期刊
PEDIATRIC AND DEVELOPMENTAL PATHOLOGY
卷 18, 期 4, 页码 310-317出版社
ALLIANCE COMMUNICATIONS GROUP DIVISION ALLEN PRESS
DOI: 10.2350/14-12-1588-OA.1
关键词
aneurysms; axillary artery; endarterectomy; femoral artery; iliac artery; Kawasaki disease
资金
- National Institutes of Health (NIH), National Heart, Lung, Blood Institute [HL69413]
- KD Foundation
- iDASH Grant [U54HL108460]
- NIH through the UCSD Neuroscience Microscopy Shared Facility [P30 NS047101]
Coronary artery aneurysms (CAA) remain an important complication of Kawasaki disease (KD), the most common form of pediatric acquired heart disease in developed countries. Potentially life-threatening CAA develop in 25% of untreated children and 5% of children treated with high-dose intravenous immunoglobulin during the acute phase of the self-limited vasculitis. Noncoronary artery aneurysms (NCAA) in extraparenchymal, muscular arteries occur in a minority of patients with KD who also have CAA, yet little is understood about their formation and remodeling. We postulated that activation of the transforming growth factor-beta (TGF-beta) pathway in KD may influence formation and remodeling of aneurysms in iliac, femoral, and axillary arteries, the most common sites for NCAA. We studied a resected axillary artery from one adult and endarterectomy tissue from the femoral artery from a second adult, both with a history of CAA and NCAA following KD in infancy. Histology of the axillary artery aneurysm revealed destruction of the internal elastic lamina and recanalization of organized thrombus, while the endarterectomy specimen showed dense calcification and luminal myofibroblastic proliferation. Immunohistochemistry for molecules in the TGF-beta signaling pathway revealed increased expression of TGF-beta 2, TGF-beta receptor 2, and phosphorylated SMAD3. These findings suggest ongoing tissue remodeling of the aneurysms decades after the acute injury and demonstrate the importance of the TGF-beta signaling pathway in this process.
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