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Pamidronate treatment stimulates the onset of recovery phase reducing fracture rate and skeletal deformities in patients with idiopathic juvenile osteoporosis: comparison with untreated patients

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Abstract

Although spontaneous remission occurs in patients with idiopathic juvenile osteoporosis (IJO), permanent bone deformities may occur. The effects of long-term pamidronate treatment on clinical findings, bone mineral status, and fracture rate were evaluated. Nine patients (age 9.8 ± 1.1 years, 7 males) with IJO were randomized to intravenous pamidronate (0.8 ± 0.1 mg/kg per day for 3 days; cycles per year 2.0 ± 0.1; duration 7.3 ± 1.1 years; n = 5) or no treatment (n = 4). Fracture rate, phalangeal quantitative ultrasound, and lumbar bone mineral density (BMD) by dual energy X-ray absorptiometry at entry and during follow-up (range 6.3–9.4 years) were assessed. Bone pain improved in treated patients. Difficulty walking continued for 3–5 years in untreated patients, and vertebral collapses occurred in three of them. During follow-up, phalangeal amplitude-dependent speed of sound (AD-SoS), bone transmission time (BTT), and lumbar BMDarea and BMDvolume progressively increased in treated patients (P < 0.05–P < 0.0001). In untreated patients AD-SoS and BTT decreased during the first 2–4 years of follow-up (P < 0.05–P < 0.01); lumbar BMDarea increased after 6 years (P < 0.001) whereas BTT and lumbar BMDvolume increased after 7 years of follow-up (P < 0.05 and P < 0.001, respectively). At the end of follow-up, AD-SoS, BTT, lumbar BMDarea, and BMDvolume Z-scores were lower in untreated patients than in treated patients (−2.2 ± 0.3 and −0.5 ± 0.2; −1.9 ± 0.2 and −0.6 ± 0.2; −2.3 ± 0.3 and −0.7 ± 0.3; −2.4 ± 0.2 and −0.7 ± 0.3, P < 0.0001, respectively). Fracture rate was higher in untreated patients than in treated patients during the first 3 years of follow-up (P < 0.02). Our study showed that spontaneous recovery of bone mineral status is unsatisfactory in patients with IJO. Pamidronate treatment stimulated the onset of recovery phase reducing fracture rate and permanent disabilities without evidence of side-effects.

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Acknowledgments

The authors are very grateful to the parents of all the patients for their consent and help to perform the study.

Conflict of interest

All authors have no conflicts of interest.

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Authors and Affiliations

  1. Pediatric Unit I, Department of Obstetrics, Gynecology and Pediatrics, University-Hospital, Via Roma 67, 56126, Pisa, Italy

    Giampiero I. Baroncelli, Francesco Vierucci & Silvano Bertelloni

  2. Division of Nuclear Medicine, Department of Nuclear Medicine, University-Hospital, Pisa, Italy

    Paola Erba

  3. Division of Dentistry, Department of Surgery, University-Hospital, Pisa, Italy

    Elisa Zampollo & Maria Rita Giuca

Authors
  1. Giampiero I. Baroncelli
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  2. Francesco Vierucci
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  3. Silvano Bertelloni
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  4. Paola Erba
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  5. Elisa Zampollo
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  6. Maria Rita Giuca
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Correspondence to Giampiero I. Baroncelli.

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Baroncelli, G.I., Vierucci, F., Bertelloni, S. et al. Pamidronate treatment stimulates the onset of recovery phase reducing fracture rate and skeletal deformities in patients with idiopathic juvenile osteoporosis: comparison with untreated patients. J Bone Miner Metab 31, 533–543 (2013). https://doi.org/10.1007/s00774-013-0438-9

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  • DOI: https://doi.org/10.1007/s00774-013-0438-9

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