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Secondary Thrombotic Microangiopathy (TMA) in children- A Central Role for the Glycocalyx
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DOI：10.1016/j.kint.2026.04.047.png)
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Abstract
En 中文
Thrombotic microangiopathy (TMA) is classically defined by the triad of haemolytic anaemia, thrombocytopenia, and organ injury, most notably kidney involvement. Secondary TMA, which arises in association with specific diseases or external triggers, is more common than primary or complement mediated TMA. Current evidence supports an important role for complement activation in the pathogenesis of secondary TMA, and emerging data highlight the contribution of the endothelial glycocalyx (eGC) to complement dysregulation at the endothelial surface. The eGC is an integrated matrix lining the luminal aspect of endothelial cells and plays a central role in maintaining endothelial homeostasis and preventing thrombosis. Its unique structure may represent a missing mechanistic link in several forms of secondary TMA that are relevant to children, including Pneumococcal associated TMA (Pneu-TMA), Shiga toxin-producing Escherichia coli-hemolytic uremic syndrome (STEC-HUS), calcineurin inhibitors associated TMA, and hypertension associated TMA. This mini review summarises current evidence and understanding of the central role of the eGC in these conditions, and discusses the implications for future therapeutic innovation.
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12.6
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1.6W
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4.8W
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