Elsevier

Pediatric Neurology

Volume 103, February 2020, Pages 21-26
Pediatric Neurology

Original Article
Epidemiology, Comorbidities, and Outcomes of Posterior Reversible Encephalopathy Syndrome in Children in the United States

https://doi.org/10.1016/j.pediatrneurol.2019.07.007Get rights and content

Abstract

Background

Posterior reversible encephalopathy syndrome (PRES) is an increasingly recognized entity with certain identified predisposing factors in children. However, the actual incidence, comorbidities, outcomes, and hospitalization charges among children (aged less than 20 years) in the United States are largely unknown.

Methods

We analyzed the Kids' Inpatient Database for incidence of PRES-related hospitalizations, associated diagnoses, in-hospital outcomes, and charges for children in the United States in 2016. We report demographics, risk factors, discharge status, mortality, length of stay, and hospitalization charges.

Results

In 2016, 825 pediatric hospitalizations related to PRES were captured in the Kids' Inpatient Database. Hospital discharges including solid organ transplant, bone marrow transplant, hypertension, renal disorder, primary immunodeficiency, malignancy, sepsis, severe sepsis, systemic connective tissue disorder, blood transfusion, hypomagnesemia, and sickle cell anemia were queried for presence of PRES. The majority of patients were discharged home. We found that PRES-related hospitalizations were significantly associated with increased length of stay and hospitalization charges in 2016 (P < 0.001). A mortality rate of 3.2% was found in PRES-related hospitalizations when compared with 0.4% in non-PRES hospitalizations (P < 0.001).

Conclusion

PRES accounted for 0.04% of the hospitalizations in this database. Hypertension and the presence of a renal disorder are the most significant risk factors found to be associated with PRES. The presence of PRES was associated with a significant increase in hospitalization charges and increased length of stay.

Introduction

Posterior reversible encephalopathy syndrome (PRES) was first recognized in 1996 as a specific clinicoradiological disease characterized by potentially reversible vasogenic edema of the brain with preferential involvement of the posterior cortex.1 Since then, it has been increasingly reported in children and is known to occur in a variety of disorders such as hypertension, eclampsia, renal insufficiency, systemic lupus erythematosus (SLE), sickle cell disease, sepsis, use of cytotoxic medications (for malignancy or immune suppression), and organ transplantation.2, 3, 4 Endothelial dysfunction and impairment of cerebral autoregulation, which disturbs the blood-brain barrier thereby causing vasogenic edema, is considered as the underlying pathophysiologic mechanism.5 Clinical signs and symptoms include cephalalgia, visual disturbances, alteration in mental status, focal neurological deficits, and seizures.2, 6, 7

PRES has been reported in children, but most data are from single-center retrospective studies and focused on a specific subset of patients. The incidences of PRES in children that have been reported were estimated from admissions to a pediatric critical care unit (0.4%) and pediatric patients with cancer (0.7%).8, 9 However, its true incidence, especially in the general pediatric population, is not known. We sought to investigate the incidence of PRES-related hospitalizations, associated risk factors, and their burden in children and adolescents from a large national-level inpatient database.

Section snippets

Materials and Methods

We analyzed the data from the Agency for Healthcare Research and Quality (AHRQ)-sponsored 2016 Kids' Inpatient Database (KID). KID captures inpatient hospital discharge-level information for patients younger than 20 years of age.10 KID 2016 was created from a stratified, random sample of discharges from all community, non-rehabilitation hospitals in 47 states in the United States participating in the Healthcare Cost and Utilization Project (HCUP), which included a total of 6,266,285 hospital

Results

On univariate (Table 2) and multiple regression (Table 3), age was independently associated with PRES (adjusted odds ratio 1.02, P < 0.001) with PRES being more common in the adolescent group (13 to 20 years). Females tend to develop PRES more often than males (P < 0.001), and after adjustments using multivariate regression analysis, male sex was still found to be protective (adjusted odds ratio 0.65, P < 0.001). Caucasians were more at risk when compared with other races (P < 0.001), but after

Discussion

We describe 825 hospitalizations that were associated with a diagnosis of PRES in 2016. This article, this is the largest cohort of PRES reported in children reported to date. In this large population-based study, PRES accounted for 0.04% of the hospitalizations of children (Table 2). Previously, multiple case series and retrospective studies have reported varying incidence of PRES in children. However, these data may not be extrapolated to the general pediatric population because they included

Conclusion

To date, this is the largest study demonstrating the incidence, associated factors, and outcomes of PRES among pediatric hospitalizations. This is the first study to analyze various associated comorbid conditions of PRES in hospitalized children. In our study, hypertension and renal disorders in children are the most common risk factors found to be associated with PRES. PRES in the pediatric population is associated with significant utilization of hospital resources and a high risk of morbidity

References (21)

There are more references available in the full text version of this article.

Cited by (0)

Multiple primary authors: Krishna Kishore Umapathi and Aravind Thavamani participated actively in the conduct of the study, analysis of data, and writing or revising the article for scientific content. Both Krishna Kishore Umapathi and Aravind Thavamani were equally responsible for the work described in this article. and are designated as first authors.

Declarations of interest: None.

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

View full text