Restoration of bone mineral density in a patient with congenital adrenal hyperplasia using hydrocortisone pump therapy

Abstract

Osteoporosis is a major public health problem. Glucocorticoids alter the number of cells within bone, reducing bone forming cells (osteoblasts) and those that resorb bone (osteoclasts), the former more so than the latter. Glucocorticoid-induced osteoporosis predominately affects regions of the skeleton such as the spine and the neck of the femur. We describe a 14.5-year-old male with congenital adrenal hyperplasia who developed osteopenia (spinal bone mineral density [BMD] Z-score -1.5; hip BMD -1.7; previously -0.2 and -1.7, respectively) as a result of treatment with prednisolone and high doses of dexamethasone. He was subsequently shown to have a rapid clearance and a half-life of 40 minutes (average normal value 80 minutes) for hydrocortisone. Hydrocortisone delivery using a continuous tiered subcutaneous infusion pump to mimic the normal cortisol circadian rhythm normalized all biochemical parameters and prevented further bone mineral loss over the following 5 years and over 20 years of treatment normalized BMD (spine Z-score +0.4; hip Z-score +0.7). Continuous tiered subcutaneous hydrocortisone pump therapy delivering plasma cortisol concentrations indistinguishable from normal individuals, can prevent bone mineral loss and over a long period leads to a normalization of BMD.