Endocrinology and Metabolism

Special Article

Miscellaneous
COVID-19 Vaccination for Endocrine Patients: A Position Statement from the Korean Endocrine Society: Cheol Ryong Ku, Kyong Yeun Jung, Chang Ho Ahn, Jun Sung Moon, Ju Hee Lee, Eun Heui Kim, Hyemi Kwon, Hee Kyung Kim, Sunghwan Suh, Sangmo Hong, Jeonghoon Ha, Eun Roh, Jin Hwa Kim, Mi-kyung Kim, the Committee of Clinical Practice Guideline of the Korean Endocrine Society; Endocrinol Metab. 2021;36(4):757-765. Published online August 17, 2021; DOI: https://doi.org/10.3803/EnM.2021.404

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Since the first outbreak of coronavirus disease 2019 (COVID-19), ongoing efforts have been made to discover an efficacious vaccine against COVID-19 to combat the pandemic. In most countries, both mRNA and DNA vaccines have been administered, and their side effects have also been reported. The clinical course of COVID-19 and the effects of vaccination against COVID-19 are both influenced by patients’ health status and involve a systemic physiological response. In view of the systemic function of endocrine hormones, endocrine disorders themselves and the therapeutics used to treat them can influence the outcomes of vaccination for COVID-19. However, there are very limited data to support the development of clinical guidelines for patients with specific medical backgrounds based on large clinical trials. In the current severe circumstances of the COVID-19 pandemic, position statements made by clinical specialists are essential to provide appropriate recommendations based on both medical evidence and clinical experiences. As endocrinologists, we would like to present the medical background of COVID-19 vaccination, as well as precautions to prevent the side effects of COVID-19 vaccination in patients with specific endocrine disorders, including adrenal insufficiency, diabetes mellitus, osteoporosis, autoimmune thyroid disease, hypogonadism, and pituitary disorders.

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Review Article

Hypothalamus and Pituitary gland
Current National and International Guidelines for the Management of Male Hypogonadism: Helping Clinicians to Navigate Variation in Diagnostic Criteria and Treatment Recommendations: Ahmed Al-Sharefi, Richard Quinton; Endocrinol Metab. 2020;35(3):526-540. Published online September 22, 2020; DOI: https://doi.org/10.3803/EnM.2020.760

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Male hypogonadism—rebadged by some as testosterone deficiency syndrome—is a clinical and biochemical diagnosis of increasing worldwide interest. Organic male hypogonadism—usually permanent—is well-established, but aging men may also exhibit lower serum testosterone levels; principally due to burden of extra-gonadal comorbidities such as obesity, diabetes and metabolic syndrome, but with an underlying intact hypothalamo-pituitary-testicular (HPT) axis capable of springing back into operation once comorbidities are addressed. Despite encouraging observational data and plausible theoretical underpinning, evidence for efficacy and safety of testosterone in this “aging” group of men is lacking; addressing comorbid illnesses remains the key priority instead. Nevertheless, in recent years, accumulation of misleading information online has triggered a global tsunami of testosterone prescriptions. Despite this, many men with organic hypogonadism remain undiagnosed or untreated; many more face a diagnostic odyssey before achieving care by the appropriate specialist. As testosterone therapy is not without risk several clinical practice guidelines have been published specialist societies to guide physicians on best practice. However, these are heterogeneous in key areas, reflecting divergent approaches to the same evidence basis. Herein, we navigate the major clinical practice guidelines on male hypogonadism and test their respective recommendations against current best evidence.

Citations

Citations to this article as recorded by

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Original Article

The Responses of Pituitary Hormones to the Combined Pituitary Stimulation Test in Hypogonadotropic Hypogonadism.: In Myung Yang, Jeong Taek Woo, Sung Woon Kim, Jin Woo Kim, Young Seol Kim, Young Kil Choi, Eun Kyung Park, Kyu Jeong Ahn; J Korean Endocr Soc. 1994;9(2):93-107. Published online November 6, 2019

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Abstract PDF: To classify the causes of hypogonadotropic hypogonadism in Korean patients, and to improve the endocrinologic evaluation for the disease, we retrospectively studied the clinical findings and result of combined pituitary stimulation test in 35 patients with hypogonadotropic hypogonadism. The following results were obtained.1) The ratio of male to female was 1.3:1, and the 50% of male patients was under 20 years of age and the 20% of female patients in 30th decades. 2) The chief complaints of male patients on the admission were the failure of secondary sexual characteristics(95.0%) and loss of hair(5.0%), those of female patients were amenorrhea(46.7%), infertility(26.7%), failure of secondary characteristics(13.3%) and loss of hair(13.3%). 3) The causes of male hypogonadotropic hypogonadism were craniopharyngioma(35.0%), idiopathic(30.0%), Kallmann's syndrome(15.0%), pituitary adenoma(10.0%) and germinoma(5.0%), and those of female hypogonadotropic hypogonadism were prolactinoma(13.3%), Sheehan's syndrome(26.6%), pituitary adenoma(6.7%), tuberculous granuloma(6.7%), germinoma(6.7%), idiopathic hypogonadotropic hypogonadism(40.0%).4) The responses of LH and FSH to GnRH test were absent or markedly blunted in diffuse pituitary diseases such as pituitary tuberculous granuloma, pituitary macroadenomas, Sheehan's syndrome. However those were also absent or blunted in Cushing's disease and hypothalamic disease such as Kallmann's syndrome, germinoma, craniopharyngioma, idiopathic hypogonadotropic hypogonadism. 5) The responses of LH, FSH increased after repeated injection of GnRH in a patient with germinoma. 6) In diffuse destructive pituitary diseases such as Sheehan's syndrome, nonfunctioning macroadenomas, tuberculous granuloma, large prolactinoma, the combined deficiency of pituitary hormones other than gonadotropins was observed. 7) In many cases with hypothalamic diseases, the combined defects of pituitary hormone response were also seen.These data suggest that GnRH test is not always useful to localize the lesion between pituitary and hypothalamus, and combined pituitary stimulation test revealed defects of pituitary hormones other than gonadotropin in various hypothalamic diseases.Therefore repeated GnRH test would be useful for the differential diagnosis, and CRH test and GRH test would be necessary to demonstrate whether pituitary abnormality is present.

Review Article

Adrenal gland
Congenital Hypogonadotropic Hypogonadism and Kallmann Syndrome: Past, Present, and Future: Soo-Hyun Kim; Endocrinol Metab. 2015;30(4):456-466. Published online December 31, 2015; DOI: https://doi.org/10.3803/EnM.2015.30.4.456

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The proper development and coordination of the hypothalamic-pituitary-gonadal (HPG) axis are essential for normal reproductive competence. The key factor that regulates the function of the HPG axis is gonadotrophin-releasing hormone (GnRH). Timely release of GnRH is critical for the onset of puberty and subsequent sexual maturation. Misregulation in this system can result in delayed or absent puberty and infertility. Congenital hypogonadotropic hypogonadism (CHH) and Kallmann syndrome (KS) are genetic disorders that are rooted in a GnRH deficiency but often accompanied by a variety of non-reproductive phenotypes such as the loss of the sense of smell and defects of the skeleton, eye, ear, kidney, and heart. Recent progress in DNA sequencing technology has produced a wealth of information regarding the genetic makeup of CHH and KS patients and revealed the resilient yet complex nature of the human reproductive neuroendocrine system. Further research on the molecular basis of the disease and the diverse signal pathways involved will aid in improving the diagnosis, treatment, and management of CHH and KS patients as well as in developing more precise genetic screening and counseling regime.

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A heterozygous microdeletion of 20p12.2–3 encompassing PROKR2 and BMP2 in a patient with congenital hypopituitarism and growth hormone deficiency
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Gene Expression Changes Underlying Idiopathic Central Hypogonadism in Cryptorchidism with Defective Mini-Puberty
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Brief Report

Obesity and Metabolism
Low Serum Testosterone Concentrations in Hospitalized Men with Poorly Controlled Type 2 Diabetes: Kyung-Soo Kim, San-Ha Kang, Moon-Jong Kim, Soo-Kyung Kim, Yoo-Lee Kim, Won-Keun Park, Seok Won Park, Yong-Wook Cho; Endocrinol Metab. 2014;29(4):574-578. Published online December 29, 2014; DOI: https://doi.org/10.3803/EnM.2014.29.4.574

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Our aim was to examine whether serum testosterone concentrations are in fact low in hospitalized men with poorly controlled type 2 diabetes compared with healthy men. In this study, 79 men aged 40 years or older (41 healthy men and 38 men with type 2 diabetes) were included. Total testosterone and sex hormone-binding globulin levels were measured. The average duration of diagnosed diabetes was 10.8 years and the mean glycated hemoglobin value was 10.8%. Total testosterone concentrations were lower in men with type 2 diabetes than in healthy men, after adjusting for age and body mass index (3.83±0.32 ng/mL vs. 5.63±0.31 ng/mL, P<0.001). In conclusion, this study shows that serum testosterone concentrations are lower in hospitalized men with poorly controlled type 2 diabetes than in healthy men. Therefore, men with poorly controlled type 2 diabetes should undergo further assessment for hypogonadism.

Citations

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Association between testosterone with type 2 diabetes in adult males, a meta-analysis and trial sequential analysis
Jianzhong Zhang, Xiao Li, Zhonglin Cai, Hongjun Li, Bin Yang
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Momordica charantia Extract Protects against Diabetes-Related Spermatogenic Dysfunction in Male Rats: Molecular and Biochemical Study
Gamal A. Soliman, Rehab F. Abdel-Rahman, Hanan A. Ogaly, Hassan N. Althurwi, Reham M. Abd-Elsalam, Faisal F. Albaqami, Maged S. Abdel-Kader
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Olive leaves extract attenuates type II diabetes mellitus-induced testicular damage in rats: Molecular and biochemical study
Gamal A. Soliman, Abdulaziz S. Saeedan, Rehab F. Abdel-Rahman, Hanan A. Ogaly, Reham M. Abd-Elsalam, Maged S. Abdel-Kader
Saudi Pharmaceutical Journal.2019; 27(3): 326. CrossRef
Effects of testosterone supplement treatment in hypogonadal adult males with T2DM: a meta-analysis and systematic review
Jianzhong Zhang, Bin Yang, Wenhui Xiao, Xiao Li, Hongjun Li
World Journal of Urology.2018; 36(8): 1315. CrossRef
RAS and sex differences in diabetic nephropathy
Sergi Clotet, Marta Riera, Julio Pascual, María José Soler
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Original Articles

Bone Metabolism
Testosterone Replacement and Bone Mineral Density in Male Pituitary Tumor Patients: Min Jeong Lee, Hyoung Kyu Ryu, So-Yeon An, Ja Young Jeon, Ji In Lee, Yoon-Sok Chung; Endocrinol Metab. 2014;29(1):48-53. Published online March 14, 2014; DOI: https://doi.org/10.3803/EnM.2014.29.1.48

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Background

Hypopituitarism is associated with osteoporosis and osteopenia especially when hypogonadotropic hypogonadism is present. Despite hypopituitarism being an important cause of secondary osteoporosis, osteoporosis in patients receiving surgery for pituitary tumors in Korea has not been studied. In this study, we evaluated the effects of testosterone replacement therapy (TRT) on bone mineral density (BMD) in postoperative hypogonadal patients with pituitary tumors.

Methods

To examine the effect of TRT on BMD, we performed a retrospective observational study in 21 postoperative male patients who underwent pituitary tumor surgery between 2003 and 2012 at the Ajou University Hospital. Testosterone was replaced in postoperative hypogonadal patients by regular intramuscular injection, daily oral medication, or application of transdermal gel. BMD (g/cm²) measurements of central skeletal sites (lumbar spine, femoral neck, and total femur) were obtained using dual-energy X-ray absorptiometry (GE Lunar). For lumbar spine BMD, L1 to L4 values were chosen for analysis. Femur neck and total femur were also analyzed.

Results

During the follow-up period (mean, 56 months; range, 12 to 99 months) serum testosterone levels increased with the administration of TRT (P=0.007). There was significant improvement (4.56%±9.81%) in the lumbar spine BMD compared to baseline BMD. There were no significant changes in the femur neck BMD or total femur BMD. We did not find any statistically significant relationships between changes in testosterone levels and BMD using Spearman correlation analysis.

Conclusion

Our results indicated that TRT used in the postoperative period for hypogonadal pituitary tumor surgery patients may have beneficial effects on the BMD of the spine.

Citations

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Testosterone supplementation and bone parameters: a systematic review and meta-analysis study
G. Corona, W. Vena, A. Pizzocaro, V. A. Giagulli, D. Francomano, G. Rastrelli, G. Mazziotti, A. Aversa, A. M. Isidori, R. Pivonello, L. Vignozzi, E. Mannucci, M. Maggi, A. Ferlin
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Physiological testosterone replacement effects on male aged rats with orchiectomy-induced osteoporosis in advanced stage: a tomographic and biomechanical pilot study
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Androgens and Androgen Receptor Actions on Bone Health and Disease: From Androgen Deficiency to Androgen Therapy
Jia-Feng Chen, Pei-Wen Lin, Yi-Ru Tsai, Yi-Chien Yang, Hong-Yo Kang
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Testosterone and male rejuvenation
Sevann Helo, Peyton Thomas, Nicholas N. Tadros
Panminerva Medica.2019;[Epub] CrossRef
Systemic Non-Reproductive Effects of Sex Steroids in Adult Males and Females
Syed Imran Ali Shah
Human Physiology.2018; 44(1): 83. CrossRef
Benefits and Health Implications of Testosterone Therapy in Men With Testosterone Deficiency
Abdulmaged M. Traish
Sexual Medicine Reviews.2018; 6(1): 86. CrossRef
Multiple Fractures in Patient with Graves' Disease Accompanied by Isolated Hypogonadotropic Hypogonadism
Hyon-Seung Yi, Ji Min Kim, Sang Hyeon Ju, Younghak Lee, Hyun Jin Kim, Koon Soon Kim
Journal of Bone Metabolism.2016; 23(1): 40. CrossRef
Severity and pattern of bone mineral loss in endocrine causes of osteoporosis as compared to age-related bone mineral loss
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Articles in 'Endocrinology and Metabolism' in 2014
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Michael S. Irwig
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Testosterone Replacement Therapy and Bone Mineral Density in Men with Hypogonadism
Se Hwa Kim
Endocrinology and Metabolism.2014; 29(1): 30. CrossRef

Androgen Receptor Gene CAG Repeat Polymorphism and Effect of Testosterone Therapy in Hypogonadal Men in Korea.: Min Joo Kim, Jin Taek Kim, Sun Wook Cho, Sang Wan Kim, Chan Soo Shin, Kyong Soo Park, Seong Yeon Kim; Endocrinol Metab. 2011;26(3):225-231. Published online September 1, 2011; DOI: https://doi.org/10.3803/EnM.2011.26.3.225

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Abstract PDF

BACKGROUND
As the number of CAG repeats in the androgen receptor (AR) gene increases, transcriptional activities decrease and the effects of testosterone decline. In this study, we evaluated the importance of the CAG repeat polymorphism in regard to the effect/safety of testosterone therapy in hypogonadal Korean men. METHODS: The number of CAG repeats was determined in 42 hypogonadal men who underwent testosterone therapy for more than 24 months between December 1999 and August 2007. Body mass index, lean body mass, body fat, bone mineral density, type I collagen N-telopeptide (NTx), osteocalcin, lipid profile, hematocrit and PSA levels prior to and after 24 months of testosterone therapy were identified in our medical record review. RESULTS: Twenty-four months of testosterone therapy increased lean body mass, hematocrit, and PSA levels and reduced body fat, NTx, and HDL cholesterol levels. The mean number of CAG repeats in the AR gene was 23 +/- 3 (range, 15-29) in hypogonadal Korean men. The number of CAG repeats was not found to be associated with changes in lean body mass, body fat, NTx, HDL cholesterol, hematocrit, or PSA levels during testosterone therapy. CONCLUSIONS: No association between the number of CAG repeats in the AR gene and the effect/safety of testosterone therapy was detected in hypogonadal Korean men.

Citations

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Androgen Receptor CAG Repeat Length as a Risk Factor of Late-Onset Hypogonadism in a Korean Male Population
Jong Wook Kim, Young Dae Bae, Sun Tae Ahn, Jin Wook Kim, Je Jong Kim, Du Geon Moon
Sexual Medicine.2018; 6(3): 203. CrossRef
Positive Correlation between Androgen Receptor CAG Repeat Length and Metabolic Syndrome in a Korean Male Population
Jong Wook Kim, Young Dae Bae, Sun Tae Ahn, Jin Wook Kim, Je Jong Kim, Du Geon Moon
The World Journal of Men's Health.2018; 36(1): 73. CrossRef
Genome-Based Approaches in Endocrinology and Metabolism: From Clinical and Research Aspects
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Endocrinology and Metabolism.2011; 26(3): 208. CrossRef

Case Reports

A Case of Ectopic Neurohypophysis Presenting with Hypogonadism.: In Woon Baek, Ji Hyun Kim, Guk Jin Lee, Kyoung Eun Lee, Hae Lim Lee, Hye Won Lee, Nam Yong Kim, Yon Kwon Ihn, Seung Hyun Ko, Seung Hwan Lee, Je Ho Han; Endocrinol Metab. 2011;26(1):67-71. Published online March 1, 2011; DOI: https://doi.org/10.3803/EnM.2011.26.1.67

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Abstract PDF

Pituitary stalk interruption and ectopic neurohypophysis seen on magnetic resonance Imaging (MRI) are often associated with either isolated growth hormone (GH) deficiency or combined anterior pituitary hormone deficiency, but their pathogenesis is not clear and the clinical data regarding these anatomical defect is limited. We experienced a 23-year-old male with the absence of secondary sexual characteristics and this was accompanied with pituitary stalk dysgenesis and ectopic neurohypophysis. He received growth hormone for a year when he was 12 years old due to his short stature. Sella MRI showed no visible pituitary stalk with minimal high signal change, suggesting ectopic neurohypophysis. The combined pituitary stimulation test revealed blunted responses of growth hormone, follicle stimulating hormone and luteinizing hormone. For the hypogonadotropic hypogonadism, the patient was given testosterone intramuscularly and he gradually developed secondary sexual characteristics. We concluded that the hypogonadism and growth hormone deficiency in this patient was caused by hypopituitarism due to pituitary stalk dysgenesis and ecopic nuerohypophysis.

Citations

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MRI of ectopic posterior pituitary gland with dysgenesis of pituitary stalk in a patient with hypogonadotropic hypogonadism
Ashim Kumar Lahiri, Ramanivas Sundareyan, David Jenkins, Anjumara Nilak
Radiology Case Reports.2018; 13(4): 764. CrossRef
Hypothalamic Hypopituitarism Caused by Pituitary Stalk Dysgenesis
Seong-Ju Lee, Hye-Jin Yoon, A-Reum Cho, Yoo-Jin Um, Keun-Young Park, Dong-Mee Lim, Byung-Joon Kim
Korean Journal of Medicine.2013; 85(4): 420. CrossRef

Primary Hypogonadism Associated with Ankylosing Spondylitis.: Byoung Yeon Jun, Guk Jin Lee, Ji Hyun Kim, Jung Min Lee, Sang Ah Chang; J Korean Endocr Soc. 2008;23(5):352-357. Published online October 1, 2008; DOI: https://doi.org/10.3803/jkes.2008.23.5.352

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Abstract PDF

Male patients with hypogonadism have an increased risk of developing rheumatic diseases. Most causes of hypogonadism related with rheumatic disease are karyotype abnormality such as Klinefelter's syndrome or Turner's syndrome and gonadal dysgenesis. A 24-year-old year male was admitted for pain of both hip joints that had worsened over 2 months. He had hip joint involvement from ankylosing spondylitis and did not show secondary sex characteristics. His sex hormones and gonadotropins levels indicated hypergonadotropic hypogonadism. The karyotype was 46 XY, and there was no obvious cause of hypogonadism. Here we report on clinical features of this first Korean case of primary hypogonadism accompanying ankylosing spondylitis.

Citations

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Ankylosing spondylitis associated with balanced reciprocal X-1 translocation
Young Hoon Kim, Jung Ouk Lee
Yeungnam University Journal of Medicine.2017; 34(1): 80. CrossRef
A Case of Klinefelter's Syndrome Accompanying with Polymyositis
Min Kyu Lee, Byung Sik Kim, Suk Hyun Jung, Gun Hwa Lee, Jin Ok Kim, Dong Hwi Rim, Yu Hwa Lee, Woong Jun Kim, So-Young Bang, Hye-Soon Lee
Journal of Rheumatic Diseases.2012; 19(3): 152. CrossRef

A Case of Patient with Opioid-Induced Adrenocortical Insufficiency and Hypogonadism.: Hai Jin Kim, Chul Sik Kim, Jong Suk Park, Jina Park, Eun Seok Kang, Chul Woo Ahn, Bong Soo Cha, Sung Kil Lim, Kyung Rae Kim, Hyun Chul Lee; J Korean Endocr Soc. 2006;21(3):257-260. Published online June 1, 2006; DOI: https://doi.org/10.3803/jkes.2006.21.3.257

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Abstract PDF: Opioids are known to decrease plasma cortisol and testosterone level in human and other mammals. Nowadays, opioid use is exponentially increasing, but little is known about its side effects. With the help of progressive human science, we can habit longer life and as result, are becoming more avid for healthy life. In this respect, analgesics play important role in maintaining good and healthy quality of life. For this reason, it is important to fully understand its side effects and handle it with special precaution. We are reporting a 22-year-old male who had been taken opioid analgesic for more than six years to relieve chronic, intractable headache. Then, his hormone test revealed hypogonadotropic hypogonadism combined with hypoadrenocorticotropic hypoadrenalism but showed no definite clinical features except for sexual frigidity. After two years of oxycodon discontinuation, we reevaluated that his hormone test, and all other laboratory tests returned to the normal range.

Original Article

Relationship between Adiponectin, Leptin and Body Fat in Men with Hypogonadism Before and After Testosterone Treatment.: Sang Wan Kim, Joon Ku Kang, Do Joon Park, Chan Soo Shin, Kyung Soo Park, Seong Yeon Kim, Bo Youn Cho, Hong Kyu Lee; J Korean Endocr Soc. 2004;19(5):473-484. Published online October 1, 2004

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Abstract PDF: BACKGROUND
Testosterone replacement therapy in men with hypogonadism improves sexual function, decreases body fat, and increases the mass and function of lean muscle. These beneficial effects of testosterone replacement therapy are accompanied by slight lowering of the high density lipoprotein (HDL) cholesterol levels, increase in the hematocrit/hemoglobin ratio and size of the prostate gland. It is presently unknown whether the effect of testosterone on body fat could also reduce the risk of atherosclerotic disease associated with obesity. We investigated the relationship between body fat and blood leptin and adiponectin levels to elucidate the effect of testosterone on body fat metabolism, as well as the effect of testosterone on lipid and bone metabolism. METHODS: We selected 28 men, who were hypogonadal (mean serum testosterone+/-SD, 22.3+/-35.3 ng/dL) due to an organic disease, and them with oral testosterone (testosterone undecanoate) for 12 months. We measured the body composition, serum leptin, plasma adiponectin, biochemical bone markers, bone mineral density, prostate-specific antigen, and serum lipids before and 3, 6 and 12 months after treatment. We analyzed the relationship between body fat and blood leptin and adiponectin levels. RESULTS: The mean serum testosterone concentration reached the subnormal range after 6 months of treatment, which remained for the duration of treatment. The fat mass decreased and muscle mass increased, not within the first 6 months, but principally within 12 months (p<0.05). Although the decrease in the serum leptin level was not statistically significant, there were positive correlations between the leptin level and fat mass before and after 6 months of treatment (p<0.05). The plasma adiponectin did not increase or correlate with body fat parameters. The bone mineral densities of the lumbar spine (L2-L4) and femoral neck did not increased, but the serum osteocalcin and urine N-telopeptide were significantly decreased (p<0.05 and <0.01, respectively). The HDL-cholesterol decreased, principally within the first 6 months (p<0.01), but the total and LDL cholesterols, and the triglycerides remained unchanged during the course of treatment. There was also no change in prostate-specific antigen. CONCLUSION: Twelve months of oral testosterone replacement in men with hypogonadism improved body composition and bone metabolism, but demonstrated subnormal serum testosterone levels, had no effect on the leptin and adiponectin levels and decrease in HDL-cholesterol levels. It will be necessary to examine the long-term effects of testosterone replacement on the incidence of cardiovascular events as well as cardiovascular risk factors in men with hypogonadism

Case Report

A Case of Type II Autoimmune Polyglandular Syndrome: Acute adrenal crisis presented as the first manifestation of Addison's disease in a patient with diabetic ketoacidosis and hypgonadism.: Young Sook Lee, Jong Min Lee, Hyun Ok Park, Sung Kyu Park, Sung Ro Yoon, Seok Young Kim, Bong Yeon Cha, Kwang Woo Lee, Ho Young Son, Sung Koo Kang; J Korean Endocr Soc. 1998;13(1):115-120. Published online January 1, 2001

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Abstract PDF: Type II autoimmune polyglandular syndrome typically presents in adulthood. Insulin dependent diabetes mellitus and thyroid dysfunction are the most frequent manifestations. Addison's disease is the third major endocrine component of this disorder. In this report, we described a thirty-two year-old male patient who had hypogonadism, insulin dependent diabetes mellitus, and mild Addison's disease presenting its first manifestation as an acute adrenal crisis due to diabetic ketoacidosis. The ACTH concentration will be elevated early in the course of Addisons disease even before a significant reduction in the basal cortisol level or its response to exogenous ACTH occurs. Therefore, plasma ACTH measurements serve as a valuable screening study for Addisons disease.

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