Height, bone mineral density and bone markers in congenital adrenal hyperplasia.
OBJECTIVE
To evaluate height, bone growth, areal bone mineral density (aBMD), volumetric bone mineral density (vBMD) and markers of bone turnover in a group of patients affected by congenital adrenal hyperplasia (CAH).


METHODS
There were 50 patients (23 males, 27 females), aged 1-28 years, affected by CAH due to 21-hydroxylase deficiency: 27 with the salt-wasting (SW); 14 with the simple virilizing (SV), and 9 with the nonclassical (NC) forms.


METHODS
Bone morphometry was evaluated with the metacarpal index (MI) and lumbar aBMD and vBMD (L2-L4) by dual energy X-ray absorptiometry. Serum osteocalcin was used as a marker of bone formation, while urinary cross-linked N-telopeptides of type-I collagen and free deoxypyridinoline levels were evaluated as indexes of bone resorption.


RESULTS
The height standard deviation score (SDS) was -0.41 +/- 1.4 in SW patients, -0.01 +/- 1.9 in SV patients, and -0.01 +/- 2.3 in NC patients. There was no significant difference among groups and against zero. The MI SDS was also not different between groups and against zero. aBMD was significantly lower in the pubertal patients compared with normal values, but only when patients with the SW and SV forms were considered together (p < 0.05). vBMD was significantly reduced in all patients with the classical form. Bone markers were not different in patients and controls.


CONCLUSIONS
Our study shows that normal height can be attained in CAH patients; however, an impairment in bone growth and mineralization may be found in adolescents and young adults affected by the classical form.
Paganini
C
C
Department of Pediatrics, Regional Hospital of Bolzano, Italy.
Radetti
G
G
Livieri
C
C
Braga
V
V
Migliavacca
D
D
Adami
S
S
D015502:Absorptiometry, Photon; D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000503:physiopathology*; D000328:Adult; D000596:Amino Acids / Q000652:urine; D015415:Biomarkers / Q000032:analysis*; D001827:Body Height; D015519:Bone Density; D001846:Bone Development; D001862:Bone Resorption; D002648:Child; D002675:Child, Preschool; D003094:Collagen / Q000652:urine; D024042:Collagen Type I; D005260:Female; D006801:Humans; D007223:Infant; D008159:Lumbar Vertebrae; D008297:Male; D008663:Metacarpus; D015675:Osteocalcin / Q000097:blood; D010455:Peptides / Q000652:urine
D000596:Amino Acids; D015415:Biomarkers; D024042:Collagen Type I; D010455:Peptides; C052929:collagen type I trimeric cross-linked peptide; D015675:Osteocalcin; C036020:deoxypyridinoline; D003094:Collagen