Diagnostic and therapeutic approaches to a case of pregnancy complicated by bilateral adrenocortical adenomas with primary aldosteronism and Cushing's syndrome.

Autor: Chen Y; Department of Endocrinology and Metabolism, Adrenal center, West China Hospital, Sichuan University, Chengdu, China., Tan L; Department of Endocrinology and Metabolism, Adrenal center, West China Hospital, Sichuan University, Chengdu, China., Chen T; Department of Endocrinology and Metabolism, Adrenal center, West China Hospital, Sichuan University, Chengdu, China., Tian H; Department of Endocrinology and Metabolism, Adrenal center, West China Hospital, Sichuan University, Chengdu, China., Li L; Institute of Clinical Pathology, West China Hospital, Sichuan University, Chengdu, China., Ren Y; Department of Endocrinology and Metabolism, Adrenal center, West China Hospital, Sichuan University, Chengdu, China. renyan@scu.edu.cn.
Jazyk: angličtina
Zdroj: Endocrine [Endocrine] 2024 Oct 08. Date of Electronic Publication: 2024 Oct 08.
DOI: 10.1007/s12020-024-04058-x
Abstrakt: Aldosterone/cortisol co-secreting adenomas (A/CPA) are a rare type of primary aldosteronism(PA), and cases of aldosterone/cortisol co-secreting adenomas during pregnancy are extremely rare, with no reported cases to date. The unique physiological state of pregnancy increases cortisol secretion through the hypothalamic-pituitary-adrenal (HPA) axis and leads to elevated levels of all components of the renin-angiotensin-aldosterone system (RAAS). This can cause overlapping symptoms with abnormal cortisol and aldosterone secretion, making diagnosis very challenging. This case involves a 29-year-old woman who developed hypercortisolism at 33 weeks of pregnancy. Despite receiving treatment for her symptoms and having a successful delivery, she continued to experience hypertension and hypokalaemia after giving birth. Eventually, she was diagnosed with ACTH-independent Cushing's syndrome and primary aldosteronism due to independent cortisol and aldosterone secretion from bilateral adrenal adenomas. Following a thorough diagnosis, classification, treatment, and follow-up, the patient achieved a clinical cure while preserving normal adrenal function. Further investigation revealed that both diseases were caused by KCNJ5 and PRKACA mutations found in the bilateral adrenal adenomas.
(© 2024. The Author(s).)
Databáze: MEDLINE
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