医学
双侧肾上腺切除术
肾上腺切除术
病理生理学
增生
库欣综合征
疾病
粘液瘤
病理
外科
内科学
内分泌学
生物
生物化学
基因
作者
Léamarie Meloche-Dumas,Frédéric Mercier,André Lacroix
标识
DOI:10.1016/j.beem.2021.101486
摘要
Primary bilateral adrenocortical hyperplasias are rare forms of pituitary ACTH-independent Cushing’s syndrome (CS). They are divided between primary bilateral macronodular adrenal hyperplasia (PBMAH) and micronodular adrenal hyperplasia (MiBAH), which is subdivided in primary pigmented nodular adrenocortical disease (PPNAD) and isolated micronodular adrenocortical disease (i-MAD). One of the most debated aspects surrounding these entities is their most appropriate therapy. Although bilateral adrenalectomy (BA) has previously been the most utilized therapy for patients with overt CS, recent studies have indicated that unilateral adrenalectomy (UA) can be effective in patients with PBMAH and some with MiBAH with fewer long-term side effects. Medical therapies can also be used for bridging to surgery or rarely in the long-term for these patients. We review the various degrees of CS resulting from PBMAH and MiBAH, with a special focus on their respective therapies including UA, taking into account the recent pathophysiological and genetics findings.
科研通智能强力驱动
Strongly Powered by AbleSci AI