Polyglandular Autoimmune Syndrome Type III with Primary Hypoparathyroidism.
10.3803/EnM.2013.28.3.236
- Author:
Sang Jin KIM
1
;
Sang Yoon KIM
;
Han Byul KIM
;
Hyukwon CHANG
;
Ho Chan CHO
Author Information
1. Department of Internal Medicine, Keimyung University School of Medicine, Daegu, Korea. ho3632@naver.com
- Publication Type:Case Report
- Keywords:
Polyendocrinopathies;
Autoimmune;
Primary hypoparathyroidism
- MeSH:
Adrenal Glands;
Adult;
Autoimmune Diseases;
Diabetes Mellitus, Type 1;
Endocrine Glands;
Female;
Fibrous Dysplasia, Polyostotic;
Hashimoto Disease;
Heredity;
Humans;
Hypoparathyroidism;
Insulin;
Intelligence;
Leukocytes;
Memory;
Neck;
Obesity;
Pseudohypoparathyroidism;
Pseudopseudohypoparathyroidism;
Thyroid Gland;
Thyroiditis;
Thyroiditis, Autoimmune
- From:Endocrinology and Metabolism
2013;28(3):236-240
- CountryRepublic of Korea
- Language:English
-
Abstract:
Polyglandular autoimmune syndrome is defined as multiple endocrine gland insufficiencies accompanied by autoimmune diseases of the endocrine and nonendocrine system. After Schmidt introduced a case of nontuberculosis adrenal gland dysfunction with thyroiditis in 1926, Neufeld defined polyglandular autoimmune syndrome by I, II, and III subtypes in 1980 by their presentation of occurrence age, heredity methods, relationship with human leukocyte antigen, and accompanying diseases. We report a case of a 32-year-old female with polyglandular autoimmune syndrome III accompanied by type 1 diabetes mellitus that was treated with insulin (36 units per day) for 11 years. She had insulin deficiency and Hashimoto thyroiditis as an autoimmune disorder. In addition, she had several features similar to Albright's hereditary osteodystrophy including short stature, truncal obesity, round face, short neck, low intelligence (full IQ 84), and decreased memory. Although Albright's hereditary osteodystrophy is morphological evidence of pseudohypoparathyroidism or pseudopseudohypoparathyroidism, she had primary hypoparathyroidism on laboratory results. Here, we report a case of polyglandular autoimmune syndrome III with type 1 diabetes mellitus, autoimmune thyroiditis, and primary hypoparathyroidism, accompanied by clinical features similar to Albright's hereditary osteodystrophy.
