Impact of total cumulative glucocorticoid dose on bone mineral density in patients with 21-hydroxylase deficiency.
OBJECTIVE
It remains controversial whether long-term glucocorticoids are charged of bone demineralization in patients with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. The aim of this study was to know whether cumulative glucocorticoid dose from the diagnosis in childhood to adulthood in patients with CAH had a negative impact on bone mineral density (BMD).


METHODS
This was a retrospective study.


METHODS
Thirty-eight adult patients with classical and non-classical CAH were included. BMD was measured in the lumbar spine and femoral neck. Total cumulative glucocorticoid (TCG) and total average glucocorticoid (TAG) doses were calculated from pediatric and adult files.


RESULTS
We showed a difference between final and target heights (-0.82+/-0.92 s.d. for women and -1.31+/-0.84 s.d. for men; P<0.001). Seventeen patients (44.7%) had bone demineralization (35.7% of women and 70% of men). The 28 women had higher BMD than the 10 men for lumbar (-0.26+/-1.20 vs -1.25+/-1.33 s.d.; P=0.02) and femoral T-scores (0.21+/-1.30 s.d. versus -1.08+/-1.10 s.d.; P=0.007). In the salt-wasting group, women were almost significantly endowed with a better BMD than men (P=0.053). We found negative effects of TCG, TAG on lumbar (P<0.001, P=0.002) and femoral T-scores (P=0.006, P<0.001), predominantly during puberty. BMI was protective on BMD (P=0.006).


CONCLUSIONS
The TCG is an important factor especially during puberty for a bone demineralization in patients with 21-hydroxylase deficiency. The glucocorticoid treatment should be adapted particularly at this life period and preventive measures should be discussed in order to limit this effect.
Chakhtoura
Zeina
Z
AP-HP, Department of Endocrinology and Reproductive Medicine, Groupe Hospitalier Pitié-Salpêtrière, 47-83 boulevard de l'Hôpital, 75651 Paris Cedex 13, France.
Bachelot
Anne
A
Samara-Boustani
Dinane
D
Ruiz
Jean-Charles
JC
Donadille
Bruno
B
Dulon
Jérôme
J
Christin-Maître
Sophie
S
Bouvattier
Claire
C
Raux-Demay
Marie-Charles
MC
Bouchard
Philippe
P
Carel
Jean-Claude
JC
Leger
Juliane
J
Kuttenn
Frédérique
F
Polak
Michel
M
Touraine
Philippe
P
D015502:Absorptiometry, Photon; D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000188:drug therapy* / Q000201:enzymology / Q000235:genetics; D000328:Adult; D001827:Body Height / Q000187:drug effects; D015519:Bone Density / Q000187:drug effects*; D005260:Female; D005938:Glucocorticoids / Q000009:adverse effects / Q000494:pharmacology / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D011627:Puberty / Q000187:drug effects; D012189:Retrospective Studies; D013255:Steroid 21-Hydroxylase / Q000235:genetics / Q000378:metabolism*
D005938:Glucocorticoids; D013255:Steroid 21-Hydroxylase