Adrenal Steroidogenesis in Inappropriate High Blood Pressure

Martins, João Martin; Vale, Sónia do; Carrilho, Bruno; Saldanha, Clyde; Silva, J. Martins e

doi:10.1097/00004872-200406002-00659

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“…Several remarks may be made at this point: 1) ACTH dependence in the context of ambulatory testing is generally considered to suggest an adenoma [2][3][4]. 2) A glucocorticoid remediable hyperaldosteronism could have been suspected; however this is rare, less than 1% of all cases of primary aldosteronism, and clinical presentation is different with a positive family history and early onset of severe high blood pressure refractory to conventional treatment [2][3][4]; although genetic testing or the measurement of 18-hydroxycortisol or 18-oxyocortisol were not available and were not performed, we suppose this is not the case; in fact we have previously found this marked ACTHdependence to be very common in patients with minor/late onset forms of congenital adrenal hyperplasia [27]; the fact remains however of a marked ACTH-dependence for aldosterone secretion.…”

Section: Discussionmentioning

confidence: 90%

“…We selected to perform a Synacthen test that is generally not used n this specific diagnostic work out. Based on our personal previous experience we wanted to exclude minor/ late onset forms of congenital adrenal hyperplasia and also to evaluate the ACTH-dependence of aldosterone secretion [27]. The tetracosactide test revealed both a possible minor 21-hydroxylase defect [28] and a marked aldosterone response to synthetic ACTH.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Primary Hyperaldosteronism: Pitfalls in the Diagnosis and a not so Peculiar Evolution - Case Report

Martins

2012

J Endocrinol Metab

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Primary hyperaldosteronism is now assumed to account for 10-15% of all cases of non selected high blood pressure. The differential diagnosis between bilateral hyperplasia and unilateral adenoma remains however a medical challenge, and the pathogenic stimulus for hyperplasia is still unknown. HS, a female patient aged 32, presented with High Blood Pressure and hypokalemia. An elevated aldosterone/renin ratio and non-suppressed aldosterone levels after saline infusion confirmed the diagnosis of primary hyperaldosteronism. Negative adrenal scintigraphic examination and a marked aldosterone response in the postural test supported the diagnosis of bilateral hyperplasia, despite the presence of left adrenal nodule in the CT-scan. Tetracosactide stimulation revealed a minor 21-hydroxilase defect and a marked ACTH-dependence of aldosterone secretion. Effective treatment with dexamethasone 1.0 mg every other night was maintained for 9 years, while in the meantime the left adrenal nodule apparently resolved and a right adrenal nodule become apparent. Finally worsening of blood pressure levels and increasing aldosterone concentrations with a positive scintigraphic labelling on the right adrenal gland led to right adrenalectomy that was so far curative. This case report demonstrates bilateral hyperplasia-adenoma evolution and suggests ACTH to be the relevant pathogenic stimulus, allowing for effective long-term treatment with dexamethasone.

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