Mineralocorticoid status and endocrine dysfunction in severe hemochromatosis.
Selective iron deposition in the zona glomerulosa of the adrenal cortex is observed in hemochromatosis. Hypoaldosteronism should be excluded before starting venesection, to avoid long-term volume depletion. We evaluated the aldosterone status in patients with hemochromatosis. As other endocrine organs can be affected by the disease as well, we simultaneously evaluated anterior pituitary, gonadal, thyroid and pancreatic beta-cell activity. Nine patients with hereditary or acquired hemochromatosis and highly increased plasma ferritin levels were investigated. In patients, liver cirrhosis had been confirmed histologically. Five patients complained of sexual dysfunction, and one had impaired glucose tolerance. Plasma aldosterone (PA) and renin activity (PRA) were measured after a period of normal (100 mmol/day) and low (10 mmol/day) sodium intake. A combined anterior pituitary function test and a glucagon stimulation test were undertaken to evaluate other endocrine functions. Both PA and PRA levels were decreased in one patient with liver cirrhosis, who also presented attenuated cortisol, prolactin and gonadotrophin secretion. No patients had signs of primary hypoaldosteronism with hyperreninemia. Hypogonadotropic hypogonadism was observed in 3 males and 1 female. Pituitary ACTH reserve was impaired in 2, GH and prolactin response in 1, and thyroid function in none of the patients. Glucagon-stimulated plasma C-peptide was impaired in one patient. In conclusion, primary aldosterone deficiency was not observed in patients with severe iron overload. Hyporeninemic hypoaldosteronism was found in one patient who also presented other endocrinopathies. Hypogonadotropic hypogonadism is the most frequent endocrine abnormality in hemochromatosis.
Hempenius
L M
LM
Department of Endocrinology, University Hospital Utrecht, The Netherlands.
Van Dam
P S
PS
Marx
J J
JJ
Koppeschaar
H P
HP
D000311:Adrenal Glands / Q000503:physiopathology; D000328:Adult; D000368:Aged; D000450:Aldosterone / Q000097:blood*; D004702:Endocrine Glands / Q000503:physiopathology*; D005260:Female; D005293:Ferritins / Q000097:blood; D005934:Glucagon; D013007:Growth Hormone-Releasing Hormone; D006432:Hemochromatosis / Q000503:physiopathology*; D006801:Humans; D007515:Islets of Langerhans / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D010053:Ovary / Q000503:physiopathology; D010903:Pituitary Gland, Anterior / Q000503:physiopathology; D011187:Posture; D012083:Renin / Q000097:blood; D012964:Sodium / Q000097:blood; D013737:Testis / Q000503:physiopathology; D013961:Thyroid Gland / Q000503:physiopathology
D000450:Aldosterone; D005293:Ferritins; D005934:Glucagon; D013007:Growth Hormone-Releasing Hormone; D012964:Sodium; D012083:Renin