PMID- 8393954
OWN - NLM
STAT- MEDLINE
DCOM- 19930903
LR  - 20190725
IS  - 0026-0495 (Print)
IS  - 0026-0495 (Linking)
VI  - 42
IP  - 7
DP  - 1993 Jul
TI  - Investigation of the mechanism of hypertension in apparent mineralocorticoid 
      excess.
PG  - 843-5
AB  - Apparent mineralocorticoid excess (AME) is a rare form of low renin hypertension 
      caused by deficiency of 11 beta-hydroxysteroid dehydrogenase (11 beta-HSD), the 
      enzyme responsible for conversion of cortisol to the bio-inactive metabolite, 
      cortisone. This results in prolonged cortisol half-life, activation of type I 
      (mineralocorticoid) receptors by cortisol, sodium and fluid retention, and 
      consequent childhood-onset hypertension. The cortisol secretion rate is low, 
      perhaps due to cortisol's binding to type II (glucocorticoid) receptors and 
      suppressing corticotropin secretion. Patients with AME thus lack stigmata of 
      Cushing's syndrome. To evaluate any potential contribution of the type II 
      (glucocorticoid) receptor to the development of hypertension in AME patients, we 
      administered RU486, a steroid analogue that acts as a pure type II receptor 
      blocker. Selective glucocorticoid receptor blockade did not decrease blood 
      pressure in our patient; instead, a significant increase in average blood 
      pressure was observed (125.1 +/- 1.7 pre-RU486 v 144.7 +/- 1.2 during RU486 
      treatment, P = .0001). We conclude that the type II receptor does not contribute 
      to the development of hypertension in patients with AME.
FAU - Speiser, P W
AU  - Speiser PW
AD  - Department of Pediatrics, New York Hospital-Cornell University Medical Center, 
      New York.
FAU - Riddick, L M
AU  - Riddick LM
FAU - Martin, K
AU  - Martin K
FAU - New, M I
AU  - New MI
LA  - eng
GR  - HD-00072/HD/NICHD NIH HHS/United States
GR  - RR-06020/RR/NCRR NIH HHS/United States
GR  - RR-47/RR/NCRR NIH HHS/United States
GR  - etc.
PT  - Case Reports
PT  - Journal Article
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - United States
TA  - Metabolism
JT  - Metabolism: clinical and experimental
JID - 0375267
RN  - 0 (Mineralocorticoids)
RN  - 0 (Receptors, Glucocorticoid)
RN  - 0 (Receptors, Mineralocorticoid)
RN  - 0 (Receptors, Steroid)
RN  - 320T6RNW1F (Mifepristone)
RN  - EC 1.1.- (Hydroxysteroid Dehydrogenases)
RN  - EC 1.1.1.146 (11-beta-Hydroxysteroid Dehydrogenases)
RN  - WI4X0X7BPJ (Hydrocortisone)
SB  - IM
MH  - 11-beta-Hydroxysteroid Dehydrogenases
MH  - Adolescent
MH  - Female
MH  - Humans
MH  - Hydrocortisone/blood
MH  - Hydroxysteroid Dehydrogenases/*deficiency
MH  - Hypertension/*etiology
MH  - Mifepristone/therapeutic use
MH  - Mineralocorticoids/*metabolism
MH  - Receptors, Glucocorticoid/physiology
MH  - Receptors, Mineralocorticoid
MH  - Receptors, Steroid/physiology
EDAT- 1993/07/01 00:00
MHDA- 1993/07/01 00:01
CRDT- 1993/07/01 00:00
PHST- 1993/07/01 00:00 [pubmed]
PHST- 1993/07/01 00:01 [medline]
PHST- 1993/07/01 00:00 [entrez]
AID - 0026-0495(93)90057-U [pii]
AID - 10.1016/0026-0495(93)90057-u [doi]
PST - ppublish
SO  - Metabolism. 1993 Jul;42(7):843-5. doi: 10.1016/0026-0495(93)90057-u.