Robert E. Greenberg

Addison's disease in children; report of two cases.

Summary Two cases of primary chronic adrenal insufficiency (Addisons disease) in children are presented. The symptoms and signs of chronicadrenocortical insufficiency seldom suffice to certify a diagnosis. Similarly, reliance on laboratory methods that do not involve actual measurements of adrenal hormones in plasma and urine may obscure an underlying adrenal insufficiency by false-negative results. The use of the quantitative adrenalresponse to ACTH stimulation as reflected in urinary excretion of 17-ketosteroids and 17-hydroxycorticoids and plasma 17-hydroxycorticoid levels provides the most accurate laboratory tool now available in the diagnosis of Addisons disease. The use of 9-alpha-fluorohydrocortisoneprovides an agent which, when used together with cortisone or hydrocortisone, promises to allow effective, safe, and relatively inexpensive oral therapy for chronic adrenal insufficiency. Appendix of Methods .—(1) Blood sugarswere done by Folin-Wu method. (2) Urinary creatinines were done by Folin method. (3) Urinary 17-ketosteroids were done by the Klendshoj modification of the Drekter method. 19 (4) Urinary 17-hydroxycorticoids were done by a modification of the Reddy procedure. 20 (5) Plasma 17-hydroxycorticoids were done by an adaptation of the method published by Silber and Bush. 21