J. Jaaskelainen et R. Voutilainen, BONE-MINERAL DENSITY IN RELATION TO GLUCOCORTICOID SUBSTITUTION THERAPY IN ADULT PATIENTS WITH 21-HYDROXYLASE DEFICIENCY, Clinical endocrinology, 45(6), 1996, pp. 707-713
OBJECTIVE There are only limited data on bone mineral density (BMD) in
adult patients with 21-hydroxylase deficiency (21-OHD). We have defin
ed the effects of different glucocorticoid substitution therapies on B
MD and body composition in these patients. DESIGN Cross-sectional. PAT
IENTS Thirty-two adult patients with 21-OHD. MEASUREMENTS Patients wer
e examined auxologically and biochemically. BMD was examined in the le
ft femoral neck and lumbar vertebrae 2-4 (L2-4) with dual X-ray absorp
tiometry. The results were compared with national reference data. RESU
LTS Mean height was 170.1 cm (-1.36 standard deviation scores (SDS) fo
r the men and 156.3 cm (-1.68 SDS) for the women. These were significa
ntly 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 significan
tly less than the reference mean values (P = 0.045 and < 0.001, respec
tively). Both current and long-term mean glucocorticoid doses showed s
ignificant negative correlations with BMD in the left femoral neck as
well as in L2-4. Patients substituted with hydrocortisone were less of
ten over-treated and had better BMD Z-score means than patients substi
tuted with prednisone, prednisolone or dexamethasone. CONCLUSIONS In t
he follow-up of patients with 21-hydroxylase deficiency, care needs to
be exercised to keep the glucocorticoid substitution dose to a minimu
m. In most cases decreased bone mineral density is a result of over-su
bstitution.