TY - JOUR
T1 - An unusual presentation of primary adrenal insufficiency with new onset type 1 diabetes
T2 - case report and review of the literature
AU - Graf, Shelby
AU - Poeppelman, Rachel Stork
AU - McVean, Jennifer
AU - Rayannavar, Arpana
AU - Sunni, Muna
N1 - Publisher Copyright:
© 2021 De Gruyter. All rights reserved.
PY - 2022/4/1
Y1 - 2022/4/1
N2 - OBJECTIVES: To describe an atypical presentation of primary adrenal insufficiency in conjunction with new onset type 1 diabetes.CASE PRESENTATION: Here, we describe a case of new-onset type 1 diabetes (T1D) presenting simultaneously with an unusual presentation of primary adrenal insufficiency in a previously healthy 16-year-old. He was admitted for a typical presentation of diabetic ketoacidosis, but with extreme hyponatremia. An extensive workup revealed a low aldosterone level, appropriate cortisol level, and positive 21-hydroxylase antibodies. While the phenomenon of multiple autoimmune conditions developing in the same patient is well-described, this particular case has several atypical aspects. Our patient's case highlights the danger of relying on random serum cortisol in the setting of acute illness to rule out adrenal insufficiency.CONCLUSIONS: Adrenal insufficiency can present as isolated hypoaldosteronism without hypocortisolemia and can manifest as severe hyponatremia in the context of diabetic ketoacidosis. Workup for an unusual presentation of T1D should include a 21-hydroxylase antibody, as well as thyroid and celiac disease studies.
AB - OBJECTIVES: To describe an atypical presentation of primary adrenal insufficiency in conjunction with new onset type 1 diabetes.CASE PRESENTATION: Here, we describe a case of new-onset type 1 diabetes (T1D) presenting simultaneously with an unusual presentation of primary adrenal insufficiency in a previously healthy 16-year-old. He was admitted for a typical presentation of diabetic ketoacidosis, but with extreme hyponatremia. An extensive workup revealed a low aldosterone level, appropriate cortisol level, and positive 21-hydroxylase antibodies. While the phenomenon of multiple autoimmune conditions developing in the same patient is well-described, this particular case has several atypical aspects. Our patient's case highlights the danger of relying on random serum cortisol in the setting of acute illness to rule out adrenal insufficiency.CONCLUSIONS: Adrenal insufficiency can present as isolated hypoaldosteronism without hypocortisolemia and can manifest as severe hyponatremia in the context of diabetic ketoacidosis. Workup for an unusual presentation of T1D should include a 21-hydroxylase antibody, as well as thyroid and celiac disease studies.
KW - Addison's disease
KW - autoimmune polyglandular syndrome
KW - primary adrenal insufficiency
KW - type 1 diabetes mellitus
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U2 - 10.1515/jpem-2021-0193
DO - 10.1515/jpem-2021-0193
M3 - Article
C2 - 34821121
AN - SCOPUS:85120917752
SN - 0334-018X
VL - 35
SP - 531
EP - 534
JO - Journal of Pediatric Endocrinology and Metabolism
JF - Journal of Pediatric Endocrinology and Metabolism
IS - 4
ER -