What is the prognosis of pediatric adrenal insufficiency (Addison disease)?

Updated: Dec 07, 2018
  • Author: Kimberly Tafuri, DO; Chief Editor: Sasigarn A Bowden, MD  more...
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Answer

With proper treatment and compliance, patients with adrenal insufficiency (Addison disease) can live a normal life span without limitations. However, the prognosis for an untreated patient with adrenal insufficiency (Addison disease) is poor. Some studies have found that those with very high concentrations of cortisol have a worse prognosis and a higher complication rate of secondary sepsis or intestinal perforation.

Death is a common outcome, usually from hypotension or cardiac arrhythmia secondary to hyperkalemia, unless replacement steroid therapy is begun.

A nationwide Swedish study, by Chantzichristos et al, indicated that the mortality risk is higher in patients with both diabetes mellitus and adrenal insufficiency (Addison disease) than in those with diabetes alone. Among the diabetes/adrenal insufficiency patients, the mortality rate was 28%, compared with 10% in patients with just diabetes, with the estimated relative risk increase in overall mortality being 3.89 for the diabetes/adrenal insufficiency group compared with the diabetes patients. Although mortality in both groups most commonly resulted from cardiovascular problems, the death rate from diabetes complications, infectious diseases, and unknown causes was higher in the diabetes/adrenal insufficiency group than in the controls with diabetes. [38]


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