6.2.b

Disorders of steroid metabolism

  • 21-hydroxylase deficiency (Congenital adrenal hyperplasia due to 21-hydroxylase deficiency)
  • 11-beta-hydroxylase deficiency (Congenital adrenal hyperplasia due to 11-beta-hydroxylase deficiency)
  • 11-beta-hydroxylase superactivity (Glucocorticoid remediable aldosteronism; familial hyperaldosteronism type 1)
  • 3-beta-hydroxysteroid dehydrogenase deficiency (Congenital adrenal hyperplasia due to 3-beta-hydroxysteroid dehydrogenase deficiency)
  • 17-hydroxylase/17,20-lyase deficiency
  • Steroidogenic acute regulatory protein deficiency (Lipoid adrenal hyperplasia)
  • Cytochrome P450 oxidoreductase deficiency
  • Steroid 18-hydroxylase deficiency (Aldosterone synthase deficiency; corticosterone methyloxidase type 1 deficiency)
  • Steroid 18-oxidase deficiency (Corticosterone methyloxidase type 2 deficiency)
  • Hexose-6-phosphate dehydrogenase deficiency (Cortisone reductase deficiency type 1)
  • 11-beta-hydroxysteroid dehydrogenase deficiency (Cortisone reductase deficiency type 2)
  • Glucocorticoid receptor deficiency (Glucocorticoid resistance)
  • Mineralocorticoid receptor deficiency (Autosomal dominant pseudohypoaldosteronism type 1)
  • Mineralocortoid receptor superactivity (Early-onset hypertension with exacerbation in pregnancy)
  • ACTH receptor deficiency (Melanocortin-2 receptor deficiency; hereditary glucocorticoid deficiency type 1)
  • Melanocortin-2 receptor accessory protein deficiency (Hereditary glucocorticoid deficiency type 2)
  • Aromatase deficiency
  • Aromatase superactivity
  • Estrogen receptor deficiency (Estrogen resistance)
  • Estrogen receptor 2 deficiency
  • Progesterone receptor deficiency
  • Side-chain cleavage enzyme deficiency (Desmolase deficiency)
  • 17-beta-hydroxysteroid dehydrogenase deficiency (17-ketosteroid reductase deficiency; male pseudohermaphroditism with gynecomastia)
  • 3-alpha-hydroxysteroid dehydrogenase type 3 deficiency
  • 11-beta-hydroxysteroid dehydrogenase type 2 deficiency (Apparent mineralocorticoid excess)
  • Steroid 5-alpha-reductase 2 deficiency
  • Androgen receptor deficiency (Androgen insensitivity syndrome)
  • X-linked spinal and bulbar muscular atrophy (Kennedy disease)
  • Hydroxysteroid sulfotransferase deficiency (Autosomal recessive congenital ichthyosis type 14)
  • Steroid sulfatase deficiency (X-linked ichthyosis)
  • Chloride channel 2 superactivity (Familial hyperaldosteronism type 2)

Subnetworks

PM MD 1

PM-MD

Disorders of pyruvate metabolism, Krebs cycle defects, mitochondrial oxidative phosphorylation disorders, disorders of thiamine transport and metabolism

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