Endocrinology and Metabolism

Original Articles

Graves' disease associated with Hashimoto's thyroiditis.: Seong Bin Hong, Myoung Sik Kim, Kwan Woo Lee, Mi Rin Kim, Hyoun Ho In, Kyoung Rae Kim, Young Up Cho, Young Chae Joo; J Korean Endocr Soc. 1996;11(2):182-188. Published online November 7, 2019

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Abstract PDF: Hashimotos thyroiditis and Graves disease have been thought to be the autoirnmune diseases having their distinct and separate clinical and pathologic features. Because of the partial and complete simi1arity in histologic feature, it has been hypothesized that Hashimoto's thyroiditis and Oraves disease may be interrelated. Several patients who had been diagnosed as Graves disease showed features of hypothyroidism, and were proven to have histologie features of Hashimotos thyroiditis in their thyroid tissue. Two meehanisms had been suggested to explain tbis phenomenon. One is that the combined Hashimotos thyroiditis in Graves' disease may become predominant with time, The other is that the amount of TSH receptor blocking antibody may increase in the course of Graves disease. Early recognition of these patients would be impartant to estabilish therapeutic plan. Futhermcee, extensive study of these patients would give more understanding of the mechanism of these diseases. Here we report 5 cases of clinically diagnosed Graves disease with pathologic features of Hashimotos thyroiditis or focal lymphocytic thyroiditis.

Clinical Study
Obesity and Hyperglycemia in Korean Men with Klinefelter Syndrome: The Korean Endocrine Society Registry: Seung Jin Han, Kyung-Soo Kim, Wonjin Kim, Jung Hee Kim, Yong-ho Lee, Ji Sun Nam, Ji A Seo, Bu Kyung Kim, Jihyun Lee, Jin Ook Chung, Min-Hee Kim, Tae-Seo Sohn, Han Seok Choi, Seong Bin Hong, Yoon-Sok Chung; Endocrinol Metab. 2016;31(4):598-603. Published online December 20, 2016; DOI: https://doi.org/10.3803/EnM.2016.31.4.598

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Abstract PDF PubReader

Background

The aim of this study was to investigate the prevalence of obesity in Korean men with Klinefelter syndrome (KS) and the associated risk factors for obesity and hyperglycemia.

Methods

Data were collected retrospectively from medical records from 11 university hospitals in Korea between 1994 and 2014. Subjects aged ≥18 years with newly diagnosed KS were enrolled. The following parameters were recorded at baseline before treatment: chief complaint, height, weight, fasting glucose level, lipid panel, blood pressure, testosterone, luteinizing hormone, follicle-stimulating hormone, karyotyping patterns, and history of hypertension, diabetes, and dyslipidemia.

Results

Data were analyzed from 376 of 544 initially enrolled patients. The rate of the 47 XXY chromosomal pattern was 94.1%. The prevalence of obesity (body mass index ≥25 kg/m²) in Korean men with KS was 42.6%. The testosterone level was an independent risk factor for obesity and hyperglycemia.

Conclusion

Obesity is common in Korean men with KS. Hypogonadism in patients with KS was associated with obesity and hyperglycemia.

Citations

Citations to this article as recorded by

A dual-center study of predictive factors for sperm retrieval through microdissection testicular sperm extraction and intracytoplasmic sperm injection outcomes in men with non-mosaic Klinefelter syndrome
Jong Hyeun Baeck, Tae Jin Kim, Tae Heon Kim, Seung-Ryeol Lee, Dong Soo Park, Hwang Kwon, Ji Eun Shin, Dong Hyeon Lee, Young Dong Yu
Investigative and Clinical Urology.2023; 64(1): 41. CrossRef
Cardiorespiratory fitness in adolescents and young adults with Klinefelter syndrome – a pilot study
Julia Spiekermann, Kathrin Sinningen, Beatrice Hanusch, Michaela Kleber, Michael M. Schündeln, Cordula Kiewert, Heide Siggelkow, Jakob Höppner, Corinna Grasemann
Frontiers in Endocrinology.2023;[Epub] CrossRef
Metabolic Profile in a Cohort of Young Sicilian Patients with Klinefelter’s Syndrome: The Role of Irisin
Stefano Radellini, Valentina Guarnotta, Vincenzo Sciabica, Giuseppe Pizzolanti, Carla Giordano, Vito Angelo Giagulli
International Journal of Endocrinology.2022; 2022: 1. CrossRef
Metabolic and Nutritional Aspects in Paediatric Patients with Klinefelter Syndrome: A Narrative Review
Chiara Mameli, Giulia Fiore, Arianna Sangiorgio, Marta Agostinelli, Giulia Zichichi, Gianvincenzo Zuccotti, Elvira Verduci
Nutrients.2022; 14(10): 2107. CrossRef
Klinefelter syndrome in an adolescent with severe obesity, insulin resistance, and hyperlipidemia, successfully treated with testosterone replacement therapy
Shota Fukuhara, Jun Mori, Hisakazu Nakajima
Clinical Pediatric Endocrinology.2021; 30(3): 127. CrossRef
Glucose metabolic disorder in Klinefelter syndrome: a retrospective analysis in a single Chinese hospital and literature review
Shixuan Liu, Tao Yuan, Shuoning Song, Shi Chen, Linjie Wang, Yong Fu, Yingyue Dong, Yan Tang, Weigang Zhao
BMC Endocrine Disorders.2021;[Epub] CrossRef
What Every Internist-Endocrinologist Should Know about Rare Genetic Syndromes in Order to Prevent Needless Diagnostics, Missed Diagnoses and Medical Complications: Five Years of ‘Internal Medicine for Rare Genetic Syndromes’
Anna G. W. Rosenberg, Minke R. A. Pater, Karlijn Pellikaan, Kirsten Davidse, Anja A. Kattentidt-Mouravieva, Rogier Kersseboom, Anja G. Bos-Roubos, Agnies van Eeghen, José M. C. Veen, Jiske J. van der Meulen, Nina van Aalst-van Wieringen, Franciska M. E. H
Journal of Clinical Medicine.2021; 10(22): 5457. CrossRef
Klinefelter Syndrome and Diabetes
Mark J. O’Connor, Emma A. Snyder, Frances J. Hayes
Current Diabetes Reports.2019;[Epub] CrossRef
Endocrine aspects of Klinefelter syndrome
Adriana Herrera Lizarazo, Michelle McLoughlin, Maria G. Vogiatzi
Current Opinion in Endocrinology, Diabetes & Obesity.2019; 26(1): 60. CrossRef
Sex differences in metabolism and cardiometabolic disorders
Karthickeyan Chella Krishnan, Margarete Mehrabian, Aldons J. Lusis
Current Opinion in Lipidology.2018; 29(5): 404. CrossRef
Klinefelter Syndrome: Integrating Genetics, Neuropsychology, and Endocrinology
Claus H Gravholt, Simon Chang, Mikkel Wallentin, Jens Fedder, Philip Moore, Anne Skakkebæk
Endocrine Reviews.2018; 39(4): 389. CrossRef
Sex differences in obesity, lipid metabolism, and inflammation—A role for the sex chromosomes?
Temeka Zore, Maria Palafox, Karen Reue
Molecular Metabolism.2018; 15: 35. CrossRef
Klinefelter syndrome, insulin resistance, metabolic syndrome, and diabetes: review of literature and clinical perspectives
Andrea Salzano, Roberta D’Assante, Liam M. Heaney, Federica Monaco, Giuseppe Rengo, Pietro Valente, Daniela Pasquali, Eduardo Bossone, Daniele Gianfrilli, Andrea Lenzi, Antonio Cittadini, Alberto M. Marra, Raffaele Napoli
Endocrine.2018; 61(2): 194. CrossRef
Síndrome de Klinefelter y riesgo cardiovascular
A. Yamaguchi, P. Knoblovits
Hipertensión y Riesgo Vascular.2018; 35(4): 195. CrossRef
Articles inEndocrinology and Metabolismin 2016
Won-Young Lee
Endocrinology and Metabolism.2017; 32(1): 62. CrossRef
Sex differences in obesity: X chromosome dosage as a risk factor for increased food intake, adiposity and co-morbidities
Karen Reue
Physiology & Behavior.2017; 176: 174. CrossRef
Klinefelter Syndrome with Morbid Obesity Before Bariatric Surgery: A Case Report
Parisa Janmohammadi, Gholamreza Mohammadi-Farsani, Hana Arghavani, Mahmoud Arshad, Tayebeh Mokhber
Journal of Minimally Invasive Surgical Sciences.2017;[Epub] CrossRef
Klinefelter Syndrome and Metabolic Disorder
Ji Cheol Bae
Endocrinology and Metabolism.2016; 31(4): 535. CrossRef

Case Reports

A Case of Spontaneous Rupture of Adrenal Pheochromocytoma.: Eun Joo Kim, Mie Jin Lim, Byoung Wook Bang, Hyun Joo Park, Seong Bin Hong, Moon Suk Nam, Yong Seong Kim, Suk Jin Choi; J Korean Endocr Soc. 2006;21(5):424-427. Published online October 1, 2006; DOI: https://doi.org/10.3803/jkes.2006.21.5.424

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Abstract PDF

Although the spontaneous rupture of a pheochromocytoma is rare, it can be fatal if not promptly diagnosed. Hemorrhagic necrosis of the tumor will require an emergency operation, as this causes intraperitoneal hemorrhaging. Excessive vasoconstriction, tachycardia and labile blood pressure are the signs of a ruptured pheochromocytoma. The standard diagnostic tool of such a condition is an abdominal computed tomographic scan. Although the exact mechanism of the rupture is still unknown, a hemorrhage inside the tumor or a high intra-cortical pressure, due to rapid growth of the tumor, is known to play a major role in the pathogenesis. Herein is reported the case of a 53-year old male patient with a pheochromocytoma and an acute abdomen. The CT scan showed a left adrenal gland mass, with a hemorrhage in the left perirenal space. The patient underwent an emergency adrenalectomy, with the final histopathological diagnosis being that of a pheochromocytoma. Prompt recognition and early surgical intervention can improve outcome.

Citations

Citations to this article as recorded by

Ruptured functioning adrenal tumour, atypical presentation with renal colic and hypertension
Amr Elmoheen, Mohamed Yousry, Ahmed Elmesery, Khalid Bashir
BMJ Case Reports.2020; 13(12): e236050. CrossRef
Spontaneous ruptured pheochromocytoma: an unusual case report and literature review
Ye Seob Jee
Annals of Surgical Treatment and Research.2017; 93(3): 170. CrossRef

A Case of Bilateral Macronodular Adrenocortical Hyperplasia Accompanied by Hyperresponsiveness to Vasopressin.: Deok In Kim, Seung Hee Lee, Eun A Kim, Hwi La Park, Gyeong Ug Lee, Seong Bin Hong, Moon Suk Nam, Seok Hwan Shin, Yong Seong Kim; J Korean Endocr Soc. 2005;20(4):390-394. Published online August 1, 2005; DOI: https://doi.org/10.3803/jkes.2005.20.4.390

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Abstract PDF: Cushing's syndrome associated with nodular adrenal hyperplasia glands is divided into 4 main categories: adrenal adenoma, adrenal carcinoma, primary pigmented nodular adrenal dysplasia and macronodular adrenal hyperplasia(MAH). The mechanism of bilateral MAH, when ACTH is suppressed, was previously unknown, and referred to as being "autonomous". Recently, several reports have shown MAH to be under the control of ectopic or eutopic membrane hormone. Here, a case of Cushing's syndrome, caused by bilateral MAH, is reported. A 62-year-old woman presented with Cushingoid features, hypertension and diabetes mellitus. In her case, abnormal adrenal stimulation of cortisol secretion in response to exogenous vasopression stimulation was shown. Her urine free cortisol was 726.0microgram/dL, which was not suppressed after administration of high-dose dexamethasone. Her plasma cortisol level was elevated, but without circadian rhythm. ACTH was undetectable. An abdomen CT scan demonstrated bilaterally enlarged multinodular adrenal glands. A Sella MRI revealed no alteration of the pituitary gland. The patient underwent a laparoscopic bilateral adrenalectomy. Histological examination revealed bilateral macronodular hyperplasia. After having recovered, the patient showed progressive regression of the Cushingoid status.

A Case of Isolated Familial Somatotropinoma.: Hwi Ra Park, Eun A Kim, Mei Hua Jiang, Chang Soo Jang, Kyoung Wook Lee, Seong Bin Hong, Eun Young Kim, Myung Kwan Lim, Moon Suk Nam, Yong Seong Kim; J Korean Endocr Soc. 2004;19(4):398-405. Published online August 1, 2004

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Abstract PDF: The familial occurrence of a pituitary adenoma associated with multiple endocrine neoplasia (MEN) type 1 or Carney complex is a well-recognized entity. However, an isolated familial somatotropinoma is a rare inherited disease, which is characterized by clustering of a somatotrophic adenoma and acromegaly or gigantism in a family, but without other manifestations of MEN type 1, with only 68 cases, in 28 families, described in the literature. The mode of inheritance is autosomal dominant, with incomplete penetration, but the genetic background of these pituitary adenomas remains unknown. A family exists where both the father and son were affected. Endocrinological investigations confirmed hypersecretion of GH and IGF-1, and the pituitary adenomas were identified by magnetic resonance image in both cases. There was no symptom of MEN type 1 or other form of endocrine dysfunction. Herein is reported a case of an isolated familial somatotropinoma in Korea, with a review of the literature

Rhabdomyolysis Associated with Hyponatremia.: Kyoung Wook Lee, Seong Bin Hong, Seung Baik Han, Bon Ju Jeong, Hwi Ra Park, Eun Sil Kim, Dong Hyo Hyun, Moon Suk Nam, Yong Seong Kim; J Korean Endocr Soc. 2003;18(3):306-310. Published online June 1, 2003

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Abstract PDF: Hyponatremia is a frequent condition of body fluid and electrolyte imbalance encountered in clinical practice. However, rhabdomyolysis has rarely been reported in association with hyponatremia. We experienced a 56-year-old woman who had developed symptomatic hyponatremia after prolonged nausea and subsequent rhabdomyolysis. Hyponatremia was probably mediated by hypersecretion of antidiuretic hormones.The woman had developed severe hypotonic hyponatremia with an alternation in mental status after suffering from severe nausea and vomiting for x months/years. She recovered with intensive supportive therapy, including hypertonic saline administration. One day after hospitalization, she complained of thigh pains; furthermore her serum creatine phosphokinase level had increased. She was treated with alkaline diuresis. Renal failure or compartment syndrome did not complicate the clinical course. The patient was discharged and returned to her normal daily activities. The possibility of rhabdomyolysis should be considered in patients with acute hyponatremia who have developed muscle pain.

Histomorphometry of Osteogenesis Imperfecta I.: Seong Bin Hong, Suk Myun Ko, Yong Koo Park, Young Joo Park, Yoon Juo Oh, Young Wan Kim, Sung Ki Kim, Moon Suk Nam, Yong Seong Kim; J Korean Endocr Soc. 2002;17(1):117-123. Published online February 1, 2002

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Abstract PDF: Osteoporosis imperfecta (OI) is a genetic disorder characterized by fragility of bone, deafness, blue sclerae; and laxity of joints. Four types of OI are distinguished by clinical findings. Although mutations affecting collagen I are responsible for the disease in the most patients, the mechanism by which the genetic defects cause abnormal bone development has not been well established. Therefore we evaluated static and dynamic bone histomorphometry of type I OI in the case study of a 15 year old boy with OI who had blue sclerae, a history of frequent fracture and a familial history of blue sclerae. Biopsy of the ilium showed loss of connection between the cortical bone and trabecular bones. The Harversian system in the cortical bone was poorly developed. In the trabecular bones, the lamellar pattern was poorly developed. Mineral apposition rate of the cortical bone was 1.0 m/day and of the trabecular bone was 0.79 m/day. Thus OI might be regard as a disease whereby abnormal collagen synthesis interferes with bone strength by multiple mechanisms.

A Case of Pancytopenia Caused by Sheehan's Syndorme Improved with Hormone Replacement Therapy.: Sung Ki Kim, Yoon Ju Oh, Park Young Joo, Young Whan Kim, Seong Bin Hong, Mi Rim Kim, Moon Suk Nam, Yong Seong Kim; J Korean Endocr Soc. 2000;15(4-5):595-599. Published online January 1, 2001

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Abstract PDF: Postpartum ischemic necrosis of the pituitary gland, known as Sheehan's syndrome, is well- established clinical entity. In anterior pituitary insufficiency, there is very often a normochromic or hypochromic anemia but pancytopenia secondary to the hypopituitarism is less common. We report a case of pancytopenia due to complete aplasia of the bone marrow associated with Sheehan's syndrome, in which hormone replacement therapy alone produced full hematological recovery.

A Case of Delayed Puberty due to Hypoplasia of Anterior Pituitary Gland with Pituitary Stalk Agenesis and Ectopic Neurohypophysis.: Seung Yong Shin, Jin Young Kim, Seung Jae Yoon, Sung Ki Kim, Seong Bin Hong, Yeo Joo Kim, Moon Suk Nam, Mi Rim Kim, Yong Seong Kim; J Korean Endocr Soc. 1999;14(3):578-586. Published online January 1, 2001

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Abstract PDF: Hypopituitarism is not a common cause of delayed puberty, however it should always be considered, especially if there are such signs as severe dwarfism, dollish face, truncal obesity, small hands and feet, and microgenitalia. Either congenital or acquired, hypopituitarism can be resulted from hypothalamic and hypophyseal lesions. The clinical feature can be diverse depending on age of the patients, rate of progression, degree of hormone deficiency and characteristics of the lesion. The recent high interest in delayed puberty and the improved detection of hypothalamic hypophyseal lesions using combined pituitary fuction stimulation test, brain CT and MRJ, has made the differential diagnosis of hypopituitarism possible as the cause of delayed puberty. MRI has shown hypophyeal hypoplasia accompanied by anterior pituitary hypoplasia and ectopic neurohypophysis in some of the patients with hypopituitasm, and although the anatomical abnormality around the hypophysis in these patients is considered the reason for hypopituitarism, the pathogensis of which has not yet to be known. We, here, report a case of delayed puberty by hypopituitarism due to hypoplasia of anterior pituitary gland, pituitary stalk agenesis and ectopic neurohypophysis with brief review of the litereature.

A Case of Normal Full Term Delivery after Afrenalectomy for Cushing's Syndrome in a Pregnant Patient.: Sung Sik Yang, Yong Seong Kim, Yong Bum Cho, Young Wan Kim, Seong Bin Hong, Yeo Joo Kim, Mi Rim Kim, Moon Suk Nam, Sei Joong Kim, Suk Hwan Shin, Byoung Ick Lee; J Korean Endocr Soc. 1999;14(3):562-567. Published online January 1, 2001

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Abstract PDF: We experienced a case of Cushings syndrome due to adrenal adenoma associated with pregnancy 23th week in a 26 year old female patient. On physical findings, hypertension, moon face, hirsutism, abdominal striae and buffalo hump were noticed. Laboratory findings showed increased serum cortisol, increased 24 hours-urinary cortisol, and decreased plasma ACTH. Abdominal MRI demonstrated 4 cm sized in long diameter, well marginated, and ovoid-shape right adrenal mass. Unilateral total adrenalectomy was performed at the 23th week of pregnancy and the mass it was confirmed to benign adrenal adenoma. Steroid replacement therapy was continued with 7.5 mg prednisolone during remaining pregnancy. At the 38th week of pregnancy, labor was developed and she delivered healthy female weighing 2.5 kg through normal vaginal delivery without complication. Pregnancy rarely occurs in patients with Cushings syndrome. We think she was the first case in Korea who had normal 38th week transvaginal delivery after adrenalectomy in adrenal Cushings syndrome during pregnancy.

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