Autor: |
Yi-Ju Chen, Kang-Yung Peng, Jeff S. Chueh, Hung-Wei Liao, Tsung-Yi Hsieh, Vin-Cent Wu, Shuo-Meng Wang |
Jazyk: |
angličtina |
Rok vydání: |
2022 |
Předmět: |
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Zdroj: |
Frontiers in Endocrinology, Vol 13 (2022) |
Druh dokumentu: |
article |
ISSN: |
1664-2392 |
DOI: |
10.3389/fendo.2022.816754 |
Popis: |
BackgroundNon-classical multiple aldosterone-producing micronodules/nodules (mAPM/mAPN) could be the pathogenesis of primary aldosteronism (PA). The co-existence of mAPM with adenomas harboring somatic mutations has not previously been reported.MethodsWe presented a PA patient with bilateral mAPM and concomitant autonomous cortisol secretion (ACS).ResultsA 46-year-old Taiwanese woman presented with hypertension, hypokalemia, and bilateral adrenal adenomas. A 1 mg low-dose dexamethasone suppression test showed elevated morning serum cortisol. An adrenal vein sampling (AVS) suggested a left-sided lateralization of hyperaldosteronism. A right partial adrenalectomy and a left total adrenalectomy were performed. The patient showed biochemical and hypertension remission after the operation. This patient had bilateral mAPM with concomitant ACS, a right histopathologically classical PA adenoma, and a left non-classical PA adenoma. The right adrenal adenoma showed CYP11B1-negative and CYP11B2-positive staining and harbored the KCNJ5-L168R mutation. The left adrenal adenoma showed CYP11B1-positive and CYP11B2-negative staining and harbored the PRKACA-L206R mutation.ConclusionIn a PA patient with concomitant ACS, bilateral APM could coexist with both histopathologically classical and non-classical PA adenomas, each with different somatic mutations. The presence of ACS could lead to the misinterpretation of AVS results. |
Databáze: |
Directory of Open Access Journals |
Externí odkaz: |
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