The Role of Renin and Angiotensin in Salt-Losing, 21- Hydroxylase-Deficient Congenital Adrenal Hyperplasia*

Horner, James M.; Hintz, Raymond L.; Luetscher, John A.

doi:10.1210/jcem-48-5-776

Cited by 47 publications

(16 citation statements)

References 20 publications

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“…This hypothesis fits both the clinical observations and the known molecular biology of 21 -hydroxylase. It must now be tested directly by determining the molecular identity of extraadrenal 2 1-hydroxylase(s).…”

Section: Discussionsupporting

confidence: 86%

“…Some patients who have been described having severe, salt-wasting 21 -hydroxylase deficiency nonetheless recover the capacity to make enough mineralocorticoids to avoid a saltlosing crisis in adult life (21,22). While this encouraged speculation that the enzymes mediating the 2 1-hydroxylation of progesterone to deoxycorticosterone and of 170HP to 11-deoxycortisol were different (22), the demonstration that there is only one P450c21 involved in adrenal 2 1-hydroxylation has ruled this out (2)(3)(4).…”

Section: Discussionmentioning

confidence: 99%

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Extraadrenal steroid 21-hydroxylation is not mediated by P450c21.

Mellon¹,

Miller²

1989

J. Clin. Invest.

119

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Section: Discussionsupporting

confidence: 86%

Section: Discussionmentioning

confidence: 99%

Extraadrenal steroid 21-hydroxylation is not mediated by P450c21.

Mellon¹,

Miller²

1989

J. Clin. Invest.

119

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“…There is general agreement that PRA is increased in untreated salt-losing CAH (6,14,18) and that mineralocorticoid therapy results in lowering of these values (6,14,8). In the present study, a highly significant correlation was demonstrated between sodium depletion or serum sodium concentrations and PRA.…”

Section: Discussionsupporting

confidence: 74%

“…It has been demonstrated that a number of subjects with saltlosing CAH, when hyperreninemic, are able to secrete aldosterone in significant quantities (6,10). In subjects without adrenal pathology, it is well known that sodium depletion due to diet or certain diuretics (13) and a reduction in blood volume due to hemorrhage can lead to increased PRA.…”

Section: Subsequent Pelletsmentioning

confidence: 99%

“…Salt-losing congenital adrenal hyperplasia (SL-CAH) is an inherited defect in steroid C 21-hydroxylation, characterized by deficiency in the secretion of cortisol, hypersecretion of androgens and progestins (2), and complete (3,12) or partial (6,10) defects in the synthesis of aldosterone. Salt-wasting in this disorder appears to be the result both of defective aldosterone synthesis and of increased production of steroids which antagonize the effects of aldosterone (9,10).…”

mentioning

confidence: 99%

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Plasma Renin Activity and the Response to Sodium Depletion in Salt-Losing Congenital Adrenal Hyperplasia

et al. 1982

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SummaryMineralocorticoid therapy was discontinued in 14 subjects, ages 7-17 years, with salt-losing congenital adrenal hyperplasia. The level of plasma renin activity (PRA) was determined in relation to the cumulative loss of sodium (intake-urinary output) over 4-12 days. Although the magnitude and rate of response varied, PRA increased in all subjects. There was a significant positive correlation between cumulative sodium loss and PRA (r = 0.585, P < 0.001, n = 76), and a significant negative correlation between serum sodium concentration and PRA (r = -0.736, P < 0.001, n = 76). Significant sodium loss and elevation of PRA were seen prior to the development of hyponatremia. Thus, elevated PRA appeared to be a more sensitive indicator of sodium loss than serum sodium concentration.Fifteen infants, ages 1-33 months with salt-losing congenital adrenal hyperplasia, who were treated with subcutaneous pellets of 11-deoxycorticosterone acetate (DOCA), were also studied. PRA decreased following implantation of the DOCA pellets over 1-6 months. In most instances there was a subsequent increase in PRA, beginning at 1-8 months post implantation. There was a significant negative correlation between serum sodium and PRA (r = -0.669, P < 0.001, n = 78) in these infants. However, there were four instances in which elevated PRA was associated with normal serum sodium.Sodium balance studies were also performed on 14 of these infants during 29 admissions. All balance studies were performed at approximately 3 or 6 months after DOCA pellet implantation. On a diet containing 2 mEq Na/kg body weight per day, sodium balance was positive and there was no significant change in sodium excretion over the 3 days of observation. When a 1 mEq Na/kg/ day diet was given, sodium balance was neutral on day 3 of the study, and fractional urinary sodium excretion (FeN,) decreased from 0.49 + 0.33, S.D. on day 1 to 0.23 + .09% on day 3 ( P < 0.001, n = 19). This change was statistically significant. Net sodium balance on either diet was independent of PRA. Elevated PRA was not necessarily associated with adverse symptomatology.Thus in infants treated with DOCA pellets, elevated PRA was associated with indices of sodium depletion and decreased availability of DOCA. The dietary sodium requirement for infants treated with DOCA appeared to be 1-2 mEq/kg body weight/day.

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States of Aldosterone Deficiency or Pseudodeficiency

Schambelan¹,

Sebastián²

1987

Hormone Resistance and Other Endocrine Paradoxes

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The Role of Renin and Angiotensin in Salt-Losing, 21- Hydroxylase-Deficient Congenital Adrenal Hyperplasia*

Cited by 47 publications

References 20 publications

Extraadrenal steroid 21-hydroxylation is not mediated by P450c21.

Extraadrenal steroid 21-hydroxylation is not mediated by P450c21.

Plasma Renin Activity and the Response to Sodium Depletion in Salt-Losing Congenital Adrenal Hyperplasia

States of Aldosterone Deficiency or Pseudodeficiency

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