Study of severity and prognostic factors in patients with pseudoxanthoma elasticum

2020 Fiscal Year Final Research Report

• Project/Area Number: 19K17802
• Research Category: Grant-in-Aid for Early-Career Scientists
• Allocation Type: Multi-year Fund
• Review Section: Basic Section 53050:Dermatology-related
• Research Institution: Nagasaki University
• Principal Investigator: IWANAGA Akira 長崎大学, 医歯薬学総合研究科(医学系), 助教 (00621947)
• Project Period (FY): 2019-04-01 – 2021-03-31
• Keywords: Pseudoxanthoma elasticum

Outline of Final Research Achievements

We measured the blood concentrations of four proteins which prevented calcification in 50 Japanese patients with pseudoxanthoma elasticum and 20 normal controls. As a result, the blood concentrations of the three proteins in patients were reduced. In addition, statistical analysis was performed on these three proteins and gene mutations and severity, but no relationship was found. In this study, we can't clarify the factors to predict severity. But, from this result, we found that there were maybe multiple factors to determine the severity of pseudoxanthoma elasticum.

Free Research Field

皮膚科学

Academic Significance and Societal Importance of the Research Achievements

弾性線維性仮性黄色腫患者において、血液中のタンパク質の濃度を測定した結果、複数のタンパクの血液中の濃度が低下していた。これらのタンパク質は石灰化を防ぐはたらきをしており、それらが低下したことで血管の石灰化をきたし、重症となってしまうのではないかと考えた。今後、これらのタンパク質がなぜ低下するのかを明らかにすることができれば、重症となる患者を予想でき、治療の開発に繋がる可能性がある。