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Presentation of Primary Adrenal Insufficiency in Childhood
Abstract
Context: Primary adrenal insufficiency is usually diagnosed in infancy or adulthood, and cases presenting in childhood have not been systematically reviewed. Objective: Our objective was to determine etiologies, signs, and symptoms of primary adrenal insufficiency presenting in childhood. Design and Setting: We conducted a retrospective chart review at a tertiary-care pediatric hospital. Patients: Patients were children with corticoadrenal insufficiency, glucocorticoid deficiency, or mineralocorticoid deficiency. Results: Seventy-seven cases were identified in 1999–2010. Thirty-five had congenital adrenal hyperplasia (CAH) and were not reviewed further. Forty-two patients (20 diagnosed at our institution) had primary adrenal insufficiency. These had etiologies as follows: autoimmune (18), autoimmune polyendocrinopathy syndrome (an additional five), ACTH resistance (four), adrenoleukodystrophy (three), adrenal hypoplasia congenita (two), adrenal hemorrhage (two), IMAGe syndrome (one), and idiopathic (two). Of 20 patients diagnosed at our institution, two were being monitored when adrenal insufficiency developed and were not included in the analysis of presenting signs and symptoms: 13 of 18 patients were hypotensive; 12 of 18 had documented hyperpigmentation. Hyponatremia (<135 mEq/liter) occurred in 16 of 18. However, hyperkalemia (>5.0 mEq/liter) was noted in only nine. Hypoglycemia and ketosis were documented in four of 15 and four of six patients in whom it was sought, respectively. Fifteen patients underwent cosyntropin stimulation testing with median baseline and stimulated cortisol of 1.1 and 1.2 μg/dl, respectively. ACTH and renin were markedly elevated in all patients. Conclusions: Hyperkalemia is not a consistent presenting sign of primary adrenal insufficiency in childhood, and its absence cannot rule out this condition. A combination of chronic or subacute clinical symptoms, hypotension, and hyponatremia should raise suspicion of adrenal insufficiency.
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“At diagnosis, 80% of boys or adolescents with cALD and 70% of AMN patients have hormonal evidence of clinically silent adrenal insufficiency [65,66,67,68,69].”
From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [69]
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Metadata
- Authors
- Hsieh, S., & White, P. C.
- Affiliations
- The University of Texas Southwestern Medical Center (US)
- Corresponding author
- White, Perrin C.
- ORCID
- White, Perrin C.
- Venue
- The Journal of Clinical Endocrinology & Metabolism
- Publisher
- The Endocrine Society
- Published
- 2011-06-01
- Volume
- 96
- Issue
- 6
- Pages
- E925-E928
- ISSN
- 0021-972X (ISSN-L)
- 1945-7197
- OpenAlex
- W2059681842
- Cited by
- 131
- References
- 12
- Citations by year
- 2026: 2 · 2025: 11 · 2024: 6 · 2023: 3 · 2022: 9 · 2021: 7 · 2020: 13 · 2019: 12 · 2018: 10 · 2017: 10 · 2016: 9 · 2015: 16 · 2014: 7 · 2013: 7 · 2012: 8
- FWCI
- 6.00
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- The Journal of Clinical Endocrinology & Metabolism (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Hormonal Regulation and Hypertension
- Health Sciences › Medicine › Nephrology › Electrolyte and hormonal disorders
- Keywords
- Addison's disease, childhood, pediatric adrenal insufficiency, autoimmune adrenal insufficiency, ACTH resistance, adrenoleukodystrophy, congenital adrenal hypoplasia, adrenal hemorrhage, IMAGe syndrome, hyponatremia, hypotension, hyperpigmentation, hyperkalemia, hypoglycemia, ketosis, ACTH stimulation test, ACTH, renin
- MeSH terms
- Addison Disease, Adolescent, Autoimmune Diseases, Child, Humans, Hypoglycemia, Hyponatremia, Hypotension, Retrospective Studies
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