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Posterior reversible encephalopathy syndrome in children with kidney disease
D N Gera1, S B Patil1, A Iyer2
1Department of Nephrology and Clinical Transplantation, Institute of Kidney Diseases and Research Center, Dr. HL Trivedi Institute of Transplantation Sciences (IKDRC-ITS), Ahmedabad, India.
Insights
Posterior reversible encephalopathy syndrome (PRES) is often underdiagnosed in pediatric renal disease patients. Early diagnosis and treatment of contributing factors like hypertension and renal disease are crucial for recovery.
Area of Science:
- Neurology
- Pediatrics
- Nephrology
Background:
- Posterior reversible encephalopathy syndrome (PRES) is a complex neurological condition with varied causes.
- Its presentation and characteristics in pediatric patients, especially those with renal disease, are not well understood.
- PRES typically involves headache, vision loss, altered mentation, and seizures, with characteristic neuro-imaging findings.
Purpose of the Study:
- To investigate the clinical profile, contributing factors, and outcomes of PRES in pediatric patients with renal disease.
- To highlight PRES as a potentially underdiagnosed entity in this population.
- To identify key triggers and emphasize the importance of early diagnosis and management.
Main Methods:
- A case series of 11 pediatric patients diagnosed with PRES between September 2010 and February 2012.
- Patients were selected from a total of 660 pediatric renal disease patients, representing a 1.66% incidence.
- Data collected included clinical symptoms, associated renal diseases, identified contributory factors, neuro-imaging results, and patient outcomes.
Main Results:
- PRES occurred in 1.66% of pediatric renal patients, with common symptoms including headache (73%), seizures (91%), and altered mentation (55%).
- Associated conditions included acute renal failure (55%), hemolytic uremic syndrome (36%), and uncontrolled hypertension (100%).
- Neurological recovery was rapid (within a week for most) with normalized imaging, underscoring the syndrome's reversibility when managed promptly.
Conclusions:
- PRES is an underdiagnosed condition in children with renal diseases.
- Uncontrolled hypertension, renal disease, and immunosuppressive therapies are significant triggers for PRES in this cohort.
- Prompt diagnosis and management of associated conditions are vital for achieving complete and early reversal of PRES symptoms and imaging abnormalities.
Abstract:
Posterior reversible encephalopathy syndrome (PRES) is a clinic-radiographic entity of heterogeneous etiologies that are grouped together because of similar findings on neuro-imaging and associated symptom complex of headache, vision loss, altered mentation, and seizures. Although usually considered benign and reversible, characteristics of this syndrome in pediatric patients remain obscure. This case series included 11 patients (8 males, 3 females, age 3-15 years) of PRES during September 2010 to February 2012 out of a total 660 renal pediatric patients (1.66%). We studied their clinical profile, contributory factors, and outcome. Presenting symptoms were headache in 73%, dimness of vision or cortical blindness in 36%, seizures in 91%, and altered mentation in 55%. The associated renal diseases were acute renal failure (55%), chronic renal failure (9%), and 36% had normal renal function. The contributory factors were uncontrolled hypertension (100%), severe hypoproteinemia (9%), persistent hypocalcemia (9%), hemolytic uremic syndrome (36%), cyclosporine toxicity (9%), lupus nephritis (9%), high hematocrit (9%), and pulse methylprednisolone (9%). Brain imaging showed involvement of occipito-parietal area (100%) and other brain areas (63%). All but one patient of hemolytic uremic syndrome had complete clinical neurological recovery in a week, and all had normal neurological imaging after 4-5 weeks. PRES is an underdiagnosed entity in pediatric renal disease patients. Associated hypertension, renal disease, and immunosuppressive treatment are important triggers. Early diagnosis and treatment of comorbid conditions is of prime importance for early reversal of syndrome.
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