Clinical analysis of familial adrenocorticotropin-independent macronodular adrenal hyperplasia
- VernacularTitle:家族性ACTH非依赖性肾上腺大结节样增生的临床分析
- Author:
Shiwu CHENG
;
Juming LU
;
Zhaohui LV
- Publication Type:Journal Article
- Keywords:
Cushing syndrome;
corticotrophin;
macronodular adrenal hyperplasia
- From:
Medical Journal of Chinese People's Liberation Army
1981;0(04):-
- CountryChina
- Language:Chinese
-
Abstract:
Objective To analyze the clinical characteristics of familial adrenocorticotropin-independent macronodular adrenal hyperplasia (AIMAH). Methods The clinical and laboratory data of 3 patients with familial AIMAH were retrospectively analyzed. Results Case 1 was the proband. The mean age of onset of familial AIMAH was 59.3 years, and mean duration of disease was 6.7 years. The plasma ACTH levels of case 1 and case 2 were below 2.2pmol/L, and the secretion rhythm of serum cortisol in them was disorderly. Low or high dose of dexamethasone failed to suppress cortisol secretion in case 1, while only low dose of dexamethasone failed to suppress cortisol secretion in case 2. In case 3, all the plasma cortisol, ACTH level and their secretion rhythm were normal, and either low or high dose of dexamethasone suppressed cortisol secretion successfully. Ultrasound examination revealed multiple hypoechoic nodules in both adrenal glands, and CT scanning showed bilateral macronodular adrenal hyperplasia in all 3 cases. Pituitary MR imaging was normal in all 3 cases. Conclusions The pathogenesis of sporadic and familial AIMAH remains unclear. Familial AIMAH provides an evidence that genetic transmission of the disease may happen. The clinical characteristics of familial AIMAH are similar to those of sporadic AIMAH. It is possible that some subclinical cases among familial AIMAH ascape the diagnosis.
