ESPE Abstracts (2016) 86 P-P1-101

ESPE2016 Poster Presentations Bone & Mineral Metabolism P1 (48 abstracts)

Effect of Bisphosphonates on Bone Fragility Due to Chronic Liver Disease in Ten Children

Emmanuel Gonzales b, , Martha Darce a, , Alessia Usardi a, & Agnès Linglart a,

aReference Center for Rare Disorders of the Mineral Metabolism, Le Kremlin Bicêtre, France; bPlatform of Expertise for Rare Diseases Paris-Sud, Le Kremlin Bicêtre, France; cReference Center for Biliary Atresia Disorder, Le Kremlin Bicêtre, France

Background: Children suffering from chronic liver disease (CLD) may develop rickets, impaired bone mineralization and are exposed to an increased risk of osteoporotic fractures. Bisphosphonate (BP) is used in children to increase the bone density and reduce the fracture incidence. Up to date, no study showing the effect of this treatment in children suffering from CLD has been reported.

Objective and hypotheses: Evaluate the effect of BPs in children with CLD and osteoporotic fractures.

Method: In this monocentric retrospective study, children with a CLD and osteoporotic fracture/s treated with BP were included. Clinical, biological, osteodensitometric data and type and doses of BP were collected.

Results: Ten patients (7M/3F) were included in the study. The mean bone mineral density (BMD) before BP was −2.2 S.D. (n=6). They experienced 6.6 fractures/patient in the 6 months preceding the BPs. The treatment with BP was started at an average age of 6.8 years (±1.6). Three patients were liver transplanted 5 months (in average) after the first BP treatment. After 1 year of BP therapy, the rate of fractures significantly decreased from 6.6 to 0.3 fractures/year and 8/10 patients did not present any new fracture. The average cumulative doses of BP was 2.8 mg/kg. patient (±1.9). No serious adverse event was observed on BP therapy.

Conclusion: Our results suggest that BP therapy could be effective and safe in children with osteoporosis and chronic liver disease.

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