Pathophysiology of cyanotic nephropathy associated with congenital heart disease

2015 Fiscal Year Final Research Report

• Project/Area Number: 25461637
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Research Field: Pediatrics
• Research Institution: Yokohama City University (2014-2015); National Research Institute for Child Health and Development (2013)
• Principal Investigator: ITO Shuichi 横浜市立大学, 医学(系)研究科(研究院), 教授 (20336572)
• Co-Investigator(Kenkyū-buntansha): KATO Hitoshi 独立行政法人国立成育医療研究センター, 器官病態系内科部, 院長 (70214393) ; MATSUOKA Kentaro 独立行政法人国立成育医療研究センター, 病理診断部, 医長 (90286443) ; ABE Jun 独立行政法人国立成育医療研究センター, 免疫アレルギー研究部, 室長 (40281688)
• Project Period (FY): 2013-04-01 – 2016-03-31
• Keywords: チアノーゼ性腎症 / 先天性心疾患 / HIF-1α / VEGF / TNF-α / 蛋白尿

Outline of Final Research Achievements

We elucidated the pathophysiology of cyanotic nephropathy associated with congenital heart disease (CHD), which is a severe complication of cyanotic CHD. In kidney tissue, glomerular hypertrophy, enlargement of capillary lumen, increase of mesangial cell and the matrix were observed, and positive immunohistochemistry staining with HIF-1αand VEGF in glomerular endothelial cells were also observed in all patients. These findings are negative in normal control. Although plasma level of VEGF and VEGF-a were not elevated in patients as compared to control, but plasma VEGF-c and TNF-α were significantly elevated in patients. Our study revealed HIF-1αand VEG expressed in glomerular endothelial cells could be involved in etiology of cyanotic nephropathy. Specific therapy targeting VEGF-c and TNF-α may be possible against cyanotic nephropathy.

Free Research Field

小児腎臓病学、小児リウマチ学