, Ishimura M, Morihana E, Ohga S, Hara T
  • Journal Title: Rheumatology(Oxford) Volume: (in Press)
• [Journal Article] Early Progression of Atherosclerosis in Children with CINCA Syndrome (2014)
  • Author(s): Yamamura K, Takada H, Uike K, Nakashima Y, HIrata Y, Nagata H, Takimoto T, Ishimura M, Morihana E, ohga S, Hara T
  • Journal Title: Rheumatology(Oxford) Volume: in Press
  • Peer Reviewed
• [Journal Article] Bilateral stenosis of carotid siphon in Hutchinson-Gilford progeria syndrome (2012)
  • Author(s): Narazaki R, Makimura M, Sanefuji M, Fukamachi S, Akiyoshi H, So H, Yamamura K, Doisaki S, Kojima S, Ihara K, Hara T, Ohga S.
  • Journal Title: Brain Dev Volume: epub ahead of print Issue: 7 Pages: 690-693
  • DOI: 10.1016/j.braindev.2012.10.008
  • Peer Reviewed
• [Journal Article] Thrombocytosis in asplenia syndrome with congenital heart disease: A previously unrecognized risk factor for thromboembolism (2012)
  • Author(s): Yamamura K, Joo K, Ohga S, Nagata H, Ikeda K, Muneuchi J, Watanabe M, Hara T.
  • Journal Title: Int J Cardiol Volume: epub ahead of print
  • Peer Reviewed
• [Presentation] Early progression of atherosclerosis in children with cryopyrin-associated periodic syndrome (2013)
  • Author(s): Yamamura K, Uike K, Nakashima Y, Hirata Y, Nagata H, Takimoto T, Ishimura M, Takada H, Ohga S, Hara T
  • Organizer: 9th Asian Society of Pediatric Research
  • Place of Presentation: Kuching, Malaysia
• [Presentation] Early Progression of Atherosclerosis in Children with Cryopyrin-associated Periodic Syndrome (2013)
  • Author(s): Yamamura K, Uike K, Nakashima Y, Hirata Y, Nagata H, Takimoto T, Ishimura M, Takada H, Ohga S, Hara T
  • Organizer: 9th Asian Society of Pediatric Research
  • Place of Presentation: Kuching, Malaysia
• [Presentation] Early Progression of Atherosclerosis in Children with Cryopyrin-associated Periodic Syndrome (2013)
  • Author(s): Kenichiro Yamamura, Kiyoshi Uike, Yasutaka Nakashima, Yuichiro Hirata, Hazumu Nagata, Tomohito Takimoto, Masataka Ishimura, Hidetoshi Takada, Shouichi Ohga, Toshiro Hara
  • Organizer: 9th Asian Society of Pediatric Research Congress
  • Place of Presentation: Kuching, Malaysia