Vasoactive hormones in familial pheochromocytoma with predominant norepinephrine secretion.
A family was investigated with an increased occurrence of pheochromocytoma. Three males out of 7 family members showed biochemical and morphological evidence of catecholamine-secreting tumor. Plasma concentrations of norepinephrine, aldosterone, arginine vasopressin and renin activity, as well as urinary excretion of norepinephrine, were enhanced in all the 3 patients. The plasma level of atrial natriuretic peptide decreased. After surgical removal of the tumor the hormonal alterations tended to be normalized; however, plasma concentration of norepinephrine remained above normal. The elevated plasma norepinephrine was postoperatively accompanied by slightly increased urinary norepinephrine excretion in 1 patient without any detectable tumor. The severity of the disease was most pronounced in the youngest patient, while in the oldest one a 'silent' pheochromocytoma was found. The occurrence of familial pheochromocytoma in successive generations may suggest an autosomal-dominant inheritance.
Tulassay
T
T
First Department of Pediatrics, Semmelweis University Medical School, Budapest, Hungary.
Verebély
T
T
Szücs
L
L
Rascher
W
W
Jellinek
K
K
Reusz
G
G
Miltényi
M
M
Nagy
I
I
D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000097:blood* / Q000235:genetics / Q000652:urine; D000450:Aldosterone / Q000097:blood; D006801:Humans; D008297:Male; D008875:Middle Aged; D009638:Norepinephrine / Q000097:blood* / Q000652:urine; D010673:Pheochromocytoma / Q000097:blood* / Q000235:genetics / Q000652:urine; D011184:Postoperative Period; D012083:Renin / Q000097:blood; D014667:Vasopressins / Q000097:blood
D014667:Vasopressins; D000450:Aldosterone; D012083:Renin; D009638:Norepinephrine