Preclinical Cushing's syndrome due to ACTH-independent bilateral macronodular adrenocortical hyperplasia with excessive secretion of 18-hydroxydeoxycorticosterone and corticosterone.
A 64-year-old woman developed hypertension and hypokalemia, due to ACTH-independent bilateral macronodular adrenocortical hyperplasia (AIMAH) with excessive secretion of 18-hydroxydeoxycorticosterone and corticosterone. Plasma cortisol did not show a diurnal rhythm, and was not suppressed by dexamethasone (8 mg). Plasma cortisol responded to ACTH and was increased by hypoglycemia without modifying ACTH levels. Radiological studies demonstrated that adrenal glands were enlarged with macronodules. Although the patient exhibited a low plasma renin activity and aldosterone levels, hypokalemia and hypertension were observed. Hormonal findings would support the hypothesis that the tumor of AIMAH originated from cells of the upper zona fasciculata.
Wada
Seiki
S
Fourth Department of Internal Medicine, Saitama Medical School.
Kitahama
Shinji
S
Togashi
Atsuhito
A
Inoue
Kiyoaki
K
Iitaka
Makoto
M
Katayama
Shigehiro
S
D015070:18-Hydroxydesoxycorticosterone / Q000378:metabolism*; D000301:Adosterol; D000306:Adrenal Cortex Neoplasms / Q000150:complications* / Q000000981:diagnostic imaging; D000311:Adrenal Glands / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology*; D003345:Corticosterone / Q000378:metabolism*; D003480:Cushing Syndrome / Q000000981:diagnostic imaging / Q000209:etiology* / Q000378:metabolism; D003940:Diagnostic Techniques, Endocrine; D005260:Female; D006801:Humans; D006965:Hyperplasia; D006973:Hypertension / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D007008:Hypokalemia / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D011877:Radionuclide Imaging; D015898:Tomography Scanners, X-Ray Computed
D015070:18-Hydroxydesoxycorticosterone; D000301:Adosterol; D003345:Corticosterone