A new family with dexamethasone-suppressible hyperaldosteronism: aldosterone unresponsiveness to angiotensin II.
A family with nine siblings in which three siblings have been shown to have dexamethasone-suppressible hyperaldosteronism was studied. All three showed no significant changes of plasma aldosterone during angiotensin II infusion at incremental rates under baseline conditions. After dexamethasone administration (2 mg/d for 4 weeks) plasma renin activity (PRA) rose to normal-supranormal range, while plasma and urinary aldosterone were maintained at low-normal levels. No restoration of aldosterone response to angiotensin II was observed on dexamethasone. Two other siblings were found to be hypertensive with normal baseline data; however, both showed plasma aldosterone hyperresponsiveness to ACTH. In the four normotensive siblings aldosterone response to ACTH was normal. The family pedigree was consistent with autosomal dominant transmission of the disorder. HLA typing showed haplotype A3 Bw35 in all five hypertensive sibs and in one normotensive. In conclusion, low aldosterone compared to PRA, and plasma aldosterone unresponsiveness to angiotensin II infusion before and during dexamethasone, show functional impairment, at least temporary, of the zona glomerulosa. These findings support the hypothesis that aldosterone may be derived from the zona fasciculata in this disorder.
Fallo
F
F
Sonino
N
N
Armanini
D
D
Luzzi
T
T
Pedini
F
F
Pasini
C
C
Mantero
F
F
D000324:Adrenocorticotropic Hormone / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D000450:Aldosterone / Q000097:blood*; D000804:Angiotensin II / Q000627:therapeutic use; D001794:Blood Pressure; D003907:Dexamethasone / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D006929:Hyperaldosteronism / Q000097:blood / Q000188:drug therapy / Q000235:genetics*; D008297:Male; D010375:Pedigree; D012083:Renin / Q000097:blood
D000804:Angiotensin II; D000450:Aldosterone; D003907:Dexamethasone; D000324:Adrenocorticotropic Hormone; D012083:Renin; D006854:Hydrocortisone