Orphanet: Apparent mineralocorticoid excess

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Apparent mineralocorticoid excess

Disease definition

A rare form of pseudohyperaldosteronism characterized by very early-onset and severe hypertension, associated with low renin levels and hypoaldosteronism.


Classification level: Disorder
  • Synonym(s):
    • 11-beta-hydroxysteroid dehydrogenase deficiency type 2
    • Ulick syndrome
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal recessive 
  • Age of onset: Infancy, Neonatal
  • ICD-10: E26.1
  • OMIM: 218030
  • UMLS: C0342488  C2936861  C3887949
  • MeSH: C537422  D043204
  • GARD: 433
  • MedDRA: -
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