Inherited Adrenal Hypoplasia: Not Just for Kids!

Lin Lin; John C. Achermann


Clin Endocrinol. 2004;60(5) 

In This Article

Medical Management of Adrenal Hypoplasia

Steroid replacement therapy remains the mainstay of treatment for patients with adrenal hypoplasia. The doses required are similar to those used in other forms of Addison's disease, and are less than the suppressive doses needed for congenital adrenal hyperplasia. In general, infants and children receive hydrocortisone (10-12 mg/m2/day, divided into three doses). Appropriate doses of longer acting steroids, such as prednisolone, may be used after adolescence, once the deleterious effects of these steroids on growth is no longer an issue. Mineralocorticoid replacement is needed when salt loss is evident (e.g, 9α-fludrocortisone, 50-200 µg/day) but the dose should be tailored to individual needs and measurement of plasma renin activity can be useful in assessing control. In general, mineralocorticoid requirements are high in the first months of life and reduce after adolescence. Salt wasters usually require supplementary sodium chloride (4-12 mmol/kg/day in divided doses) for the first 6-12 months of life. Following the diagnosis, the patient (and/or their parents/carers) should be educated in illness-management; intramuscular hydrocortisone and Hypostop© (Bio-Diagnostics Ltd, Worcester UK) should be available to be administered in emergencies; and patients should wear a MedicAlert or Medi-Tag bracelet/pendant and carry a steroid card. Future therapies may include more physiological steroid release preparations or even adrenal transplantation. The potential benefits of adrenal androgen replacement (e.g. DHEA) on mood and sexual function in women with classic Addison's disease is being investigated - these studies could have ramifications for adults with genetic forms of adrenal hypoplasia too (Achermann & Silverman, 2001).


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