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Hypertension secondary to renin-secreting juxtaglomerular cell tumor: case report and review of 38 cases

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Abstract

A 15-year-old girl with severe high renin hypertension caused by a juxtaglomerular cell tumor (JCT) was successfully treated with the calcium channel blocker nifedipine until surgical removal effected a permanent cure. This case was incorporated into a review of the 37 cases previously published. Comparison of the children and adolescents with the adult population showed that the features of JCT were similar in the two groups except for the average duration of symptoms prior to diagnosis (pediatric group 2.6 years vs. 6.0 years for the adult group). Analysis of all 38 cases demonstrated the following:

  1. 1.

    Teenagers constituted the largest single population with JCT (39%) and approximately two-thirds of the entire population were female.

  2. 2.

    Many patients failed to show persistent hypokalemia despite high plasma renin activity and secondary hyperaldosteronism.

  3. 3.

    Renal angiography was initially negative in more than half the cases.

  4. 4.

    Renal vein renin failed to show lateralization to the affected kidney in 52% of the cases.

  5. 5.

    Computerized tomography demonstrated a renal mass in all of the cases in which it was performed, even when other imaging studies were negative.

  6. 6.

    Calcium channel blockers may evolve as the preferred treatment for the high renin hypertension of JCT.

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Author notes

  1. Melinda McVicar

    Present address: Department of Pediatrics, St. Vincent's Hospital and Medical Center of New York, 153 West 11th Street, 10011, New York, NY, USA

Authors and Affiliations

  1. Department of Pediatrics, North Shore University Hospital-Cornell University Medical College, 11030, Manhasset, New York, USA

    Melinda McVicar, Christine Carman & Manju Chandra

  2. Department of Pathology, North Shore University Hospital-Cornell University Medical College, 11030, Manhasset, New York, USA

    Rakesh Jumari Abbi, Saul Teichberg & Ellen Kahn

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  1. Melinda McVicar
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  2. Christine Carman
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McVicar, M., Carman, C., Chandra, M. et al. Hypertension secondary to renin-secreting juxtaglomerular cell tumor: case report and review of 38 cases. Pediatr Nephrol 7, 404–412 (1993). https://doi.org/10.1007/BF00857553

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  • DOI: https://doi.org/10.1007/BF00857553

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