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Congenital renal artery stenosis

Disease definition

A rare renal disease characterized by congenital unilateral or bilateral narrowing of the renal artery leading to severe arterial hypertension and progressive renal failure in the neonate. Manifestations include hypertensive encephalopathy and/or neurological signs and symptoms due to hyponatremia, polyuria, renal electrolyte loss, proteinuria, and hematuria.


Classification level: Disorder
  • Synonym(s):
    • Congenital renovascular hypoplasia
  • Prevalence: -
  • Inheritance: -
  • Age of onset: Neonatal
  • ICD-10: Q27.1
  • OMIM: -
  • UMLS: C0495523
  • MeSH: -
  • GARD: -
  • MedDRA: -
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