Nephrotic state as a risk factor for developing posterior reversible encephalopathy syndrome in paediatric patients with nephrotic syndrome.

Background: Posterior reversible encephalopathy syndrome (PRES) is a distinctive and potentially serious complication of the nephrotic syndrome. The objective of the present study is to characterize the factors predisposing the development of PRES in paediatric patients with nephrotic syndrome.

Methods: We investigated paediatric patients with idiopathic nephrotic syndrome who developed PRES between 1999 and 2005 in our institution. Patients with steroid-sensitive nephrotic syndrome and those with steroid-resistant nephrotic syndrome that were proven to be idiopathic were eligible.

Results: In total, seven patients ranging in age from 1.5 to 15.1 years old were analysed. At the onset of PRES, six of the seven patients were in a nephrotic state. Various degrees of acute renal insufficiency were shown in four patients. The re-administration of cyclosporine after the episodes of PRES was carried out in four patients. During the observation for 17-51 months after the re-administration, the recurrence of PRES did not develop in these patients.

Conclusions: The development of PRES occurred at the time of moderate to severe nephrotic state in most of our paediatric patients with nephrotic syndrome. Besides the administration of cyclosporine and having hypertension, there appear to be several additive factors predisposing the development of PRES in these patients, namely low serum albumin level, generalized oedema, increase in vascular permeability, unstable fluid status and renal insufficiency. The re-administration of cyclosporine to those patients with anamnesis of PRES may be considered after the management and close monitoring of these factors as well as hypertension.