Background: An iatrogenic adrenal insufficiency as the consequence of chronic oral high doses steroid therapy is well known to developmental age medicine, other iatrogenic causes of this condition being rare.
Objective and hypotheses: The primary objective of this study is to describe cases of adrenal dysfunction hypofunction as well as hyperfunction - being caused by factors other than oral steroid therapy.
Method: This is an observational and diagnostic study based on two cases admitted to endocrinology clinics.
Results: Patient 1. at the age of 14 years, with Netherton syndrome (severe erythrodermia with ichtyosis, immunological disorders), was being diagnosed in our clinic because of short stature. During a standard examination, a morning hypocortisolism was identified. Hypoglycemia was not found. Steroid profile confirmed complete supression of adrenal cortex, and Synacthen test was typical for secondary hypoadrenalism. According to the patients history, the most probable cause of adrenal cortex suppression was prolonged use of steroid unguents on a large skin surface. Patient 2. at the age of 5.5, with spinal muscular atrophy, was admitted to the clinic because of the appearance of pubic hair. Urine steroid profile showed significantly increased excretion of glucocorticoids and androgens. Analysis of all data and the lack of symptoms of adrenal dysfunction in the follow-up observation, suggested that a prolonged stress due to the necessity of constant use of a mechanical ventilation was responsible for the adrenal hyperfunction.
Conclusion: Analysis of both cases led to diagnosis of rare iatrogenic causes of adrenal dysfunctions, other than a chronic oral steroid therapy: in case of patient 1 the adrenal cortex suppression was caused by steroid unguents applied externally, in case of patient 2 adrenal hyperfunction was most probably a result of prolonged stress.
01 - 03 Oct 2015
European Society for Paediatric Endocrinology