An unusual case of an aldosterone-producing adenoma in a prepubertal girl with severe post-surgical adrenal suppression.
An 11-year-old girl was referred with hypertension, hypokalemia, low renin activity, metabolic alkalosis and hyperaldosteronism. Adrenal computed tomography revealed the presence of a large left adrenal adenoma of 4 x 4.5 cm in size. Following adrenalectomy, the child presented an unusual acute salt-loosing syndrome which necessitated administration of a large quantity of sodium chloride and corticoids. The cells from the tumor were plated. They rapidly proliferated to a monolayer. After incubation for 24 h, basal production of aldosterone (55.4 +/- 9.07 pmol/ml) was observed. This production was stimulated by the presence of ACTH 10(-8) M and KCl 55 mM; angiotensin II 10(-8) M failed to enhance aldosterone secretion. In the four experimental conditions, however (control, ACTH, angiotensin II and KCl), ANF 10(-8) M decreased aldosterone secretion. We conclude that hyperaldosterone secretion by an adrenal adenoma is not due to resistance to the inhibitory effect of ANF but rather to cell multiplication itself. In this patient, the post-surgical salt-loosing syndrome was attributed to the combined effect of the chronic state of high ANF, low potassium and low ACTH in the contralateral gland on the one hand and a certain degree of transitory renal unresponsiveness of the renal distal tubule to aldosterone on the other.
Legault
L
L
Maisonneuve-Rosemont Hospital, Montreal, Canada.
De Lean
A
A
Brunette
M G
MG
D000236:Adenoma / Q000150:complications / Q000378:metabolism* / Q000473:pathology / Q000601:surgery; D000310:Adrenal Gland Neoplasms / Q000150:complications / Q000378:metabolism* / Q000473:pathology / Q000601:surgery; D000450:Aldosterone / Q000096:biosynthesis* / Q000097:blood; D002648:Child; D005260:Female; D006801:Humans; D007010:Hyponatremia / Q000209:etiology*; D011183:Postoperative Complications; D014407:Tumor Cells, Cultured
D000450:Aldosterone