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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References
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).
Arlt, W. et al. Dehydroepiandrosterone replacement in women with adrenal insufficiency. N. Engl. J. Med. 341, 1013–1020 (1999).
Spital, A. Hyponatremia in adrenal insufficiency: review of pathogenetic mechanisms. South. Med. J. 75, 581–585 (1982).
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).
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).
Soule, S. Addison's disease in Africa—a teaching hospital experience. Clin. Endocrinol. (Oxf.) 50, 115–120 (1999).
Arlt, W. & Allolio, B. Adrenal insufficiency. Lancet 361, 1881–1893 (2003).
Moser, H. W., Mahmood, A. & Raymond, G. V. X-linked adrenoleukodystrophy. Nat. Clin. Pract. Neurol. 3, 140–151 (2007).
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).
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).
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).
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).
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).
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).
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).
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).
Løvås, K. & Husebye, E. S. Continuous subcutaneous hydrocortisone infusion in Addison's disease. Eur. J. Endocrinol. 157, 109–112 (2007).
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).
Debono, M. et al. Modified-release hydrocortisone to provide circadian cortisol profiles. J. Clin. Endocrinol. Metab. 94, 1548–1554 (2009).
Arlt, W. The approach to the adult with newly diagnosed adrenal insufficiency. J. Clin. Endocrinol. Metab. 94, 1059–1067 (2009).
White, K. & Arlt, W. Adrenal crisis in treated Addison's disease: a predictable but under-managed event. Eur. J. Endocrinol. 162, 115–120 (2010).
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).
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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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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DOI: https://doi.org/10.1038/nrendo.2010.211