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Renal artery stenosis and nephrotic syndrome: a rare combination in an infant

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Abstract.

We describe an uncommon pediatric finding of unilateral renal artery stenosis, which presented as nephrotic syndrome, hypertension, failure to thrive, and hyponatremia. The child was a previously well 8–month-old male who looked well but had mild periorbital edema with severe hypertension. After 3 days of captopril therapy, the nephrotic–range proteinuria significantly improved. However, the hypertension persisted. Renal imaging revealed a small left kidney with reduced parenchymal uptake and no significant excretion. A renal angiogram demonstrated left renal artery stenosis with increased left renal vein renin activity. The hypertension resolved within 24 h of a left nephrectomy, but non-nephrotic–range proteinuria persisted for 8 months post operatively. Pathology of the left kidney was consistent with fibromuscular dysplasia. Although a few glomeruli (1%) had changes consistent with focal segmental glomerulosclerosis, such a few abnormal glomeruli were unlikely to account for the nephrotic syndrome. Hypertension–induced changes in the unaffected right kidney probably caused the nephrotic–range proteinuria.

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Acknowledgements.

This paper was presented as a poster at the Annual Meeting of ASN/ISN World Congress of Nephrology in San Francisco, California, USA on 15 October 2001 and is published as an abstract in J Am Soc Nephrol (2001)12: A2514. The authors thank the Department of Radiology at the University of Alberta Hospitals for help with radiological investigations and interpretation, and Patricia Jennings for her help with typing the manuscript.

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Correspondence to Manjula Gowrishankar.

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Lang, M.E., Gowrishankar, M. Renal artery stenosis and nephrotic syndrome: a rare combination in an infant. Pediatr Nephrol 18, 276–279 (2003). https://doi.org/10.1007/s00467-003-1069-5

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  • DOI: https://doi.org/10.1007/s00467-003-1069-5

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