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      SUN-161 Primary Aldosteronism and Klinefelter’s Syndrome: Two Cases

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          Abstract

          Background: Primary aldosteronism (PA) is more common than expected. Aberrant adrenal expression of LH receptor in patients with PA has been reported, however, its physiological role on the development of PA is still unknown. Herein, we report two unique cases of PA in patients with untreated Klinefelter’s syndrome, characterized as increased serum LH, suggesting a possible contribution of the syndrome to PA development. Clinical Cases: Case 1 was a 39-year-old man with obesity and hypertension since his 20s. His plasma aldosterone concentration (PAC) and renin activity (PRA) were 220 pg/mL and 0.4 ng/mL/h, respectively. He was diagnosed as having bilateral PA by confirmatory tests and adrenal venous sampling (AVS). Klinefelter’s syndrome was suspected as he showed gynecomastia and small testes, and it was confirmed on the basis of a low serum total testosterone level (57.3 ng/dL), high serum LH level (50.9 mIU/mL), and chromosome analysis. Case 2 was a 28-year-old man who had untreated Klinefelter’s syndrome diagnosed in his childhood and a two-year history of hypertension and hypokalemia. PAC and PRA were 247 pg/mL and 0.3 ng/mL/h, respectively. He was diagnosed as having a 10 mm-sized aldosterone-producing adenoma (APA) by AVS. In the APA, immunohistochemical analysis showed co-expression of LH receptor and CYP11B2. Conclusion: Our cases of untreated Klinefelter’s syndrome complicated with PA suggest that increased serum LH levels and adipose tissues, caused by primary hypogonadism, could contribute to PA development. The possible complication of PA in hypertensive patients with Klinefelter’s syndrome should be carefully considered.

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          Author and article information

          Journal
          J Endocr Soc
          J Endocr Soc
          jes
          Journal of the Endocrine Society
          Oxford University Press (US )
          2472-1972
          08 May 2020
          08 May 2020
          08 May 2020
          : 4
          : Suppl 1 , ENDO 2020 ABSTRACTS SCHEDULED FOR THE ANNUAL MEETING OF THE ENDOCRINE SOCIETY – MARCH 28 – 31, 2020 - SAN FRANCISCO, CALIFORNIA (CANCELLED)
          Affiliations
          [1 ] Kanto Central Hospital , Tokyo, Japan
          [2 ] Tokyo Women’s Medical University , Tokyo, Japan
          Article
          bvaa046.066
          10.1210/jendso/bvaa046.066
          7207820
          © Endocrine Society 2020.

          This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs licence ( http://creativecommons.org/licenses/by-nc-nd/4.0/), which permits non-commercial reproduction and distribution of the work, in any medium, provided the original work is not altered or transformed in any way, and that the work is properly cited. For commercial re-use, please contact journals.permissions@oup.com

          Page count
          Pages: 2
          Product
          Categories
          Adrenal
          Adrenal Case Reports II
          AcademicSubjects/MED00250

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