Endocrinology and Metabolism

Case Reports

A Korean Pedigree of Paget Bone Disease: Including a Case of Panostotic Paget Bone Disease complicated with Giant Cell Reparative Granuloma.: Eui Tae Jeong, Jae Hong Park, Do Hyeong Kim, Byoung Oh Jeong, Kyung Soo Ko, Byoung Doo Rhee; J Korean Endocr Soc. 1995;10(4):456-466. Published online November 7, 2019

Abstract PDF: The polyglandular autoimmune syndrome designates the dysfunction of endocrine and nonendocrine system involving two or more organs on the basis of an autoimmune mechanism. The autoimmune nature of these diseases has been based on the presence of lymphocytic infiltration in the affected gland, organ specific autoantibodies in the serum, cellular immune defects and an association with the HLA DR/DQ genes or immune response genes. This syndrome is usually classified into three classes and their etiology or pathogenesis is still not completely understood. A 28-year-old woman developed vitiligo and insulin dependent diabetes mellitus during the treatment of Graves' disease with antithyroid drug. She had a tendency of spontaneous ketonemia and serum c-peptide levels were low(0.21, 0.16ng/mL: fasting and glucagon stimulated). Thyrotrophin binding inhibitor immunoglobulin and pancreas iIslet cell cytoplasmic antibody were positive. We report here a case of polyglandular autoimmune syndrome, type III manifesting Graves' disease, vitiligo, and insulin dependent diabetes mellitus.

A Case of Polyglandular Autoimmune Syndrome.: In Pyo Jun, Suck Hwan Lim, Won Hyep Bae, Seung Jun Kim, Youn Ho Lee, Sun Ho Kim, Jung Kyu Lim, Jin Duk Hur; J Korean Endocr Soc. 1994;10(2):142-147. Published online November 6, 2019

Abstract PDF: Polyglandular autoimmune(PGA) syndrome designates the dysfunction of endocrine and nonendocrine system involving two or more glands on the basis of autoimmunity. There are three types of PGA syndrome and their pathogenesis is still not completely understood. A 27-year-old woman developed polyglandular autoimmune syndrome manifesting insulin-dependent diabetes mellitus, myasthenia gravis and Graves' disease associated papillary thyroid carcinoma. The thyroid antimicrosomal antibody and antiacetylcholine receptor antibody were positive. Her HLA serotype was A2, A11, A62, B60, CW3, CW4, DR4, DR9.We report here a case of polyglandular autoimmune syndrome, type III manifesting insulin-dependent diabetes mellitus, myasthenia gravis and Graves' disease associated with papillary thyroid carcinoma.

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

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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