title: Adrenal cortical insufficiency--a life threatening illness with multiple etiologies.
abstract: BACKGROUND
  The clinical signs of adrenal cortical insufficiency (incidence, ca. 25 per million per year; prevalence, ca. 400 per million) are nonspecific, and misdiagnoses are therefore common. Glucocorticoid substitution therapy has been in use for 50 years but is not a wholly adequate treatment. Our understanding of this disease remains incomplete in many ways.
  
  
  METHODS
  We selectively searched the Medline database for publications on adrenal cortical insufficiency, with particular attention to studies from the year 2000 onward (search terms: "adrenal insufficiency" or "Addison's disease" or "hypopituitarism").
  
  
  RESULTS
  Hydrocortisone substitution therapy is often given in doses of 10-25 mg/day, timed according to the circadian rhythm. Gastrointestinal and other, febrile infections account for 30-50% of life-threatening adrenocortical crises. Such crises affect 8 of 100 persons with adrenal cortical insufficiency per year and must be treated by the immediate administration of glucocorticoids and fluids. When persons with adrenal cortical insufficiency are acutely ill or are otherwise under unusual stress, they may need additional amounts of hydrocortisone, often in the range of 5-10 mg but occasionally as high as 200 mg. The sustained administration of excessive amounts of steroid can shorten patients lives by several years. Inappropriate substitution therapy can cause other major medical conditions, such as metabolic syndrome and osteoporosis.
  
  
  CONCLUSIONS
  Important measures for the prevention of adrenocortical crises include improved care by treating physicians, education of patients and their families, the provision of emergency identifying documents, and the prescription of glucocorticoid emergency kits.
authors: Department of Endocrinology and Metabolic Diseases, Charité Campus Mitte, Charité Universitätsmedizin Berlin.
authors: Marcus
authors: M
authors: Quinkler
authors: Endocrine Research Unit, Medizinische Klinik - Campus Innenstadt, Klinikum der Ludwig-Maximilian-Universität München.
authors: Felix
authors: F
authors: Beuschlein
authors: Department of Internal Medicine I, Universitätsklinikum Würzburg, Department of Endocrinology.
authors: Stefanie
authors: S
authors: Hahner
authors: Department of Internal Medicine, Division of Endocrinology, Johann Wolfgang Goethe-University, Frankfurt am Main/Germany.
authors: Gesine
authors: G
authors: Meyer
authors: Department of Medicine 1 - Gastroenterology, Pneumology and Endocrinology, Universitätsklinikum Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg.
authors: Christof
authors: C
authors: Schöfl
authors: Department of Clinical Neuroendocrinology, Max Planck Institute of Psychiatry, Munich.
authors: Günter K
authors: GK
authors: Stalla
mesh_terms: D000309:Adrenal Insufficiency / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*; D003131:Combined Modality Therapy / Q000379:methods; D005440:Fluid Therapy / Q000379:methods*; D020249:Hormone Replacement Therapy / Q000009:adverse effects / Q000379:methods*; D006801:Humans; D006854:Hydrocortisone / Q000008:administration & dosage* / Q000009:adverse effects
chemical_list: D006854:Hydrocortisone