Steroid Therapy Delays the Progression of Glomerular Sclerosis But Not Nephrotic Symptoms in the ICGN Mouse Strain

摘要

Prognosis of chronic nephrotic syndrome (NS) is poor especially in cases of steroid resistance. Thus, it is important to elucidate a mechanism(s) of steroid-resistant NS, but little information is available due to a lack of animal models. The nephrotic mice (ICGN strain) show albuminuria soon after birth and thus they are considered as a model of pediatric NS. In this study, we examined the effect of predonisolone on the NS-related phenotypes in the ICGN mice. Early administrations of predonisolone into the mice before onset of mesangial injuries did not alter the natural courses of albuminuria and hypoalbuminemia during growing periods,. However, this steroid therapy delayed the progression of glomer-ulosclerosis in the murine model. Our findings indicate that: 1) NS features in the ICGN mice are resistant to repeated steroid-therapy; and 2) there may be a different mechanism between albuminuria and glomerulosclerosis in pediatric NS, Overall, this mouse strain could be an experimental tool to explore pathogenesis of steroid-resistant NS.