An unusual presentation of primary adrenal insufficiency with new onset type 1 diabetes: case report and review of the literature
Abstract
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.
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Research funding: None declared. 
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Author contributions: All the authors have accepted responsibility for the entire content of this submitted manuscript and approved submission. 
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Competing interests: Authors state no conflict of interest. The funding organization(s) played no role in the study design; in the collection, analysis, and interpretation of data; in the writing of the report; or in the decision to submit the report for publication. 
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Informed consent: Informed consent was obtained from all individuals included in this study. 
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Ethical approval: The research related to human use has been complied with all the relevant national regulations, institutional policies, and in accordance with the tenets of the Helsinki Declaration. 
References
1. Erichsen, MM, Løvås, K, Skinningsrud, B, Wolff, AB, Undlien, DE, Svartberg, J, et al.. Clinical, immunological, and genetic features of autoimmune primary adrenal insufficiency: observations from a Norwegian registry. J Clin Endocrinol Metab 2009;94:4882–90. https://doi.org/10.1210/jc.2009-1368.Search in Google Scholar PubMed
2. Brewer, KW, Parziale, VS, Eisenbarth, GS. Screening patients with insulin-dependent diabetes mellitus for adrenal insufficiency. N Engl J Med 1997;337:202. https://doi.org/10.1056/nejm199707173370314.Search in Google Scholar PubMed
3. Baker, PR, Nanduri, P, Gottlieb, PA, Yu, L, Klingensmith, GJ, Eisenbarth, GS, et al.. Predicting the onset of Addison’s disease: ACTH, renin, cortisol and 21‐hydroxylase autoantibodies. Clin Endocrinol 2012;76:617–24. https://doi.org/10.1111/j.1365-2265.2011.04276.x.Search in Google Scholar PubMed PubMed Central
4. Betterle, C, Scalici, C, Presotto, F, Pedini, B, Moro, L, Rigon, F, et al.. The natural history of adrenal function in autoimmune patients with adrenal autoantibodies. J Endocrinol 1988;117:467–75. https://doi.org/10.1677/joe.0.1170467.Search in Google Scholar PubMed
5. American Diabetes Association. Standards of medical care in diabetes—2010. Diabetes Care 2010;33(1 Suppl):S11–61. https://doi.org/10.2337/dc10-S004.Search in Google Scholar PubMed PubMed Central
6. Dittmar, M, Kahaly, GJ. Polyglandular autoimmune syndromes: immunogenetics and long-term follow-up. J Clin Endocrinol Metab 2003;88:2983–92. https://doi.org/10.1210/jc.2002-021845.Search in Google Scholar PubMed
7. Eisenbarth, GS, Gottlieb, PA. Autoimmune polyendocrine syndromes. N Engl J Med 2004;350:2068–79. https://doi.org/10.1056/nejmra030158.Search in Google Scholar PubMed
© 2021 Walter de Gruyter GmbH, Berlin/Boston
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Articles in the same Issue
- Frontmatter
- Review Article
- Nephrogenic diabetes insipidus: a comprehensive overview
- Original Articles
- Prevalence of type 2 diabetes mellitus, metabolic syndrome, and related morbidities in overweight and obese children
- Impact of sports participation on components of metabolic syndrome in adolescents: ABCD growth study
- Expected or unexpected clinical findings in liver glycogen storage disease type IX: distinct clinical and molecular variability
- Evaluation of patients with phenylalanine metabolism disorder: a single center experience
- The association of grandparental co-residence and dietary knowledge with excess body weight among children aged 7–15 years in China
- Clinical characteristics of polyglandular autoimmune syndromes in pediatric age: an observational study
- Serum kisspeptin, leptin, neuropeptide Y, and neurokinin B levels in adolescents with polycystic ovary syndrome
- Ultrasonographic measurements of the testicular volume in Turkish boys aged 0–8 years and comparison with international references
- Fructose 1,6 bisphosphatase deficiency: outcomes of patients in a single center in Turkey and identification of novel splice site and indel mutations in FBP1
- Benefits of metformin add-on insulin therapy (MAIT) for HbA1c and lipid profile in adolescents with type 1 diabetes mellitus: preliminary report from a double-blinded, placebo-controlled, randomized clinical trial
- Evaluation of efficacy and safety of long-acting PEGylated recombinant human growth hormone (Jintrolong) for patients with growth hormone deficiency
- Evaluation of endocrinological involvement and metabolic status in patients with Gaucher disease Type 1 and Fabry disease under enzyme replacement therapy
- Letter to the Editor
- Correspondence on “Obesity after the Covid-19 pandemic”
- Case Reports
- An unusual presentation of primary adrenal insufficiency with new onset type 1 diabetes: case report and review of the literature
- Niemann–Pick type C disease with a novel intronic mutation: three Turkish cases from the same family
- Weight management in youth with rapid-onset obesity with hypothalamic dysregulation, hypoventilation, autonomic dysregulation, and neural crest tumor (ROHHAD-NET): literature search and case report
- Successful use of cinacalcet monotherapy in the management of siblings with homozygous calcium-sensing receptor mutation