PMID- 3463439
OWN - NLM
STAT- MEDLINE
DCOM- 19861119
LR  - 20190919
IS  - 0730-0077 (Print)
IS  - 0730-0077 (Linking)
VI  - 8
IP  - 4-5
DP  - 1986
TI  - Apparent mineralocorticoid excess causing hypertension and hypokalemia in 
      children.
PG  - 751-72
AB  - Cortisol 11 beta-hydroxysteroid dehydrogenase (11 beta-HSD) deficiency was 
      observed in four patients with apparent mineralocorticoid excess. The 11 beta-HSD 
      deficiency was demonstrated by a markedly decreased urinary 
      tetrahydrocortisone/tetrahydrocortisol (THE/THF) ratio (less than 1 in normal 
      children) during infusion of ACTH and administration of hydrocortisone. We 
      propose that in these patients the 11 beta-HSD deficiency impairs the metabolism 
      of cortisol to cortisone, resulting in a prolonged cortisol half-life, 
      suppression of ACTH, and normal serum cortisol. The 11 beta-HSD deficiency 
      protects the patient from adrenal insufficiency despite the low cortisol 
      secretion; the prolonged half-life of cortisol may contribute to the hypertension 
      and hyporeninemia observed in this disorder. Continuous intravenous 
      hydrocortisone administration resulted in increased blood pressure and decreased 
      serum potassium. Addition of spironolactone during continued administration of 20 
      mg per day of hydrocortisone resulted in a decrease in blood pressure and a rise 
      in serum potassium. These studies suggest that an abnormality in cortisol action 
      or metabolism results in cortisol behaving as a potent mineralocorticoid. These 
      findings may account for this syndrome of apparent mineralocorticoid excess.
FAU - New, M I
AU  - New MI
FAU - Stoner, E
AU  - Stoner E
FAU - DiMartino-Nardi, J
AU  - DiMartino-Nardi J
LA  - eng
GR  - AM-07280/AM/NIADDK NIH HHS/United States
GR  - HD-00072/HD/NICHD NIH HHS/United States
GR  - HL-17749/HL/NHLBI 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  - Clin Exp Hypertens A
JT  - Clinical and experimental hypertension. Part A, Theory and practice
JID - 8207790
RN  - 0 (Mineralocorticoids)
RN  - 27O7W4T232 (Spironolactone)
RN  - 4964P6T9RB (Aldosterone)
RN  - EC 1.1.- (Hydroxysteroid Dehydrogenases)
RN  - EC 1.1.1.146 (11-beta-Hydroxysteroid Dehydrogenases)
RN  - WI4X0X7BPJ (Hydrocortisone)
RN  - ZS9KD92H6V (Metyrapone)
SB  - IM
MH  - 11-beta-Hydroxysteroid Dehydrogenases
MH  - Aldosterone/pharmacology
MH  - Child
MH  - Child, Preschool
MH  - Female
MH  - Humans
MH  - Hydrocortisone/metabolism/pharmacology
MH  - Hydroxysteroid Dehydrogenases/deficiency
MH  - Hypertension/*etiology
MH  - Hypokalemia/*etiology
MH  - Male
MH  - Metyrapone/pharmacology
MH  - Mineralocorticoids/*metabolism
MH  - Spironolactone/pharmacology
MH  - Syndrome
EDAT- 1986/01/01 00:00
MHDA- 1986/01/01 00:01
CRDT- 1986/01/01 00:00
PHST- 1986/01/01 00:00 [pubmed]
PHST- 1986/01/01 00:01 [medline]
PHST- 1986/01/01 00:00 [entrez]
AID - 10.3109/10641968609046592 [doi]
PST - ppublish
SO  - Clin Exp Hypertens A. 1986;8(4-5):751-72. doi: 10.3109/10641968609046592.