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Primary aldosteronism in Klinefelter’s syndrome: two cases

SUMMARY: Primary aldosteronism (PA) is more common than expected. Aberrant adrenal expression of luteinizing hormone (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 wi...

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Autores principales: Seki, Yasufumi, Morimoto, Satoshi, Yoshida, Naohiro, Bokuda, Kanako, Sasaki, Nobukazu, Yatabe, Midori, Yatabe, Junichi, Watanabe, Daisuke, Morita, Satoru, Hata, Keisuke, Yamamoto, Tomoko, Nagashima, Yoji, Ichihara, Atsuhiro
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Bioscientifica Ltd 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6935713/
https://www.ncbi.nlm.nih.gov/pubmed/31841437
http://dx.doi.org/10.1530/EDM-19-0126
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author Seki, Yasufumi
Morimoto, Satoshi
Yoshida, Naohiro
Bokuda, Kanako
Sasaki, Nobukazu
Yatabe, Midori
Yatabe, Junichi
Watanabe, Daisuke
Morita, Satoru
Hata, Keisuke
Yamamoto, Tomoko
Nagashima, Yoji
Ichihara, Atsuhiro
author_facet Seki, Yasufumi
Morimoto, Satoshi
Yoshida, Naohiro
Bokuda, Kanako
Sasaki, Nobukazu
Yatabe, Midori
Yatabe, Junichi
Watanabe, Daisuke
Morita, Satoru
Hata, Keisuke
Yamamoto, Tomoko
Nagashima, Yoji
Ichihara, Atsuhiro
author_sort Seki, Yasufumi
collection PubMed
description SUMMARY: Primary aldosteronism (PA) is more common than expected. Aberrant adrenal expression of luteinizing hormone (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. 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 2-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. 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. LEARNING POINTS: The pathogenesis of primary aldosteronism is still unclear. Expression of luteinizing hormone receptor has been reported in aldosterone-producing adenoma. Serum luteinizing hormone, which is increased in patients with Klinefelter’s syndrome, might contribute to the development of primary aldosteronism.
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spelling pubmed-69357132020-01-06 Primary aldosteronism in Klinefelter’s syndrome: two cases Seki, Yasufumi Morimoto, Satoshi Yoshida, Naohiro Bokuda, Kanako Sasaki, Nobukazu Yatabe, Midori Yatabe, Junichi Watanabe, Daisuke Morita, Satoru Hata, Keisuke Yamamoto, Tomoko Nagashima, Yoji Ichihara, Atsuhiro Endocrinol Diabetes Metab Case Rep Insight into Disease Pathogenesis or Mechanism of Therapy SUMMARY: Primary aldosteronism (PA) is more common than expected. Aberrant adrenal expression of luteinizing hormone (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. 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 2-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. 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. LEARNING POINTS: The pathogenesis of primary aldosteronism is still unclear. Expression of luteinizing hormone receptor has been reported in aldosterone-producing adenoma. Serum luteinizing hormone, which is increased in patients with Klinefelter’s syndrome, might contribute to the development of primary aldosteronism. Bioscientifica Ltd 2019-11-28 /pmc/articles/PMC6935713/ /pubmed/31841437 http://dx.doi.org/10.1530/EDM-19-0126 Text en © 2019 The authors http://creativecommons.org/licenses/by-nc-nd/4.0/ This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. (http://creativecommons.org/licenses/by-nc-nd/4.0/) .
spellingShingle Insight into Disease Pathogenesis or Mechanism of Therapy
Seki, Yasufumi
Morimoto, Satoshi
Yoshida, Naohiro
Bokuda, Kanako
Sasaki, Nobukazu
Yatabe, Midori
Yatabe, Junichi
Watanabe, Daisuke
Morita, Satoru
Hata, Keisuke
Yamamoto, Tomoko
Nagashima, Yoji
Ichihara, Atsuhiro
Primary aldosteronism in Klinefelter’s syndrome: two cases
title Primary aldosteronism in Klinefelter’s syndrome: two cases
title_full Primary aldosteronism in Klinefelter’s syndrome: two cases
title_fullStr Primary aldosteronism in Klinefelter’s syndrome: two cases
title_full_unstemmed Primary aldosteronism in Klinefelter’s syndrome: two cases
title_short Primary aldosteronism in Klinefelter’s syndrome: two cases
title_sort primary aldosteronism in klinefelter’s syndrome: two cases
topic Insight into Disease Pathogenesis or Mechanism of Therapy
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6935713/
https://www.ncbi.nlm.nih.gov/pubmed/31841437
http://dx.doi.org/10.1530/EDM-19-0126
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