Bone mineral density in relation to glucocorticoid substitution therapy in adult patients with 21-hydroxylase deficiency.
OBJECTIVE
There are only limited data on bone mineral density (BMD) in adult patients with 21-hydroxylase deficiency (21-OHD). We have defined the effects of different glucocorticoid substitution therapies on BMD and body composition in these patients.


METHODS
Cross-sectional.


METHODS
Thirty-two adult patients with 21-OHD.


METHODS
Patients were examined auxologically and biochemically. BMD was examined in the left femoral neck and lumbar vertebrae 2-4 (L2-4) with dual X-ray absorptiometry. The results were compared with national reference data.


RESULTS
Mean height was 170.1 cm (-1.36 standard deviation scores (SDS) for the men and 156.3 cm (-1.68 SDS) for the women. These were significantly less (P < 0.001) than the mean national final heights. Mean BMD Z-score ((raw score - population reference mean)/SD) was -0.52 in L2-4 and -0.83 in the left femoral neck. Both these values were significantly less than the reference mean values (P = 0.045 and < 0.001, respectively). Both current and long-term mean glucocorticoid doses showed significant negative correlations with BMD in the left femoral neck as well as in L2-4. Patients substituted with hydrocortisone were less often over-treated and had better BMD Z-score means than patients substituted with prednisone, prednisolone or dexamethasone.


CONCLUSIONS
In the follow-up of patients with 21-hydroxylase deficiency, care needs to be exercised to keep the glucocorticoid substitution dose to a minimum. In most cases decreased bone mineral density is a result of over-substitution.
Jääskeläinen
J
J
Department of Paediatrics, Kuopio University Hospital, Finland.
Voutilainen
R
R
D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000188:drug therapy / Q000503:physiopathology*; D000328:Adult; D015992:Body Mass Index; D015519:Bone Density / Q000187:drug effects*; D003430:Cross-Sectional Studies; D003907:Dexamethasone / Q000627:therapeutic use; D004305:Dose-Response Relationship, Drug; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use; D011241:Prednisone / Q000627:therapeutic use
D005938:Glucocorticoids; D003907:Dexamethasone; D011239:Prednisolone; D011241:Prednisone; D006854:Hydrocortisone