Objective:Children with frequently relapsing nephrotic syndrome (FRNS) are usually treated with long term low dose alternate day prednisolone with or without glucocorticoid sparing therapy such as levamisole or cyclosporine A to maintain remission. The degree of hypothalamic-pituitary-adrenal axis (HPA) suppression with such therapeutic strategies has not been studied systematically. HPA suppression could place a child at risk of a relapse or adrenal crisis.
Study design:To study this possibility further, modified low dose synacthen test (0.5mg) was performed in 32 patients (22M: 10F) aged 3.8-17.6 (mean 9.7) years with NS receiving long-term alternate day prednisolone for over 12 months period. Twelve patients received alternate day prednisolone, 11 alternate prednisolone + levamisole and 9 received alternate prednisolone + cyclosporine A. All patients were followed up for a period of 3 years focusing on the relapse rate.
Results:20/32 (62.5%) had a peak serum cortisol concentration below 500nmol/l suggesting suboptimal cortisol secretion and possible HPA suppression. 10/12 children in the prednisolone group and 8/11 in the levamisole group had a suboptimal cortisol response compared with 2/9 in the cyclosporine A group. During follow up, 20 children who had a suboptimal cortisol response had a significantly greater number of relapses (95 relapses) when compared to the 12 children with a normal cortisol response with 24 relapses (p=0.01).
Conclusions:Children with FRNS receiving long- term alternate day prednisolone therapy are at risk of developing HPA suppression and should be evaluated using modified Synacthen test. The children with evidence of HPA suppression are at a greater risk of relapse.
- glucocorticoid sparing therapy
- glucocorticoid therapy
- synacthen test
Statistics from Altmetric.com
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.