CASE REPORT article
Front. Med.
Sec. Nephrology
Volume 12 - 2025 | doi: 10.3389/fmed.2025.1567332
This article is part of the Research TopicAdvancements in Congenital Heart Disease: Diagnosis and Management InnovationsView all 10 articles
Midaortic syndrome with IgA nephropathy in a child: a case report
Provisionally accepted
- Children's Hospital, Fudan University, Shanghai, China
Select one of your emails
You have multiple emails registered with Frontiers:
Notify me on publication
Please enter your email address:
If you already have an account, please login
You don't have a Frontiers account ? You can register here
Background: IgA nephropathy (IgAN) is one of the most common glomerulonephritis characterized by deposition of IgA immune complex in the mesangial region and mesangial proliferation in children. It could progress to end stage renal disease. The underlying mechanism of IgAN is not fully understood. It has been known that IgAN could be secondary to autoimmune disorders, respiratory tract diseases, neoplasia, infection, gastrointestinal and liver diseases. Midaortic syndrome (MAS) is characterized by narrowing or occlusion of the distal thoracoabdominal aorta and the openings of its major branches, which may lead to organ damage like heart failure, renal dysfunction and even death. It may be congenital (present at birth) or acquired later in life. So far, no cases of IgAN concomitant with MAS have been reported. Case presentation: A 12-year-old boy was diagnosed with biopsy-proven IgAN, which was characterized by heavy proteinuria and hematuria. Additionally, MAS was found by performing abdominal computed tomography angiography (CTA) due to blood pressure difference between upper arm and thigh. Methylprednisolone and cyclophosphamide were used for treating IgAN. MAS was corrected with GORE-TEX Vascular Grafts (expanded polytetrafluoroethylene, ePTFE). Remission was achieved eventually. To the best of our knowledge, we are the first to report a childhood case who has IgAN concomitant with congenital MAS. We also presented a case-based review regarding the association between IgAN and vascular stenosis. Conclusions: MAS is an aggravating factor and might be a new cause of secondary IgAN.
Keywords: IgA nephropathy, Midaortic syndrome, Glomerulonephritis, vascularmalformation, Proteinuria
Received: 27 Jan 2025; Accepted: 22 Aug 2025.
Copyright: © 2025 Shen, Shen, Li, Cai, Zheng, Huang, Zhang and Kang. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Yulin Kang, Children's Hospital, Fudan University, Shanghai, China
Disclaimer: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.