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Abstract: FR-PO0916

Bilateral Renal Artery Stenosis in a Pediatric Patient with Neurofibromatosis Type 1 (NF1)

Session Information

Category: Pediatric Nephrology

  • 1800 Pediatric Nephrology

Authors

  • Stickles, Eric Benjamin, Lincoln Memorial University, Harrogate, Tennessee, United States
  • Vari, Angela, Lincoln Memorial University, Harrogate, Tennessee, United States
  • Cox, Meredith, Lincoln Memorial University, Harrogate, Tennessee, United States
  • Mannemuddhu, Sai Sudha, East Tennessee Children's Hospital, Knoxville, Tennessee, United States
Introduction

Secondary hypertension (HTN) is the most common cause of HTN in children, with renovascular HTN being the most common cause. We present a 7-year-old girl with NF1, refractory HTN due to bilateral renal artery stenosis (RAS).

Case Description

Patient was diagnosed with HTN at age 3, and the workup for secondary etiologies (Echocardiogram, renal ultrasound (RUS), plasma renin level (15.8 ng/mL/h), and serum creatinine (S.Cr 0.51 mg/dL)) was normal. Her BP readings remained>95%ile for age, ht, and sex despite adherence to Lisinopril 10 mg daily, amlodipine 2.5 mg BID, and atenolol 20 mg BID.
At age 6, another clinic evaluated for refractory HTN. RUS suggested bilateral RAS due to elevated arterial velocities (Fig 1), confirmed by a CT angiogram (Fig 2). Lisinopril was discontinued; amlodipine was increased to 5 mg BID; prazosin was added; and atenolol was continued. She received bilateral renal artery reimplantation, which was successful, following which prazosin was discontinued. The procedure was complicated by transient acute kidney injury (S.Cr 1.19 mg/dL), which normalized in 1 month. She is maintained on Amlodipine and Atenolol, with good BP control and improved post-op renal Doppler (Fig 2).

Discussion

This case highlights the importance of maintaining high clinical suspicion for secondary causes of HTN in children, particularly in those with genetic conditions associated with vasculopathy.