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Abstract: FR-PO0697

FSGS-Like Features and Proteinuria in an Adolescent with Unilateral Renal Artery Stenosis

Session Information

Category: Pediatric Nephrology

  • 1900 Pediatric Nephrology

Authors

  • Saenz, Bianca Esmeralda, Baylor College of Medicine Department of Pediatrics, Houston, Texas, United States
  • Orjuela, Alvaro H., Baylor College of Medicine Department of Pediatrics, Houston, Texas, United States
  • Hernandez, Jose Alberto, Baylor College of Medicine, Houston, Texas, United States
  • Cortes-Santiago, Nahir, Baylor College of Medicine Department of Pediatrics, Houston, Texas, United States
Introduction

Renal artery stenosis (RAS) is a potentially reversible cause of hypertension (HTN) in pediatric patients. We present an unusual case of unilateral RAS in an adolescent with secondary focal segmental glomerulosclerosis(FSGS) like features on biopsy and proteinuria that resolved after angioplasty.

Case Description

A 15 year old male was admitted for hypertensive emergency with posterior reversible encephalopathy syndrome requiring nicardipine drip.Workup showed acute kidney injury (AKI),creatinine1.64, urinary protein creatinine ratio (UPCR)1.2, bland UA,with normal CBC,albumin,PTH and C3/C4. Ultrasound showed asymmetric kidneys and angiogram revealed severe left RAS. He was transitioned to Procardia and discharged but re-admitted due to persistent AKI and worsening UPCR to 2.54.Biopsies showed glomerulomegaly, pseudocrescents,and mesangial hypercellularity in the right kidney concerning for secondary FSGS. Both kidneys had foot process effacement and negative immunofluorescence. He later underwent left renal artery recanalization and acheived normotension with improved UPCR to 0.15 and transitioned to lisinopril. After few weeks lisinopril was stopped due to inconsistent use and he maintained normotension, stabilized creatinine(0.95), and resolution of proteinuria.

Discussion

Elevation of angiotensin II and resulting hypertension in unilateral RAS can be associated with high glomerular perfusion pressure and hyperfiltration in the contralateral kidney and present as nephrotic range proteinuria. Chronic elevated angiotensin II may induce FSGS like changes. Our patient case is unusual as FSGS like lesions are very uncommon in children. The presence of bilateral podocyte effacement in this case of unilateral RAS remains unexplained. The patient’s proteinuria and HTN resolved with stabilized renal function post angioplasty suggesting attenuation of the injurious effects of circulating angiotensin II independent of blood pressure control.

Digital Object Identifier (DOI)