Endothelial dysfuntion in children with idiopathic nephrotic syndrome.

Background: Impaired endothelial function is the initial step in atherogenesis, which is largely responsible for ischaemic heart disease and thrombotic strokes decades later.

Methods: Fourty two children with first episode nephrotic syndrome (FENS) aged 1-16 years and 40 controls were enrolled. Soluble thrombomodulin (sTM), tissue plasminogen activator (t-PA), plasminogen activator inhibitor -1 (PAI-1) and von-willebrand factor (vWF) levels were measured in plasma in FENS, at 12 weeks of drug induced remission and in steroid resistant nephrotic syndrome (SRNS) patients at diagnosis.

Results: PAI-1, sTM, vWF and t-PA were significantly raised at the onset of nephrotic syndrome (p<0.0001). All the markers had a fall after 12 weeks of steroid treatment, but were still raised. Children with SRNS had higher levels of sTM, tPA, vWF as compared to infrequent relapsers, at onset and at 4 weeks of steroid treatment.

Conclusion: Children with idiopathic nephrotic syndrome have endothelial dysfunction which is largely dependent upon disease activity.