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Roth, Christian

Journal of Pediatric Endocrinology and Metabolism

Editor-in-Chief: Kiess, Wieland

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Volume 23, Issue 11 (Jan 2010)


21-hydroxylase Autoantibody-negative Addison's Disease in a 5-year-old Boy with Adrenal Crisis and Type 1 Diabetes Mellitus

Jenn-Tzong Chang
  • Department of Pediatrics, Kaohsiung Veterans General Hospital, Kaohsiung. Taiwan, R.O.C.
/ Ying-Yao Chen
  • Department of Pediatrics, Kaohsiung Veterans General Hospital, Kaohsiung. Taiwan, R.O.C.
/ Pao-Chin Chiu
  • Corresponding author
  • Department of Pediatrics, Kaohsiung Veterans General Hospital, Kaohsiung. Taiwan, R.O.C.
Published Online: 2010-12-22 | DOI: https://doi.org/10.1515/jpem.2010.189


Patients with type 1 diabetes mellitus (T1DM) have an increased risk of other autoimmune disorders. The combination of Addison's disease with T1DM and/or autoimmune thyroid disease is known as autoimmune polyendocrinopathy type 2 (APS-2). 21-hydroxylase autoantibody (21OHAb) is considered as a valuable marker for identifying patients with autoimmune Addison's disease (AD); however, it is not available in some countries. Here we present a 5-year-old boy with newly diagnosed T1DM, who developed AD with adrenal crisis within only six months, and after 1-year treatment, the test of 21OHAb was negative. This was a rare and the first APS-2 case in Taiwan, because APS-2 affects female adults more often, but not boys. At diagnosis of T1DM, we suggest that checking diurnal cortisol and adrenocorticotropic hormone levels as a baseline evaluations, and if it is available, checking 21OHAb as well. If there is subtle evidence of AD, such as unexplained hypoglycemia or unreasonably reduced insulin requirements, adrenal functions must be studied as soon as possible, even in the 21OHAb-negative T1DM patients. Even if nothing is abnormal, the patient still needs an annual measurement.

KEY WORDS: Addison's disease; autoimmune polyendocrinopathy; autoimmune polyglandular syndrome; type 1 diabetes mellitus; 21-hydroxylase autoantibody

About the article

Corresponding Author: Dr. Pao-Chin Chiu,

Published Online: 2010-12-22

Published in Print: 2010-11-01

Citation Information: Journal of Pediatric Endocrinology and Metabolism, ISSN (Online) 2191-0251, ISSN (Print) 0334-018X, DOI: https://doi.org/10.1515/jpem.2010.189. Export Citation

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