Diagnosis of adrenal insufficiency in children
- Patricia A Donohoue, MD
Patricia A Donohoue, MD
- Professor of Pediatrics
- Medical College of Wisconsin
Adrenal insufficiency is defined by the impaired synthesis and release of adrenocortical hormones. It is classified based upon whether the etiology is primary, secondary, or tertiary:
●Primary adrenal insufficiency, also known as Addison's disease, results from disease intrinsic to the adrenal cortex (table 1).
●Secondary adrenal insufficiency is caused by either impaired release or effect of adrenocorticotropic hormone (ACTH) from the pituitary gland (table 2).
●Tertiary adrenal insufficiency results from the impaired release or effect of corticotropin releasing factor (CRH) from the hypothalamus (table 3).
In addition, there are disorders of end-organ unresponsiveness to adrenocortical hormones that present in a similar manner as diseases caused by adrenocortical hormone deficiencies. These include glucocorticoid (cortisol) resistance and aldosterone resistance. (See "Causes and clinical manifestations of primary adrenal insufficiency in children", section on 'End-organ unresponsiveness'.)
- Oelkers W. Adrenal insufficiency. N Engl J Med 1996; 335:1206.
- Bornstein SR, Allolio B, Arlt W, et al. Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab 2016; 101:364.
- Donohoue PA. The adrenal gland and its disorders. In: Principles and Practice of Pediatric Endocrinology, Kappy MS, Allen DB, Geffner ME (Eds), Charles C. Thomas Publisher, LTD, Springfield 2005. p.357.
- Pacák K, Palkovits M. Stressor specificity of central neuroendocrine responses: implications for stress-related disorders. Endocr Rev 2001; 22:502.
- Cetinkaya S, Ozon A, Yordam N. Diagnostic value of salivary cortisol in children with abnormal adrenal cortex functions. Horm Res 2007; 67:301.
- Müller J. Aldosterone: the minority hormone of the adrenal cortex. Steroids 1995; 60:2.
- Fibiger J. Beitrage zur kenntniss des weiblichen schweinzwittertums. Virchow Arch Path and Anat 1905; 181:1.
- Haber E, Koerner T, Page LB, et al. Application of a radioimmunoassay for angiotensin I to the physiologic measurements of plasma renin activity in normal human subjects. J Clin Endocrinol Metab 1969; 29:1349.
- Nye EJ, Grice JE, Hockings GI, et al. Comparison of adrenocorticotropin (ACTH) stimulation tests and insulin hypoglycemia in normal humans: low dose, standard high dose, and 8-hour ACTH-(1-24) infusion tests. J Clin Endocrinol Metab 1999; 84:3648.
- Streeten DH, Anderson GH Jr, Dalakos TG, et al. Normal and abnormal function of the hypothalamic-pituitary-adrenocortical system in man. Endocr Rev 1984; 5:371.
- Vanderschueren-Lodeweyckx M, Wolter R, Malvaux P, et al. The glucagon stimulation test: effect of plasma growth hormone and on immunoreactive insulin, cortisol, and glucose in children. J Pediatr 1974; 85:182.
- DIAGNOSTIC APPROACH
- Confirming adrenal insufficiency
- Primary versus secondary and tertiary adrenal insufficiency
- Evaluation for cause
- - Primary adrenal insufficiency
- - Secondary and tertiary adrenal insufficiency
- TESTS OF ADRENOCORTICAL FUNCTION
- Static tests
- - Cortisol
- - ACTH
- - Mineralocorticoid status
- - Adrenal androgens
- - Urinary steroids
- Dynamic tests
- - ACTH stimulation
- Short intravenous ACTH test
- Prolonged ACTH test
- - Tests of ACTH secretory ability
- SOCIETY GUIDELINE LINKS
- SUMMARY AND RECOMMENDATIONS