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Addisonian crisis in a young man with atypical anorexia nervosa

Abstract

Background. A 22-year-old man was admitted to an intensive care unit with severe hyponatremia, hypotension and somnolence. He had a 9-month history of weight loss and vomiting that had led to a previous diagnosis of atypical anorexia, and he had been transferred from a psychiatric clinic.

Investigations. Generalized hyperpigmentation and dehydration were noted on physical examination. Baseline concentrations of plasma adrenocorticotropic hormone, serum cortisol, plasma renin and serum aldosterone were analyzed. Determination of antibodies against adrenal steroid 21-hydroxylase, measurement of very-long-chain fatty acids and thyroid function tests were performed.

Diagnosis. Primary adrenal insufficiency caused by isolated autoimmune adrenalitis and adrenal crisis.

Management. Immediate administration of hydrocortisone (100 mg bolus dose followed by 100 mg over 24 h per continuous infusion) and physiological saline infusions (initially 4 l over 24 h). After stabilization, initiation of chronic hormone replacement therapy with hydrocortisone (initially 30 mg per day) and fludrocortisone (0.1 mg daily). The patient was instructed on how to adjust hydrocortisone doses in stressful situations and provided with an emergency card.

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Figure 1
Figure 2: Skin hyperpigmentation is a key sign of primary adrenal insufficiency.
Figure 3: Electrocardiogram of the case patient.

References

  1. Erichsen, M. M. et al. Clinical, immunological, and genetic features of autoimmune primary adrenal insufficiency: observations from a Norwegian registry. J. Clin. Endocrinol. Metab. 94, 4882–4890 (2009).

    Article  CAS  PubMed  Google Scholar 

  2. Arlt, W. et al. Dehydroepiandrosterone replacement in women with adrenal insufficiency. N. Engl. J. Med. 341, 1013–1020 (1999).

    Article  CAS  PubMed  Google Scholar 

  3. Spital, A. Hyponatremia in adrenal insufficiency: review of pathogenetic mechanisms. South. Med. J. 75, 581–585 (1982).

    Article  CAS  PubMed  Google Scholar 

  4. Oelkers, W., Diederich, S. & Bähr, V. Diagnosis and therapy surveillance in Addison's disease: rapid adrenocorticotropin (ACTH) test and measurement of plasma ACTH, renin activity, and aldosterone. J. Clin. Endocrinol. Metab. 75, 259–264 (1992).

    CAS  PubMed  Google Scholar 

  5. Betterle, C., Dal Pra, C., Mantero, F. & Zanchetta, R. Autoimmune adrenal insufficiency and autoimmune polyendocrine syndromes: autoantibodies, autoantigens, and their applicability in diagnosis and disease prediction. Endocr. Rev. 23, 327–364 (2002).

    Article  CAS  PubMed  Google Scholar 

  6. Soule, S. Addison's disease in Africa—a teaching hospital experience. Clin. Endocrinol. (Oxf.) 50, 115–120 (1999).

    Article  CAS  Google Scholar 

  7. Arlt, W. & Allolio, B. Adrenal insufficiency. Lancet 361, 1881–1893 (2003).

    Article  CAS  PubMed  Google Scholar 

  8. Moser, H. W., Mahmood, A. & Raymond, G. V. X-linked adrenoleukodystrophy. Nat. Clin. Pract. Neurol. 3, 140–151 (2007).

    Article  PubMed  Google Scholar 

  9. Arlt, W., Rosenthal, C., Hahner, S. & Allolio, B. Quality of glucocorticoid replacement in adrenal insufficiency: clinical assessment vs. timed serum cortisol measurements. Clin. Endocrinol. (Oxf.) 64, 384–389 (2006).

    CAS  Google Scholar 

  10. Alkatib, A. A. et al. A systematic review and meta-analysis of randomized placebo-controlled trials of DHEA treatment effects on quality of life in women with adrenal insufficiency. J. Clin. Endocrinol. Metab. 94, 3676–3681 (2009).

    Article  CAS  PubMed  Google Scholar 

  11. Bornstein, S. R., Breidert, M., Ehrhart-Bornstein, M., Kloos, B. & Scherbaum, W. A. Plasma catecholamines in patients with Addison's disease. Clin. Endocrinol. (Oxf.) 42, 215–218 (1995).

    Article  CAS  Google Scholar 

  12. Zuckerman-Levin, N., Tiosano, D., Eisenhofer, G., Bornstein, S. & Hochberg, Z. The importance of adrenocortical glucocorticoids for adrenomedullary and physiological response to stress: a study in isolated glucocorticoid deficiency. J. Clin. Endocrinol. Metab. 86, 5920–5924 (2001).

    Article  CAS  PubMed  Google Scholar 

  13. Elbelt, U., Hahner, S. & Allolio, B. Altered insulin requirement in patients with type 1 diabetes and primary adrenal insufficiency receiving standard glucocorticoid replacement therapy. Eur. J. Endocrinol. 160, 919–924 (2009).

    Article  CAS  PubMed  Google Scholar 

  14. Løvås, K., Loge, J. H. & Husebye, E. S. Subjective health status in Norwegian patients with Addison's disease. Clin. Endocrinol. (Oxf.) 56, 581–588 (2002).

    Article  Google Scholar 

  15. Hahner, S. et al. Impaired subjective health status in 256 patients with adrenal insufficiency on standard therapy based on cross-sectional analysis. J. Clin. Endocrinol. Metab. 92, 3912–3922 (2007).

    Article  CAS  PubMed  Google Scholar 

  16. Bergthorsdottir, R., Leonsson-Zachrisson, M., Odén, A. & Johannsson, G. Premature mortality in patients with Addison's disease: a population-based study. J. Clin. Endocrinol. Metab. 91, 4849–4853 (2006).

    Article  CAS  PubMed  Google Scholar 

  17. Løvås, K. & Husebye, E. S. Continuous subcutaneous hydrocortisone infusion in Addison's disease. Eur. J. Endocrinol. 157, 109–112 (2007).

    Article  PubMed  Google Scholar 

  18. Johannsson, G. et al. Improving glucocorticoid replacement therapy using a novel modified-release hydrocortisone tablet: a pharmacokinetic study. Eur. J. Endocrinol. 161, 119–130 (2009).

    Article  CAS  PubMed  Google Scholar 

  19. Debono, M. et al. Modified-release hydrocortisone to provide circadian cortisol profiles. J. Clin. Endocrinol. Metab. 94, 1548–1554 (2009).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  20. Arlt, W. The approach to the adult with newly diagnosed adrenal insufficiency. J. Clin. Endocrinol. Metab. 94, 1059–1067 (2009).

    Article  CAS  PubMed  Google Scholar 

  21. White, K. & Arlt, W. Adrenal crisis in treated Addison's disease: a predictable but under-managed event. Eur. J. Endocrinol. 162, 115–120 (2010).

    Article  CAS  PubMed  Google Scholar 

  22. Hahner, S. et al. Epidemiology of adrenal crisis in chronic adrenal insufficiency: the need for new prevention strategies. Eur. J. Endocrinol. 162, 597–602 (2010).

    Article  CAS  PubMed  Google Scholar 

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Acknowledgements

Written consent for publication was obtained from the patient. C. P. Vega, University of California, Irvine, CA, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the MedscapeCME-accredited continuing medical education activity associated with this article.

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B. Allolio, K. Lang and S. Hahner researched the data for the article. B. Allolio and S. Hahner provided a substantial contribution to discussions of the content, contributed equally to writing the article and reviewed and/or edited the manuscript before submission.

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Correspondence to Bruno Allolio.

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The authors declare no competing financial interests.

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Allolio, B., Lang, K. & Hahner, S. Addisonian crisis in a young man with atypical anorexia nervosa. Nat Rev Endocrinol 7, 115–121 (2011). https://doi.org/10.1038/nrendo.2010.211

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