Endocrinology and Metabolism

Case Report

Adrenal gland
Untreated Congenital Adrenal Hyperplasia with 17-α Hydroxylase/17,20-Lyase Deficiency Presenting as Massive Adrenocortical Tumor: Su Jin Lee, Je Eun Song, Sena Hwang, Ji-Yeon Lee, Hye-Sun Park, Seunghee Han, Yumie Rhee; Endocrinol Metab. 2015;30(3):408-413. Published online August 4, 2015; DOI: https://doi.org/10.3803/EnM.2015.30.3.408

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Congenital adrenal hyperplasia (CAH) with 17α-hydroxylase/17,20-lyase deficiency is usually characterized by hypertension and primary amenorrhea, sexual infantilism in women, and pseudohermaphroditism in men. hypertension, and sexual infantilism in women and pseudohermaphroditism in men. In rare cases, a huge adrenal gland tumor can present as a clinical manifestation in untreated CAH. Adrenal cortical adenoma is an even more rare phenotype in CAH with 17α-hydroxylase/17,20-lyase deficiency. A 36-year-old female presented with hypertension and abdominal pain caused by a huge adrenal mass. Due to mass size and symptoms, left adrenalectomy was performed. After adrenalectomy, blood pressure remained high. Based on hormonal and genetic evaluation, the patient was diagnosed as CAH with 17α-hydroxylase/17,20-lyase deficiency. The possibility of a tumorous change in the adrenal gland due to untreated CAH should be considered. It is important that untreated CAH not be misdiagnosed as primary adrenal tumor as these conditions require different treatments. Adequate suppression of adrenocorticotropic hormone (ACTH) in CAH is also important to treat and to prevent the tumorous changes in the adrenal gland. Herein, we report a case of untreated CAH with 17α-hydroxylase/17,20-lyase deficiency presenting with large adrenal cortical adenoma and discuss the progression of adrenal gland hyperplasia due to inappropriate suppression of ACTH secretion.

Citations

Citations to this article as recorded by

Congenital adrenal hyperplasia disorder due to 17 α-hydroxylase deficiency: a case report
Yunling Tian, Lijie Hou, Shulan Xiang, Xuguang Tian, Jinhui Xu
Gynecological Endocrinology.2023;[Epub] CrossRef
Landscape of Adrenal Tumours in Patients with Congenital Adrenal Hyperplasia
Mara Carsote, Ana-Maria Gheorghe, Claudiu Nistor, Alexandra-Ioana Trandafir, Oana-Claudia Sima, Anca-Pati Cucu, Adrian Ciuche, Eugenia Petrova, Adina Ghemigian
Biomedicines.2023; 11(11): 3081. CrossRef
17α-Hydroxylase/17,20-Lyase Deficiency in 46,XY: Our Experience and Review of Literature
Madhur Maheshwari, Sneha Arya, Anurag Ranjan Lila, Vijaya Sarathi, Rohit Barnabas, Khushnandan Rai, Vishwambhar Vishnu Bhandare, Saba Samad Memon, Manjiri Pramod Karlekar, Virendra Patil, Nalini S Shah, Ambarish Kunwar, Tushar Bandgar
Journal of the Endocrine Society.2022;[Epub] CrossRef
17α-hydroxylase Deficiency Mimicking Hyperaldosteronism by Aldosterone-producing Adrenal Adenoma
Yun Kyung Cho, Hyeseon Oh, Sun-myoung Kang, Sujong An, Jin-Young Huh, Ji-Hyang Lee, Woo Je Lee
The Korean Journal of Medicine.2016; 91(2): 191. CrossRef

Original Article

Bone Metabolism
Increased Sclerostin Levels after Further Ablation of Remnant Estrogen by Aromatase Inhibitors: Wonjin Kim, Yoonjung Chung, Se Hwa Kim, Sehee Park, Jae Hyun Bae, Gyuri Kim, Su Jin Lee, Jo Eun Kim, Byeong-Woo Park, Sung-Kil Lim, Yumie Rhee; Endocrinol Metab. 2015;30(1):58-64. Published online March 27, 2015; DOI: https://doi.org/10.3803/EnM.2015.30.1.58

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Abstract PDF PubReader

Background

Sclerostin is a secreted Wnt inhibitor produced almost exclusively by osteocytes, which inhibits bone formation. Aromatase inhibitors (AIs), which reduce the conversion of steroids to estrogen, are used to treat endocrine-responsive breast cancer. As AIs lower estrogen levels, they increase bone turnover and lower bone mass. We analyzed changes in serum sclerostin levels in Korean women with breast cancer who were treated with an AI.

Methods

We included postmenopausal women with endocrine-responsive breast cancer (n=90; mean age, 57.7 years) treated with an AI, and compared them to healthy premenopausal women (n=36; mean age, 28.0 years). The subjects were randomly assigned to take either 5 mg alendronate with 0.5 µg calcitriol (n=46), or placebo (n=44) for 6 months.

Results

Postmenopausal women with breast cancer had significantly higher sclerostin levels compared to those in premenopausal women (27.8±13.6 pmol/L vs. 23.1±4.8 pmol/L, P<0.05). Baseline sclerostin levels positively correlated with either lumbar spine or total hip bone mineral density only in postmenopausal women (r=0.218 and r=0.233; P<0.05, respectively). Serum sclerostin levels increased by 39.9%±10.2% 6 months after AI use in postmenopausal women; however, no difference was observed between the alendronate and placebo groups (39.9%±10.2% vs. 55.9%±9.13%, P>0.05).

Conclusion

Serum sclerostin levels increased with absolute deficiency of residual estrogens in postmenopausal women with endocrine-responsive breast cancer who underwent AI therapy with concurrent bone loss.

Citations

Citations to this article as recorded by

Voluntary Wheel Running Partially Compensates for the Effects of Global Estrogen Receptor-α Knockout on Cortical Bone in Young Male Mice
Rebecca K. Dirkes, Nathan C. Winn, Thomas J. Jurrissen, Dennis B. Lubahn, Victoria J. Vieira-Potter, Jaume Padilla, Pamela S. Hinton
International Journal of Molecular Sciences.2021; 22(4): 1734. CrossRef
Role of Osteocytes in Cancer Progression in the Bone and the Associated Skeletal Disease
Manish Adhikari, Jesús Delgado-Calle
Current Osteoporosis Reports.2021; 19(3): 247. CrossRef
Gestational and lactational exposure to BPA or BPS has minimal effects on skeletal outcomes in adult female mice
Rebecca K. Dirkes, Rebecca J. Welly, Jiude Mao, Jessica Kinkade, Victoria J. Vieira-Potter, Cheryl S. Rosenfeld, Pamela S. Bruzina
Bone Reports.2021; 15: 101136. CrossRef
Modulation of bone turnover aberration: A target for management of primary osteoporosis in experimental rat model
Enas A. Fouad-Elhady, Hadeer A. Aglan, Rasha E. Hassan, Hanaa H. Ahmed, Gilane M. Sabry
Heliyon.2020; 6(2): e03341. CrossRef
Aromatase inhibitors attenuate the effect of alendronate in women with breast cancer
Sung Hye Kong, Jung Hee Kim, Sang Wan Kim, Chan Soo Shin
Journal of Bone and Mineral Metabolism.2020; 38(5): 730. CrossRef
Global estrogen receptor-α knockout has differential effects on cortical and cancellous bone in aged male mice
Rebecca K. Dirkes, Nathan C. Winn, Thomas J. Jurrissen, Dennis B. Lubahn, Victoria J. Vieira-Potter, Jaume Padilla, Pamela S. Hinton, Vance L. Trudeau
FACETS.2020; 5(1): 328. CrossRef
The Emerging Role of Osteocytes in Cancer in Bone
Emily G Atkinson, Jesús Delgado‐Calle
JBMR Plus.2019;[Epub] CrossRef
Effect of denosumab on low bone mineral density in postmenopausal Japanese women receiving adjuvant aromatase inhibitors for non-metastatic breast cancer: 24-month results
Katsuhiko Nakatsukasa, Hiroshi Koyama, Yoshimi Ouchi, Hisako Ono, Kouichi Sakaguchi, Takayuki Matsuda, Makoto Kato, Takashi Ishikawa, Kimito Yamada, Mana Yoshimura, Kei Koizumi, Teruhisa Sakurai, Hideo Shigematsu, Shunji Takahashi, Shinichiro Taira, Masat
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Association of Wnt Inhibitors, Bone Mineral Density and Lifestyle Parameters in Women with Breast Cancer Treated with Anastrozole Therapy
Kristina Bojanić, Ines Bilić Ćurčić, Lucija Kuna, Tomislav Kizivat, Robert Smolic, Nikola Raguž Lučić, Kristina Kralik, Vatroslav Šerić, Gordana Ivanac, Sandra Tucak-Zorić, Aleksandar Včev, Martina Smolić
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Peyman Hadji, Matti S. Aapro, Jean-Jacques Body, Michael Gnant, Maria Luisa Brandi, Jean Yves Reginster, M. Carola Zillikens, Claus-C. Glüer, Tobie de Villiers, Rod Baber, G. David Roodman, Cyrus Cooper, Bente Langdahl, Santiago Palacios, John Kanis, Nass
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Effects of raloxifene against letrozole-induced bone loss in chemically-induced model of menopause in mice
Abul Kalam, Sushama Talegaonkar, Divya Vohora
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Sclerostin: an Emerging Target for the Treatment of Cancer-Induced Bone Disease
Michelle M. McDonald, Jesus Delgado-Calle
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Differential profile of letrozole and exemestane on bone turnover markers in vinylcyclohexene diepoxide treated ovotoxic female mice
Abul Kalam, Sushama Talegaonkar, Divya Vohora
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Osteoblasts Are the Centerpiece of the Metastatic Bone Microenvironment
Hyo Min Jeong, Sun Wook Cho, Serk In Park
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Case Report

A Case of Latent Autoimmune Diabetes in Adults Developed after Surgical Cure of Growth Hormone Secreting Pituitary Tumor.: Wonjin Kim, Jung Ho Kim, Youngsook Kim, Ji Hye Huh, Su Jin Lee, Mi Sung Park, Eun Yeong Choe, Jeong Kyung Park, Myung Won Lee, Jae Won Hong, Byung Wan Lee, Eun Seok Kang, Bong Soo Cha, Eun Jig Lee, Hyun Chul Lee; Endocrinol Metab. 2012;27(4):318-322. Published online December 20, 2012; DOI: https://doi.org/10.3803/EnM.2012.27.4.318

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Abstract PDF: Acromegaly is generally caused by a benign growth hormone (GH)-secreting pituitary adenoma. It is characterized by a wide range of complications; cardiovascular, respiratory, bone and joint, and metabolic complications. Among them, impaired glucose tolerance and diabetes mellitus, due to GH-induced insulin resistance, has been reported in approximately 16-46% and 19-56%. They are usually improved following the treatment of acromegaly, surgical or medical therapy. We report a first case of 36-year-old man who was paradoxically diagnosed with GAD antibody positive latent autoimmune diabetes in adults (LADA) after the surgical cure of acromegaly.

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