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. 2013 Feb 19:11:6.
doi: 10.1186/1546-0096-11-6. eCollection 2013.

Idiopathic juvenile osteoporosis: a cross-sectional single-centre experience with bone histomorphometry and quantitative computed tomography

Justine Bacchetta  1 , Katherine Wesseling-Perry  1 , Vicente Gilsanz  2 , Barbara Gales  1 , Renata C Pereira  1 , Isidro B Salusky  1
Affiliations

Idiopathic juvenile osteoporosis: a cross-sectional single-centre experience with bone histomorphometry and quantitative computed tomography

Justine Bacchetta et al. Pediatr Rheumatol Online J. .

Abstract

Background: Idiopathic juvenile osteoporosis (IJO) is a rare condition of poorly understood etiology and pathophysiology that affects otherwise healthy children. This condition is characterized clinically by bone pain and vertebral fractures; spontaneous recovery is observed after puberty in the majority of cases. Although decreased trabecular bone turnover has been noted previously, cortical and trabecular bone characteristics as determined by quantitative computed tomography (QCT) and their relationship to bone histomorphometry are unknown.

Methods: All children with a clinical diagnosis of IJO who were followed in our center since 1995 and who had undergone at least one diagnostic bone biopsy were included in this cross-sectional analysis.

Results: Fifteen patients (11 males/4 females) with median ages of 5.8 and 10.2 years at first symptoms and at referral, respectively, were included in the analysis. Histomorphometric analysis demonstrated decreased trabecular bone turnover (BFR/BS) in the majority of patients with heterogeneous parameters of trabecular mineralization and volume. QCTresults demonstrated that bone mineral density (BMD) was reduced in both trabecular/lumbar and cortical/femoral bone: Z score: -2.1 (-3.6;-1.0) and -0.9 (-8.2;1.4)in the two compartments, respectively. In the eight patients who underwent both bone biopsy and QCT, cortical BMD was associated with trabecular separation and with trabecular bone formation rate (r = 0.898 and -0.881, respectively, both p < 0.05).

Conclusions: This series confirms that IJO is characterized by impaired trabecular architecture that can be detected by both bone biopsy and QCT. The association between bone biopsy and QCT results may have implications for diagnosis, treatment, and follow-up of these children.

Keywords: Bone biopsy; Histomorphometry; Idiopathic juvenile osteoporosis; QCT.

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Figures

Figure 1
Figure 1
Bone biopsy (Goldner'strichrome, magnification 40x) showing in red, the unmineralized matrix and in green the mineralized bone matrix. A- in a healthy control: the trabecular micro-architecture and connectivity are normal. B- in a patient with IJO: in addition to a global thinning of the trabeculae, there are disconnections within the trabecular network.
Figure 2
Figure 2
Relationship between trabecular BMD at the spine (QCT) and BFR/BS on bone biopsy (r = −0.881, p = 0.004). BMD: bone mineral density. QCT: quantitative computed tomography. BFR/BS: bone formation rate/bone surface.
See this image and copyright information in PMC

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