Pseudo-Addison's disease. Isolated corticotropin deficiency associated with hyporeninemic hypoaldosteronism

Manser, Thomas; Estep, Herschel L.

doi:10.1001/archinte.146.5.996

Cited by 3 publications

(4 citation statements)

References 5 publications

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“…Although a few studies have been reported, the system seems to be maintained adequately in the isolated ACTH deficiency syndrome [1], Only a single case of hyporeninemic hypoaldosteronism associated with this syndrome has been reported. Unfortunately, a detailed study on aldosterone regulation has not been performed [10].…”

Section: Discussionmentioning

confidence: 99%

Normoreninemic Hypoaldosteronism in a Case of Isolated ACTH Deficiency

Miyabo¹,

Minami²,

Inazu³

et al. 1989

Horm Res

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This paper documents the rare and hitherto unreported association between isolated ACTH deficiency and normoreninemic hypoaldosteronism in a 63-year-old woman. Baseline plasma aldosterone and 18-hydroxycorticosterone were extremely low. Both steroids did not respond to exogenous angiotensin II infusion, whereas they were increased in parallel to ACTH stimulation. Thus, acquired dysfunction or congenital dysgenesis of the zona glomeruk > sa was suspected. The upright posture-furosemide test showed a subnormal but definite plasma aldosterone response coupled with a normal increase in plasma renin activity, indicating that there may be a yet unidentified mechanism(s) underlying the postural increase of aldosterone.

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Section: Discussionmentioning

confidence: 99%

Normoreninemic Hypoaldosteronism in a Case of Isolated ACTH Deficiency

Miyabo¹,

Minami²,

Inazu³

et al. 1989

Horm Res

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“…As far as we know, isolated ACTH deficiency with hyporeninemic hy poaldosteronism after the administration of GC has not been reported so far. Although Manser and Estep [13] reported a case of isolated ACTH deficiency with hvporeninemic hypoaldosteronism, renin release stimulation test was performed without replacement therapy. On the other hand.…”

Section: Fig 2 the Comparison Of Urine Volume (Uv)mentioning

confidence: 99%

A Case of Isolated ACTH Deficiency with Hyporeninemic Hypoaldosteronism

Okada

Takahashi²

1996

Nephron

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A case of isolated ACTH deficiency with hyporeninemic hypoaldosteronism, presenting severe hyponatremia, is described. A 57-year-old man complaining of nausea, vomiting and fatigability was admitted to our hospital because of hyponatremia (114 mEq/l). The low levels of serum cortisol and urinary 17-OHCS suggested glucocorticoid deficiency, and that the glucocorticoid deficiency was due to isolated ACTH deficiency was confirmed by a continuous ACTH loading test and pituitary gland stimulation tests. Although the low level of serum sodium was normalized after the administration of cortisone acetate (50 mg/day) combined with an increase in oral salt intake, urinary sodium loss persisted by the results of hypertonic saline infusion test. Treatment led to improvement of impairment of water diuresis due to hypersecretion of ADH. Hyporeninemic hypoaldosteronism persisted after treatment. We have shown that severe hyponatremia that occurs with combined deficiency of glucocorticoids and mineralocorticoids can be corrected with high salt intake and glucocorticoid replacement without correcting mineralocorticoid deficiency.

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“… HA.14 reports, 33 patients, plus an unreported case of our own 8–21 . Twenty‐two of these patients had associated renal impairment and two also had PHA.…”

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confidence: 94%

“… Plasma aldosterone and potassium in patients obtained from patient reports, defined with either HA 8–21 or RAR 3,18,22–37 according to criteria outlined in the text. The defined lower limit was set from the data in Fig.…”

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confidence: 99%