Endocrinology and Metabolism

Original Articles

A Case of Thyroid Papillary Cancer Derived from Diffuse Goiter in a Patients with Acromegaly.: Dong Hee Kim, Jick Hwa Nam, Byoung Ho Sin, Ye Kyung Seo, Jung Guk Kim, Sung Woo Ha, Bo Wan Kim, Young Ha Lee, In Su Seo; J Korean Endocr Soc. 1996;11(3):311-317. Published online November 7, 2019

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Abstract PDF: Patients with acromegaly have a reduced life expectancy rnainly due to cardiovascular, respiratory or cerebrovascular diseas-. Malignancy also seems to occur with greater than the expected incidence. In particular, the published retrospective or prospective studies have suggested a strong association of colonic neoplasia with acromegaly. But, there were a few reports of thyroid cancer in acrornegaly. We report a case of thyroid papillary cancer derived from diffuse goiter in acromegaly, sugge- sting the possible carcinogenic role of growth hormone.

Adrenal gland
Association between the Growth Hormone Receptor Exon 3 Polymorphism and Metabolic Factors in Korean Patients with Acromegaly: Hye Yoon Park, In Ryang Hwang, Jung Bum Seo, Su Won Kim, Hyun Ae Seo, In Kyu Lee, Jung Guk Kim; Endocrinol Metab. 2015;30(3):312-317. Published online January 5, 2015; DOI: https://doi.org/10.3803/EnM.2015.30.3.312

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Background

This study investigated the association between the frequency of growth hormone receptor (GHR) exon 3 polymorphism (exon 3 deletion; d3-GHR) and metabolic factors in patients with acromegaly in Korea.

Methods

DNA was extracted from the peripheral blood of 30 unrelated patients with acromegaly. GHR genotypes were evaluated by polymerase chain reaction and correlated with demographic data and laboratory parameters.

Results

No patient had the d3/d3 genotype, while four (13.3%) had the d3/fl genotype, and 26 (86.7%) had the fl/fl genotype. Body mass index (BMI) in patients with the d3/fl genotype was significantly higher than in those with the fl/fl genotype (P=0.001). Age, gender, blood pressure, insulin-like growth factor-1, growth hormone, fasting plasma glucose, triglycerides, high density lipoprotein cholesterol, and low density lipoprotein cholesterol levels showed no significant differences between the two genotypes.

Conclusion

The d3-GHR polymorphism may be associated with high BMI but not with other demographic characteristics or laboratory parameters.

Citations

Citations to this article as recorded by

The Exon 3-Deleted Growth Hormone Receptor (d3GHR) Polymorphism—A Favorable Backdoor Mechanism for the GHR Function
Ghadeer Falah, Lital Sharvit, Gil Atzmon
International Journal of Molecular Sciences.2023; 24(18): 13908. CrossRef
Gender Specificity and Local Socioeconomic Influence on Association of GHR fl/d3 Polymorphism With Growth and Metabolism in Children and Adolescents
Xiaotian Chen, Chunlan Liu, Song Yang, Yaming Yang, Yanchun Chen, Xianghai Zhao, Weiguang Zhu, Qihui Zhao, Chuan Ni, Xiangyuan Huang, Weili Yan, Chong Shen, Harvest F. Gu
Frontiers in Pediatrics.2022;[Epub] CrossRef
Exon 3-deleted growth hormone receptor isoform is not related to worse bone mineral density or microarchitecture or to increased fracture risk in acromegaly
J. Pontes, M. Madeira, C. H. A. Lima, L. L. Ogino, F. de Paula Paranhos Neto, L. M. C. de Mendonça, M. L. F. Farias, L. Kasuki, M. R. Gadelha
Journal of Endocrinological Investigation.2020; 43(2): 163. CrossRef
MECHANISMS IN ENDOCRINOLOGY: Lessons from growth hormone receptor gene-disrupted mice: are there benefits of endocrine defects?
Reetobrata Basu, Yanrong Qian, John J Kopchick
European Journal of Endocrinology.2018; 178(5): R155. CrossRef
MECHANISMS IN ENDOCRINOLOGY: Clinical and pharmacogenetic aspects of the growth hormone receptor polymorphism
Cesar L Boguszewski, Edna J L Barbosa, Per-Arne Svensson, Gudmundur Johannsson, Camilla A M Glad
European Journal of Endocrinology.2017; 177(6): R309. CrossRef

Review Article

Thyroid
Molecular Pathogenesis and Targeted Therapies in Well-Differentiated Thyroid Carcinoma: Jung Guk Kim; Endocrinol Metab. 2014;29(3):211-216. Published online September 25, 2014; DOI: https://doi.org/10.3803/EnM.2014.29.3.211

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Four proto-oncogenes commonly associated with well-differentiated thyroid carcinoma, rearranged during transfection (RET)/papillary thyroid cancer, BRAF, RAS, and PAX8/peroxisome proliferator activated receptor-γ, may carry diagnostic and prognostic significance. These oncogenes can be used to improve the diagnosis and management of well-differentiated thyroid carcinoma. Limited therapeutic options are available for patients with metastatic well-differentiated thyroid cancer, necessitating the development of novel therapies. Vascular endothelial growth factor (VEGF)- and RET-directed therapies such as sorafenib, motesanib, and sunitinib have been shown to be the most effective at inducing clinical responses and stabilizing the disease process. Further clinical trials of these therapeutic agents may soon change the management of thyroid cancer.

Citations

Citations to this article as recorded by

Estimating the Growth Rate of Lung Metastases in Differentiated Thyroid Carcinoma: Response Evaluation Criteria in Solid Tumors or Doubling Time?
Eyun Song, Jonghwa Ahn, Min Ji Jeon, Sang Min Lee, Jeong Hyun Lee, Tae Yong Kim, Jung Hwan Baek, Won Bae Kim, Young Kee Shong, Won Gu Kim
Thyroid.2020; 30(3): 418. CrossRef
Lenvatinib for Radioactive Iodine-Refractory Differentiated Thyroid Carcinoma and Candidate Biomarkers Associated with Survival: A Multicenter Study in Korea
Eyun Song, Mijin Kim, Eui Young Kim, Bo Hyun Kim, Dong Yeob Shin, Ho-Cheol Kang, Byeong-Cheol Ahn, Won Bae Kim, Young Kee Shong, Min Ji Jeon, Dong Jun Lim
Thyroid.2020; 30(5): 732. CrossRef
The role of Slit2 as a tumor suppressor in thyroid cancer
Min Ji Jeon, Seonhee Lim, Mi-hyeon You, Yangsoon Park, Dong Eun Song, Soyoung Sim, Tae Yong Kim, Young Kee Shong, Won Bae Kim, Won Gu Kim
Molecular and Cellular Endocrinology.2019; 483: 87. CrossRef
Extended Real-World Observation of Patients Treated with Sorafenib for Radioactive Iodine-Refractory Differentiated Thyroid Carcinoma and Impact of Lenvatinib Salvage Treatment: A Korean Multicenter Study
Hye-Seon Oh, Dong Yeob Shin, Mijin Kim, So Young Park, Tae Hyuk Kim, Bo Hyun Kim, Eui Young Kim, Won Bae Kim, Jae Hoon Chung, Young Kee Shong, Dong Jun Lim, Won Gu Kim
Thyroid.2019; 29(12): 1804. CrossRef
Cytological features of warthin‐like papillary thyroid carcinoma: A case report with review of previous cytology cases
Archana George Vallonthaiel, Shipra Agarwal, Deepali Jain, Rajni Yadav, Nishikant A. Damle
Diagnostic Cytopathology.2017; 45(9): 837. CrossRef
Low Prevalence of Somatic TERT Promoter Mutations in Classic Papillary Thyroid Carcinoma
Min Ji Jeon, Won Gu Kim, Soyoung Sim, Seonhee Lim, Hyemi Kwon, Tae Yong Kim, Young Kee Shong, Won Bae Kim
Endocrinology and Metabolism.2016; 31(1): 100. CrossRef
Molecular Diagnosis Using Residual Liquid-Based Cytology Materials for Patients with Nondiagnostic or Indeterminate Thyroid Nodules
Hyemi Kwon, Won Gu Kim, Markus Eszlinger, Ralf Paschke, Dong Eun Song, Mijin Kim, Suyeon Park, Min Ji Jeon, Tae Yong Kim, Young Kee Shong, Won Bae Kim
Endocrinology and Metabolism.2016; 31(4): 586. CrossRef
Alpha lipoic acid inhibits proliferation and epithelial mesenchymal transition of thyroid cancer cells
Min Ji Jeon, Won Gu Kim, Seonhee Lim, Hyun-Jeung Choi, Soyoung Sim, Tae Yong Kim, Young Kee Shong, Won Bae Kim
Molecular and Cellular Endocrinology.2016; 419: 113. CrossRef
Upregulation of long noncoding RNA LOC100507661 promotes tumor aggressiveness in thyroid cancer
Daham Kim, Woo Kyung Lee, Seonhyang Jeong, Mi-Youn Seol, Hyunji Kim, Kyung-Sup Kim, Eun Jig Lee, Jandee Lee, Young Suk Jo
Molecular and Cellular Endocrinology.2016; 431: 36. CrossRef
Articles in 'Endocrinology and Metabolism' in 2014
Won-Young Lee
Endocrinology and Metabolism.2015; 30(1): 47. CrossRef

Original Article

Adrenal gland
Clinical Characteristics for 348 Patients with Adrenal Incidentaloma: Jongho Kim, Kwi Hyun Bae, Yeon Kyung Choi, Ji Yun Jeong, Keun Gyu Park, Jung Guk Kim, In Kyu Lee; Endocrinol Metab. 2013;28(1):20-25. Published online March 25, 2013; DOI: https://doi.org/10.3803/EnM.2013.28.1.20

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Background

Adrenal incidentaloma is an adrenal neoplasm frequently encountered in clinical practice for which detection rates have recently increased. We describe here the clinical characteristics of adrenal incidentalomas.

Methods

A retrospective study was performed examining the age, sex, location, size, function, and the histological findings for 348 patients with an adrenal mass discovered incidentally on computed tomography (CT) undertaken for health examination or nonadrenal disease from August 2005 to May 2012.

Results

Patients consisted of 156 males (44.8%) and 192 females (55.2%), aged between 20 and 86. Adrenal masses were most commonly found in patients in their sixth decade (32.5%). Regarding the location of the masses, 62.0% were found in the left adrenal gland, 30.2% were found in the right, and 7.8% were found bilaterally. Of all of the masses analyzed, 87.1% were 1 to 4 cm in size, and an adenoma-like appearance was the most common finding (75.3%) seen on CT scans. Hormonal analysis showed that 82.2% of the masses were nonfunctioning, 6.0% were diagnosed as subclinical Cushing's syndrome, 4.6% were aldosterone-producing adenomas, and 7.2% were pheochromocytomas. Adrenalectomy was performed in a total of 69 patients having adenoma (50.7%), pheochromocytoma (24.6%), and carcinoma (4.3%).

Conclusion

The characteristics of benign, malignant, nonfunctional, and functional adrenal masses that were incidentally found at our hospital were similar to those presented in other studies.

Citations

Citations to this article as recorded by

Impact of Size Thresholds on the Diagnosis of Incidental Adrenal Lesions: A Systematic Review and Meta-Analysis
Mitchell P. Wilson, Shubreet Randhawa, Bo Bao, Roger Croutze, Mohammad H. Murad, Matthew D.F. McInnes, Gavin Low
Journal of the American College of Radiology.2024; 21(1): 107. CrossRef
Quantitative analysis of catecholamines and their metabolites in 491 patients with adrenal tumors: a retrospective single-center cohort study
Hao-Long Zeng, Xu Wang, Hui-Jun Li, Qing Yang
Journal of Cancer Research and Clinical Oncology.2023; 149(8): 4979. CrossRef
Biochemical Assessment of Pheochromocytoma and Paraganglioma
Graeme Eisenhofer, Christina Pamporaki, Jacques W M Lenders
Endocrine Reviews.2023; 44(5): 862. CrossRef
Primary Aldosteronism Prevalence – An Unfolding Story
Suranut Charoensri, Adina F. Turcu
Experimental and Clinical Endocrinology & Diabetes.2023; 131(07/08): 394. CrossRef
Could the presence of adrenal incidentaloma negatively affect COVID 19 outcomes?
Metin Guclu, Bilal Burcak Aslan, Tirdad Setayeshi, Sinem Kiyici
Endocrine.2023; 82(2): 406. 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
CT-based Abdominal Adipose Tissue Area Changes in Patients Undergoing Adrenalectomy Due to Cushing’s Syndrome and Non-functioning Adenomas
Elif Gündoğdu, Emre Emekli
Experimental and Clinical Endocrinology & Diabetes.2022; 130(06): 368. CrossRef
The Etiological Profile of Adrenal Incidentalomas
Fatima-Zahra Lahmamssi, Loubna Saadaoui, Hayat Aynaou, Houda Salhi, Hanan El Ouahabi
Cureus.2022;[Epub] CrossRef
Clinical Characteristics and Follow-Up Results of Adrenal Incidentaloma
Nusret Yilmaz, Esin Avsar, Gokhan Tazegul, Ramazan Sari, Hasan Altunbas, Mustafa Kemal Balci
Experimental and Clinical Endocrinology & Diabetes.2021; 129(05): 349. CrossRef
A case of adrenocortical adenoma harboring venous thrombus mimicking adrenal malignancy
Agena Suzuki, Yuji Kamata, Tomomi Taguchi, Koji Takano, Yuto Yamazaki, Hironobu Sasano, Masayoshi Shichiri
Endocrine Journal.2021; 68(7): 857. CrossRef
Adrenal adenomas: what to do with them? Review 2
S. Rybakov
INTERNATIONAL JOURNAL OF ENDOCRINOLOGY (Ukraine).2021; 17(3): 241. CrossRef
A Brief Overview of the Epidemiology of Pheochromocytoma and Paraganglioma in Korea
Yun Mi Choi
Endocrinology and Metabolism.2020; 35(1): 95. CrossRef
Adrenal Incidentalomas are Tied to Increased Risk of Diabetes: Findings from a Prospective Study
Giuseppe Reimondo, Elena Castellano, Maurizio Grosso, Roberto Priotto, Soraya Puglisi, Anna Pia, Micaela Pellegrino, Giorgio Borretta, Massimo Terzolo
The Journal of Clinical Endocrinology & Metabolism.2020; 105(4): e973. CrossRef
Adrenal İnsidentaloma ve Otonom Kortizol Sekresyonu Vakalarının İzlemi: 14 Yıllık Tek Merkez Çalışması- Retrospektif Kohort
Nazlı Gülsoy KIRNAP, Sanem ÖZTEKİN, Neslihan Başçıl TÜTÜNCÜ
Dicle Tıp Dergisi.2020; 47(1): 154. CrossRef
Comparison between the clinical characteristics of patients with adrenal incidentalomas and those with hypertension-associated adrenal tumors in a single center in Japan
Mari Aoe, Akira Okada, Tomoko Usui, Katsunori Manaka, Masaomi Nangaku, Noriko Makita
Endocrine Journal.2020; 67(6): 645. CrossRef
Clinical outcomes of primary aldosteronism based on lateralization index and contralateral suppression index after adrenal venous sampling in real-world practice: a retrospective cohort study
Jeongmin Lee, Borami Kang, Jeonghoon Ha, Min-Hee Kim, Byungil Choi, Tae-Ho Hong, Moo IL Kang, Dong-Jun Lim
BMC Endocrine Disorders.2020;[Epub] CrossRef
Adrenal Incidentaloma
Mark Sherlock, Andrew Scarsbrook, Afroze Abbas, Sheila Fraser, Padiporn Limumpornpetch, Rosemary Dineen, Paul M Stewart
Endocrine Reviews.2020; 41(6): 775. CrossRef
Presentation and outcome of patients with an adrenal mass: A retrospective observational study
Nadeema Rafiq, Tauseef Nabi, SajadAhmad Dar, Shahnawaz Rasool
Clinical Cancer Investigation Journal.2020; 9(5): 198. CrossRef
Enfoque diagnóstico de feocromocitomas y paragangliomas
R.M. Gómez, M. Hernaiz, V. de Miguel, L.S. Aparicio, M.J. Marín, S. Lupi, M. Barontini
Hipertensión y Riesgo Vascular.2019; 36(1): 34. CrossRef
Accuracy of new recommendations for adrenal incidentalomas in the evaluation of excessive cortisol secretion and follow‐up
Ana Ruiz, Theodora Michalopoulou, Ana Megia, Silvia Näf, Inmaculada Simón‐Muela, Esther Solano, Laia Martínez, Joan Vendrell
European Journal of Clinical Investigation.2019;[Epub] CrossRef
Lateralizing Asymmetry of Adrenal Imaging and Adrenal Vein Sampling in Patients With Primary Aldosteronism
Norio Wada, Yui Shibayama, Takashi Yoneda, Takuyuki Katabami, Isao Kurihara, Mika Tsuiki, Takamasa Ichijo, Yoshihiro Ogawa, Junji Kawashima, Masakatsu Sone, Takanobu Yoshimoto, Yuichi Matsuda, Megumi Fujita, Hiroki Kobayashi, Kouichi Tamura, Kohei Kamemur
Journal of the Endocrine Society.2019; 3(7): 1393. CrossRef
Incidentally detected adrenal tumors – characteristics of patients and incidence of hormonal disorders
Iga Grochoła, Mateusz Winder, Krzysztof Kocot, Tomasz Menżyk, Jerzy Wojnar
Annales Academiae Medicae Silesiensis.2019; 73: 74. CrossRef
Characteristics of Adrenal Incidentalomas in a Large, Prospective Computed Tomography-Based Multicenter Study: The COAR Study in Korea
Seong Hee Ahn, Jae Hyeon Kim, Seung Hee Baek, Hyeonmok Kim, Yoon Young Cho, Sunghwan Suh, Beom-Jun Kim, Seongbin Hong, Jung-Min Koh, Seung Hun Lee, Kee-Ho Song
Yonsei Medical Journal.2018; 59(4): 501. CrossRef
Severe Cushing Syndrome Due to an ACTH-Producing Pheochromocytoma: A Case Presentation and Review of the Literature
Jenan N Gabi, Maali M Milhem, Yara E Tovar, Emhemmid S Karem, Alaa Y Gabi, Rodhan A Khthir
Journal of the Endocrine Society.2018; 2(7): 621. CrossRef
Clinical Investigation of Adrenal Incidentalomas in Japanese Patients of the Fukuoka Region with Updated Diagnostic Criteria for Sub-clinical Cushing's Syndrome
Ichiro Abe, Kaoru Sugimoto, Tetsumasa Miyajima, Tomoko Ide, Midori Minezaki, Kaori Takeshita, Saori Takahara, Midori Nakagawa, Yuki Fujimura, Tadachika Kudo, Shigero Miyajima, Hiroshi Taira, Kenji Ohe, Tatsu Ishii, Toshihiko Yanase, Kunihisa Kobayashi
Internal Medicine.2018; 57(17): 2467. CrossRef
Mortality in Patients with Endogenous Cushing’s Syndrome
Pedram Javanmard, Daisy Duan, Eliza B. Geer
Endocrinology and Metabolism Clinics of North America.2018; 47(2): 313. CrossRef
The Lateralizing Asymmetry of Adrenal Adenomas
Meng Hao, Diana Lopez, Miguel Angel Luque-Fernandez, Kathryn Cote, Jessica Newfield, Molly Connors, Anand Vaidya
Journal of the Endocrine Society.2018; 2(4): 374. CrossRef
Radiology of the adrenal incidentalomas. Review of the literature
F. A. Farrugia, G. Martikos, C. Surgeon, P. Tzanetis, E. Misiakos, N. Zavras, A. Charalampopoulos
Endocrine Regulations.2017; 51(1): 35. CrossRef
Baseline Demographic and Clinical Characteristics of Patients with Adrenal Incidentaloma from a Single Center in China: A Survey
Lele Li, Guoqing Yang, Ling Zhao, Jingtao Dou, Weijun Gu, Zhaohui Lv, Juming Lu, Yiming Mu
International Journal of Endocrinology.2017; 2017: 1. CrossRef
Guidelines for the Management of Adrenal Incidentaloma: the Korean Endocrine Society, Committee of Clinical Practice Guidelines
Jung-Min Lee, Mee Kyoung Kim, Seung-Hyun Ko, Jung-Min Koh, Bo-Yeon Kim, Sang-Wan Kim, Soo-Kyung Kim, Hae-Jin Kim, Ohk-Hyun Ryu, Juri Park, Jung-Soo Lim, Seong Yeon Kim, Young Kee Shong, Soon Jib Yoo
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Case Report: An incidentaloma that catches your eye - adrenal myelolipoma
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Clinical Guidelines for the Management of Adrenal Incidentaloma
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PIRMĄ KARTĄ NUSTATYTI ANTINKSČIŲ DARINIAI: 2010 IR 2014 METŲ IŠTYRIMO TAKTIKOS PALYGINIMAS
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Sveikatos mokslai.2017; 27(2): 60. CrossRef
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Reynaldo M. Gómez, Raul Chervín, Ester M. Pardes, Susana Lupi, María Elena Surraco, Jorge Herrera, León Schurman, Claudio Gonzáles
Revista Argentina de Endocrinología y Metabolismo.2016; 53(2): 51. CrossRef
Clinical and endocrinological characteristics of adrenal incidentaloma in Osaka region, Japan
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Clinicopathological Features Associated With the Prognosis of Patients With Adrenal Cortical Carcinoma
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Screening for Cushing’s syndrome: Is it worthwhile?
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Subclinical hypercortisolism in adrenal incidentaloma
Miguel Debono, John Newell-Price
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Incidental Adrenal Nodules and Masses: The Imaging Approach
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Adriana G. Ioachimescu, Erick M. Remer, Amir H. Hamrahian
Endocrinology and Metabolism Clinics of North America.2015; 44(2): 335. CrossRef
Brief Review of Articles in 'Endocrinology and Metabolism' in 2013
Won-Young Lee
Endocrinology and Metabolism.2014; 29(3): 251. CrossRef
Clinical Characteristics and Metabolic Features of Patients with Adrenal Incidentalomas with or without Subclinical Cushing's Syndrome
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"Arterial’naya Gipertenziya" ("Arterial Hypertension").2013; 19(6): 532. CrossRef

Case Report

A Case of Parathyroid Apoplexy of Primary Hyperparathyroidism Presenting as Auditory Hallucinations Accompanied with Hypocalcemia.: Eon Ju Jeon, Ji Yun Jeong, Jung Guk Kim; Endocrinol Metab. 2012;27(2):163-168. Published online June 20, 2012; DOI: https://doi.org/10.3803/EnM.2012.27.2.163

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Abstract PDF: The natural history of primary hyperparathyroidism, due to parathyroid adenoma, is unknown. Furthermore, spontaneous resolution of parathyroid necrosis or hemorrhage is rare and usually asymptomatic. Here, we report a case of parathyroid apoplexy of primary hyperparathyroidism, presenting as auditory hallucinations, accompanied with hypocalcemia. A 39-year-old man who was incidentally diagnosed with primary hyperparathyroidism, and waiting surgery for parathyroidectomy presented to psychiatric service with auditory hallucinations. He developed tetany, while taking psychiatric drugs. On a follow-up investigation, his serum calcium level fell from 11.8 to 5.8 mg/dL. His intact parathyroid hormone level also decreased from 1,017 pg/mL to 71.1 pg/mL. The parathyroid apoplexy was confirmed after a surgical removal of the infarcted adenoma. The auditory hallucinations disappeared, and serum calcium level was returned to within the normal range.

Clinical Trial

Recent Developments of Anti-diabetic Agents Undergoing 2 and 3 Phase Clinical Trials.: Jung Guk Kim; J Korean Endocr Soc. 2010;25(1):18-21. Published online March 1, 2010; DOI: https://doi.org/10.3803/jkes.2010.25.1.18

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Abstract PDF: No abstract available.

Editorial

Glucose Counterregulatory Hormone Response During Hypoglycemia due to Endogenous Hyperinsulinemia.: Jung Guk Kim; J Korean Endocr Soc. 2009;24(3):162-164. Published online September 1, 2009; DOI: https://doi.org/10.3803/jkes.2009.24.3.162

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Abstract PDF: No abstract available.

Case Reports

A Case of Pseudopseudohypoparathyroidism with Normal Stature.: Sae Rom Kim, Yun Jeong Doh, Hee Kyung Kim, Seong Su Moon, Ju Young Lee, Jae Han Jeon, Soo Won Kim, Bo Wan Kim, In Kyu Lee, Jung Guk Kim; J Korean Endocr Soc. 2009;24(2):138-143. Published online June 1, 2009; DOI: https://doi.org/10.3803/jkes.2009.24.2.138

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Abstract PDF

Pseudopseudohypoparathyroidism (PPHP) is characterized by the phenotype of Albright hereditary osteodystrophy (AHO) alone without biochemical evidence of multihormone resistance, which is unlike pseudohypoparathyroidism. AHO is associated with characteristic developmental abnormalities that include a short stocky stature, a short neck, brachydactyly, a round face, central obesity, mental retardation and subcutaneous ossifications. AHO is an autosomal dominant disease that's caused by heterozygous inactivating mutations in the Gsalpha gene (GNAS1). Melanocortin-4 receptor (MC4R) is a hypothalamic Gs-coupled receptor that is thought to mediate the central effect of leptin on satiety. MC4R mutations cause morbid obesity starting in infancy, as well as an elevated leptin level. A 62 year old man with a height of 171.5 cm, a round face, a short neck, central obesity and brachydactyly had normal ranges of serum calcium, phosphorus and PTH and a normal Ellsworth-Howard test. GNAS1 gene analysis revealed substitution of alanine to cysteine in the 165 codon of exon 6 and substitution of alanine to cysteine in the 231 codon of exon 9. Two known SNPs (Cyt-1042Thy, Gua-719Ade) in the MC4R were detected in the patient. We report here on a case of PPHP and the patient had normal stature. We propose that MC4R may have contributed to the obesity & normal stature of this patient.

Citations

Citations to this article as recorded by

Polyglandular Autoimmune Syndrome Type III with Primary Hypoparathyroidism
Sang Jin Kim, Sang-Yoon Kim, Han-Byul Kim, Hyukwon Chang, Ho-Chan Cho
Endocrinology and Metabolism.2013; 28(3): 236. CrossRef

Urinary Bladder Pheochromocytoma with a Long Asymptomatic Period.: Soon Hong Park, Jung Eun Lee, Sung Soo Moon, Joo Young Lee, Eui Dal Jung, Jung Guk Kim, In Kyu Lee, Bo Wan Kim; J Korean Endocr Soc. 2006;21(5):402-407. Published online October 1, 2006; DOI: https://doi.org/10.3803/jkes.2006.21.5.402

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Abstract PDF: Pheochromocytoma arises from chromaffin cells of the paraganglionic system. Urinary bladder pheochromocytoma is very rare tumor that accounts for less than 0.06% of all urinary bladder tumors and it is less than 1% of all pheochromocytoma. Urinary bladder pheochromocytoma usually arises from the sympathetic nervous system of the bladder wall. Paroxysmal headache and palpitation precipitated by micturition are common specific symptoms of this tumor. The paroxysm commonly persists or the patients relapse in a few days or months. Herein, we report a case of urinary bladder pheochromocytoma with a long asymptomatic period.

A Case of Acute Adrenal Insufficiency with Bilateral Adrenal Hemorrhagic Infarction due to Ebstein-Barr Virus Infection in a Patient with Asymptomatic Chronic Adrenal Insufficiency.: Sung Chang Chung, Soon Hong Park, Joo Young Lee, Shin Won Lee, Gui Hwa Jeong, Sun Zoo Kim, Han Ik Bae, Hun Kyu Ryeom, Jung Guk Kim, Young Oh Kweon, Sung Woo Ha, Bo Wan Kim; J Korean Endocr Soc. 2004;19(2):217-222. Published online April 1, 2004

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Abstract PDF: Acute adrenal insufficiency may result from adrenal crisis, hemorrhagic destruction, or the rapid withdrawal of steroids from patient with chronic steroid medication, congenital adrenal hyperplasia or those on other drugs. Acute hemorrhagic destruction of both adrenal glands can occur due to infection, trauma, anticoagulant therapy, antiphospholipid syndrome or a coagulation disorder. However, there have been no reports on acute hemorrhagic adrenal insufficiency due to the Ebstein-Barr virus (EBV). Herein, a case of acute adrenal insufficiency, with bilateral adrenal hemorrhagic infarction, is reported in a patient with asymptomatic chronic adrenal insufficiency. A 42-year-old man presented with general weakness, weight loss and hyperpigmentation of several months duration. He suffered from a sore throat, general myalgia and a headache on admission. The laboratory findings were lymphocytosis, positive EBV IgM antibody, low cortisol level and a high level of adrenocorticotropic hormone (ACTH). Adrenocortical autoantibody and PCR for Mycobacterium tuberculosis showed negative findings. The serologic findings for CMV and HIV were negative. Fine needle aspiration of the adrenal gland revealed a hemorrhagic infarction and positive staining for the anti-EBV antibody. Acute adrenal insufficiency was then diagnosed with a bilateral adrenal hemorrhagic infarction due to the EBV infection in the patient, also with asymptomatic chronic adrenal insufficiency. This is the first case of acute adrenal insufficiency with bilateral hemorrhagic infarction, due to an EBV infection

Original Articles

Influence of Anti-thyroglobulin Antibody on the Measurement of Thyroglobulin using the Immunoradiometric Assay.: Byeong Cheol Ahn, Jin Ho Bae, Shin Young Jeong, Ho Yong Park, Jung Guk Kim, Sung Woo Ha, Jaetae Lee, Bo Wan Kim, Kyu Bo Lee; J Korean Endocr Soc. 2004;19(1):42-47. Published online February 1, 2004

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Abstract PDF: BACKGROUND
Serum thyroglobulin(Tg) is a valuable and sensitive tool needed in the follow-up of patients with differentiated thyroid cancer(DTC), but antithyroglobulin antibody(Anti-Tg), common in patients with DTC, can interfere with the assay for Tg. In this study, we evaluated the influence of Anti-Tg on the measurement of Tg using the immunoradiometric assay(IRMA). METHODS: In using ELSA-hTg in vivo test(CIS international, Schering, France), a solid phase two-site IRMA was used to measure Tg(23.5ng/mL, 62.5ng/mL) under the absence or presence of three concentrations of Anti-Tg(25U/mL, 50U/mL, 100U/mL). We also performed Tg measurement using patients serum that was mixed with patients serum containing high Anti-Tg. ANOVA and Scheffe tests were performed to evaluate the effect of Anti-Tg on Tg IRMA, and an inverse regression was made to calculate the level of Tg from measured Tg and used Anti-Tg levels and also to assess the degree of effect of anti-Tg on Tg IRMA. RESULTS: In measuring Tg using the standard solution, the presence of Anti-Tg resulted in a falsely suppressed Tg value. The IRMAs for 23.5ng/mL of the standard Tg solution resulted in 24.5+/-.1 ng/mL under no Anti-Tg, 11.8+/-.4ng/mL under 25U/mL of Anti-Tg, 7.7+/-.1ng/mL under 50U/mL of Anti-Tg, and 4.5+/-.4ng/mL under 100U/mL of Anti-Tg. IRMAs 62.5ng/mL of the standard Tg solution resulted in 65.9+/-.7ng/mL under no Anti-Tg, 36.3+/-.2ng/mL under 25U/mL of Anti-Tg, 23.7+/-.7ng/mL under 50U/mL of Anti-Tg, and 14.0+/-.0ng/mL under 100U/mL of Anti-Tg. (ANOVA test, p=0.000). The degree of suppression of the measured Tg value was positively correlated with the Anti-Tg level (Quadratic model regression, Sig T=0.000). The presence of Anti-Tg also resulted in a falsely suppressed Tg value for the Tg measurement using patient's serum. CONCLUSION: The presence of Anti-Tg could consist of the use of Tg as a tumor, therefore Anti-Tg should be measured in all patients diagnosed with DTC. The interpretation of the Tg level must be performed with extreme caution in patients with Anti-Tg.

Association of Polymorphism in beta3-Adrenergic Receptor Gene with Fat Distribution.: Tae Sung Yun, Yong Deuk Kim, Hye Soon Kim, Mi Jung Kim, Young Sung Suh, Jung Hyeok Kwon, Jin Soo Choi, Jung Guk Kim, Sung Woo Ha, Bo Wan Kim, Kyu Chang Won, Hyong Woo Lee, Ho Sang Shon, Ji Hyun Lee, Hyun Dae Yoon, Won Ho Kim, Young Gil Yun, In Kyu Lee; J Korean Endocr Soc. 2003;18(2):184-192. Published online April 1, 2003

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Abstract PDF: BACKGROUND
Reasons for obesity include environmental factors and, more largely so, genetic factors. There have been many studies on these genetic factors. So far, genes related to obesity such as Leptin, Uncoupling Protein(UCP), Peroxisome proliferator activated receptor-gamma(PPAR-gamma), and Beta3-adrener-gic receptor(beta3-AR) gene have been discovered. Among these, beta3-AR is expressed in visceral adipose tissue and is thought to contribute to the regulation of resting metabolic rate and lipolysis. The missense mutation of beta3-AR gene, resulting in replacement of tryptophan by arginine at position 64(Trp64Arg), is associated with decreased resting metabolic rate and weightgain. We performed this study to determine if Trp64Arg polymorphism of beta3-AR gene is associatedwith obesity in Koreans. METHOD: We investigated the relationship between the beta3-AR gene mutation and body mass index (BMI), waist circumference, hip circumference, waist to hip ratio(WHR), area of subcutaneous fat, area of visceral fat, visceral to subcutaneous fat ratio(VSR), and lipid profile. 198 subjects were included in this study of which 97 were of normal weight and 101 were obese. Anthropometric data was obtained from physical examination and medical records. RESULT: In the cases of beta3-AR gene mutation of the obese group, the ratio of Trp/Arg and Arg/Arg are 43% and 5%, respectively, which were higher than the normal group(36%, 1%), although a statistical significant was not found. There was significant difference in the are of subcutaneous fat. Normal group(Trp/Trp) measured at 213.9+/-109.6cm2 versus 244.0+/-127.7cm2 (Trp/Arg) and 323.9+/-189.9cm2(Arg/Arg) for the mutation groups. Circumference of waist, circumference of hip, WHR, area of visceral fat, and VSR were higher in the mutation groups than in normal subject, but not significantly different. CONCLUSION: These results suggest that a genetic mutation in the beta3-AR gene can affect body fat composition, and is associated with obesity in Korean adults.

Case Reports

A Case of Calciphylaxis Mimicking Dermatomyositis.: Jeung Hun Han, Sin Won Lee, Gui Hwa Jung, Chang Hoon Choi, Soon Hee Lee, Jung Guk Kim, Sung Woo Ha, Jong Myung Lee, Nung Soo Kim, Bo Wan Kim; J Korean Endocr Soc. 2002;17(2):297-301. Published online April 1, 2002

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Abstract PDF: Calciphylaxis is a rare, but fatal, condition that is characterized by a rapidly progressive ischemic necrosis of the skin, underlying tissue and other organs, as well as rapid vascular calcification. It results in death due to sepsis, heart or respiratory failure. A 67-year old female was admitted to hospital with the chief complaint of constant pain to both lower legs of 1 week duration. She was treated with calcitonin-salmon due to a prior unexplained hypercalcemia of 2 weeks. On the third day post admission. pain and weakness in the lower legs were aggravated, became painful, with violaceous skin lesions developing on the thigh with findings similar to those of rhabdomyolysis. Because she was suspected of having dermatomyositis, she was treated with methylpredrisolone. However, the skin lesions and symptoms were aggravated, and she died of sepsis due to a skin infection. About 160 cases of calciphylaxis have been reported, with most of these cases being associated with secondary hyperparathyroidism due to end-stage renal disease, but cases of calciphylaxis without renal failure are very rare. We now report a case of calciphylaxis without renal failure, mimicking dermatomyositis, and present a brief review of the pathophysiology and treatments of calciphylaxis inform the relevant literature.

A Case of Functioning Paraganglioma in Posterior Mediastinum.: Chang Hoon Choi, Sin Won Lee, Gui Hwa Jung, Si Hyung Park, Soon Hee Lee, Jung Guk Kim, Sung Woo Ha, Bo Wan Kim, Sang Chul Lee, Eung Bae Lee, Tae In Park; J Korean Endocr Soc. 2002;17(2):292-296. Published online April 1, 2002

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Abstract PDF: Paraganglioma is an extraadrenal pheochromocytoma originating from chromaffin cells distributed in the sympathetic nervous systems. Functioning extraadrenal paragangliomas represent more than 10% of all pheochromocytomas, and seems to be highly malignant tumor in comparison to intraadrenal pheochromocytomas. Recently, we experienced a case of a paraganglioma in the posterior mediastinum. A 32-year-old woman was admitted to hospital due to dyspnea on exertion, and intractable hypertension. A chest X-ray showed a well-defined mass density on the right cardiac border, and biochemical studies showed characteristic findings of pheochromocytoma. A solitary pheochromocytoma was located in the posterior mediastinum using 131I-MIBG scintigraphy. The clinical manifestations, including hypertension and dyspnea were improved after operation.

A Case of McCunt-Albright Syndrome Associated with Acremegaly and Fibrous Dysplasia.: Jung Guk Kim, Sung Woo Ha, Sang Won Chung, Seong Mo Koo, Jae Tae Lee, Kyu Bo Lee, Yong Sun Kim, Sam Kwon, Bo Wan Kim; J Korean Endocr Soc. 1998;13(1):108-114. Published online January 1, 2001

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Abstract PDF: Acromegaly and hyperprolactinemia have been described in association with polyostotic fibrous dysplasia. The pathogenic mechanisms of this endocrinopathy are not clear. We experienced a 19-year-old male with hypersecretion of GH, hyperprolactinemia and fibrous dysplasia. He was referred for evaluation of suspected acromegaly. He had no skin pigmentation. Plasma GH, PRL, somatomedin-C, LH, FSH, testosterone, estradiol, progesterone, T3, T4, TSH and cortisol were measured. Among those, the levels of plasma GH, PRL and somatomedin-C were high. Serum alkaline phosphatase was increased. OGTT did not suppress plasma OH concentration and GH showed paradoxical response to TRH and LHRH. GH was suppressed after a test-dose of somatastatin and bromocriptine. Brain MRI demonstrated a mass lesion in sella turcica and another mass lesions in nasal cavity and posterior occipital bone. Whole body bone scan revealed increased uptake in skull, nasal bone, both 9th posterior rib, both femurs, both tibias, left scapular and pelvic bone. These fmdings were consistent with bone tumor such as fibrous dysplasia. We report a case with incomplete MeCune-Albright syndrome including acromegaly, hyperprolactinemia and polyostotic fibrous dysplasia.

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