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

Department of Pediatric Nephrology, Tokyo Metropolitan Kiyose Children's Hospital, Tokyo, Japan.
Nephrology Dialysis Transplantation (Impact Factor: 3.49). 03/2008; 23(8):2531-6. DOI: 10.1093/ndt/gfn013
Source: PubMed

ABSTRACT 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.
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.
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.
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.

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    ABSTRACT: Reversible posterior leukoencephalopathy syndrome (RPLS) is a clinical syndrome based on changes in clinical imaging, and it has been reported to mainly occur in adults. However, it has been recently discovered that RPLS is also prevalent in infant patients, particularly in those using glucocorticoids, immunosuppressant medications and cytotoxic drugs. The current study presents a 5-year-old male with a previous diagnosis of systemic-onset juvenile idiopathic arthritis (SoJIA) and macrophage-activation syndrome who developed posterior reversible encephalopathy syndrome during treatment with glucocorticoids, disease-modifying antirheumatic drugs and biological agent (etanercept) therapy. After ~5 days of treatment, the patient made a complete clinical recovery; the magnetic resonance imaging reviewed 2 weeks later showed that the previous hyper-intensity signal had disappeared and the multiple lesions in the brain had been completely absorbed. The case report shows that, conforming to recent literature, SoJIA in infants should be considered a risk factor for developing RPLS. The clinical manifestations of the disease are multiple, but usually reversible, and the patients mostly have a good prognosis. Rapid diagnosis and treatment is essential as early treatment may prevent progression to irreversible brain damage. By increasing the awareness of RPLS, the patient care may improve and further insight may be gained.
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    ABSTRACT: IntroductionNephrotic syndrome is a disorder characterized by severe proteinuria, hypoproteinemia, and generalized edema resulting from damage to the glomerular basement membrane. In Western countries, nephrotic syndrome affects 2 of 100,000 children per year [1]. In Japan, approximately 1,300 new cases per year of pediatric nephrotic syndrome are reported to the Medical Aid for Specific Chronic Disease of Children and the disease develops in 5 of 100,000 children per year. Approximately 90 % of the cases of pediatric nephrotic syndrome are idiopathic, or of unknown cause. The first-line treatment for an initial episode of pediatric idiopathic nephrotic syndrome is oral steroid therapy, which leads to remission in approximately 80 % of cases (steroid-sensitive nephrotic syndrome) [2]. However, 80 % of children with steroid-sensitive nephrotic syndrome experience one or more relapses, [3] and 50 % of these children have frequent relapses [4]. Those with frequently relapsing nephrotic sy ...
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    ABSTRACT: Posterior reversible encephalopathy syndrome is an uncommon state that occurs in patients with high blood pressure, immunosuppressant therapy, or renal disease, among other conditions, which is diagnosed based on clinical and neuroimage analysis. Objective: describe the characteristics of posterior reversible encepha- lopathy syndrome in Mexican children and compare the results with those reported in the specialized literature. Material and methods: retrospective analysis of clinical records in the period 2006-2013 at the National Institute of Pediatrics in Mexico. Results: 22 patients were included: 60% male and 40% female, mean age 8.6 years. The most commonly associated conditions were acute lymphoblastic leukemia (27%), lupus nephritis (18%) and hemolytic uremic syndrome (9%). Clinically, the patients had seizures (86.3%), headache (12%), nausea and vomiting (7%). High blood pressure was confirmed in 90% of the cases. At diagnosis 50% were in chemothera- py, 27% were receiving immunosuppressants, and 22% high doses of steroids. Based on neuroimage findings, they were classified as grade I (36%); grade II (50%), and grade III (13%). Complications were detected such as stroke in 13% and cortical necrosis in 9%. Conclusions: the characteristics of posterior reversible encephalopathy syndrome in Mexican children are similar to those reported in the spe- cialized literature. Due to the limited number of cases its incidence or prevalence is unknown. High blood pressure may be a risk factor for development of the syndrome or part of a more complex pathophysio- logical mechanism. Although chemotherapy, use of immunosuppres- sants, and high doses of steroids have been associated significantly, the underlying conditions may be relevant to the development of posterior reversible encephalopathy syndrome. Magnetic resonance is the study of choice to diagnose the condition and to detect irreversible sequelae.

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