Aldosterone Secretion Rate in Congenital Adrenal Hyperplasia. A Discussion of the Theories on the Pathogenesis of the Salt-losing Form of the Syndrome*

Kowarski, A. Avinoam; Finkelstein, Jordan W.; Spaulding, John S.; Holman, Gerald H.; Migeon, Claude J.

doi:10.1172/jci105257

Cited by 83 publications

(23 citation statements)

References 21 publications

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“…If sequence A were responsible for the overproduction of aldosterone, an increase in dietary sodium alone should have little effect on the overabundance of precursors to the biogenesis of aldosterone and thus should not decrease its production so effectively. Accordingly, both lines of evidence favor sequence B; these results lend indirect support to the hypothesis that "sodium-losing" steroids (such as progesterone) play an important role in the increased aldosterone production in the "non-salt-losing" adrenogenital syndrome as previously suggested (8).…”

Section: Methodssupporting

confidence: 76%

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Aldosterone hypersecretion in “non-salt-losing” congenital adrenal hyperplasia

Bartter¹,

Henkin²,

Bryan³

1968

J. Clin. Invest.

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A B S T R A C T Patients with the "non-salt-losing" form of the adrenogenital syndrome were studied before and after suppression of adrenal cortical activity with carbohydrate-active steroids. The response of aldosterone secretion to sodium deprivation was measured; in some patients response to adrenocorticotropic hormone (ACTH) was measured as well.The aldosterone secretion was normal and responded normally to sodium deprivation in all patients studied during suppression with carbohydrate-active steroids. This finding suggests that 21-hydroxylation of progesterone is normal in this syndrome.The sole abnormality in the production of aldosterone in these patients was found to be excessive secretion of aldosterone while they were not receiving suppressive doses of carbohydrate-active steroids. This finding strongly supports the view that the biogenetic pathways through which aldosterone is produced from progesterone are intact in this syndrome.No patient showed hypertension or hypokalemic alkalosis despite very high aldosterone secretion rates. This observation suggests that the hyperaldosteronism is secondary to a tendency to sodium loss in the patient whose ACTH production is not suppressed.

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Section: Methodssupporting

confidence: 76%

“…DISCUSSION It is clear from the evidence presented and from previous studies (8,9) that the biosynthetic pathways for aldosterone production are not defective in the "non-salt-losing" form of the adrenogenital syndrome. Thus (9).…”

Section: Methodsmentioning

confidence: 60%

Aldosterone hypersecretion in “non-salt-losing” congenital adrenal hyperplasia

Bartter¹,

Henkin²,

Bryan³

1968

J. Clin. Invest.

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“…The ASR values of our six children can be regarded as on the high side. Above normal values for ASR in nonsalt losers have also been found in two of three cases by JAILER et al [23], and high values have also been reported by Ko-WARSKI et al [25] and by BARTTER et al [3].…”

Section: Loras Haour and Bertrandsupporting

confidence: 57%

“…I) Steroid therapy brings a fall in the aldosterone secretion rate (3,21,25, and caw nos.3 and 6). 2) A positive relation between the excretion of pregnanetriol in urine and that of aldosterone has been described by HALL and HOKFELT [22] and by MATTOX et al 1281 in both types of CAH.…”

Section: Loras Haour and Bertrandmentioning

confidence: 99%

Exchangeable Sodium and Aldosterone Secretion in Children with Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency

1970

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Aldosterone secretion rates (ASR) and exchangeable body sodium (Na,) have been measured simultaneously in 25 cases of congenital adrenal hyperplasia (CAH) with and without salt loss, both during normal and during low salt intake. The degree of 21 -hydroxylase deficiency was assessed from the cortisol secretion rate (CSR) and urinary excretion of pregnanetriol and 11-ketopregnanetriol. In cases with salt loss, the sodium equilibrium was unstable, even with optimum treatment and increased dietary salt intake. Clinical evidence of salt-losing crisis appeared when 15-25% of Na, was lost. In these subjects, a parallel decrease in ASR and CSR usually occurred. The ASR were somewhat elevated in the nonsalt-losing form. Speculation

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“…In the untreated state with volume depletion, the increased PRA is an index of MCH deficiency (Schambelan et al, 1972a) and of inadequate replacement of salt and MCHs. (Bongiovanni & Root, 1963) (a) 21-Hydroxylation deficiency (salt-losing type) : In this virilizing form of congenital adrenal hyperplasia, aldosterone deficiency results from interruption of the conversion of biosynthetic precursors to aldosterone at the progesterone to DOC step (Kowarski et al, 1965;Bryan et af., 1965). Volume depletion with hyperkalaemia is a constant feature of this type of congenital adrenal hyperplasia.…”

Section: Primary Adrenocortical Disordersmentioning

confidence: 99%

A Perspective on Aldosterone Abnormalities

Biglieri

1976

Clinical Endocrinology

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Not all the varied clinical disorders in which aldosterone and the mineralocorticoid hormones are involved have been reviewed. Only those disorders in which the mineralocorticoid hormones and their regulatory factors are the principal cause of the biochemical and clinical abnormalities have been examined. These are many and varied. Appreciation of the extent and magnitude of their involvement in the regulation of blood pressure, body fluids, and electrolyte composition continues to grow.The major direct clinical impact of the mineralocorticoid hormones appears to be in two areas: hypertension and potassium homeostasis. Their part in the mosaic of hypertension is established in primary hyperaldosteronism, but they also appear to affect and modify the hypertensive process in primary or essential hypertension. The probe continues.Hypoaldosteronism is more than the rare occurrence associated with Addison's disease. It may be the clue to the presence of nonaldosterone mineralocorticoid excess syndromes, and is obviously of critical importance in an increasing number of patients with chronic renal failure of varied aetiologies.The principal mineralocorticoid hormones (MCHs) of the adrenal cortex are deoxycorticosterone (DOC) and aldosterone. Deoxycorticosterone at normal levels binds to corticosteroid-binding globulin and has weak sodium-retaining and potassium-secreting properties and therefore contributes little to total mineralocorticoid activity, whereas aldosterone is free of binding and thus is physiologically the more potent MCH.Abnormalities in sodium and potassium metabolism, volume regulation, and blood pressure are the clinical conditions in which disorders of aldosterone secretions are thought to have a role. In this article, alterations of aldosterone production in disorders of blood pressure and potassium metabolism will be discussed.

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Aldosterone Secretion Rate in Congenital Adrenal Hyperplasia. A Discussion of the Theories on the Pathogenesis of the Salt-losing Form of the Syndrome*

Cited by 83 publications

References 21 publications

Aldosterone hypersecretion in “non-salt-losing” congenital adrenal hyperplasia

Aldosterone hypersecretion in “non-salt-losing” congenital adrenal hyperplasia

Exchangeable Sodium and Aldosterone Secretion in Children with Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency

A Perspective on Aldosterone Abnormalities

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