Abstract
perfecta (OI), with the aim of improving height velocity, bone mineral density (BMD) and projected bone areas.Study design: We observed a randomized population of patients: 30 pre-pubertal children (14 boys and 16 girls) suffering from OI (type I, IV and III), already receiving treatment with Neridronate. After an observational period of 12 months during ongoing Neridronate treatment, the patients were randomized into two groups: Group A, 15 children (7 boys and 8 girls) were treated for 12 months with GH and Neridronate; Group B, 15 (7 boys and 8 girls) continued Neridronate alone. We evaluated auxological parameters, number of fractures, bone age (BA), bone metabolic parameters and bone mass measurements with DXA (lumbar column and radius), in the year before and throughout the GH treatment. Results: Growth velocity was significantly higher during GH treatment in Group A vs Group B and vs pre-treatment period (p<0.05), with no difference in Bone Age advancement. The number of fractures did not increase during GH treatment. IGF-I levels were in the low-normal range before therapy and increased significantly after 12 months in Group A vs Group B and vs pre-treatment (p<0.05). Bone mineral density (BMD) at the lumbar column (L2L3L4), distal radius and ultra distal radius (Δ % BMD), and the projected area of lumbar column increased significantly in Group A vs Group B (p<0.05). With a complete molecular characterization in both type I collagen genes (COL1A1 and COL1A2 ), we differentiate between qualitative and quantitative collagen synthesis defects. Patients with quantitative defects had a higher, although not significant, response to GH treatment in terms of growth velocity and BMD. Conclusions: In patients with Osteogenesis Imperfecta the combined GH Bisphosphonate treatment may give better results than Bisphosphonate treatment alone in terms of growth velocity, higher BMD at the lumbar column and wrist, lumbar column projected area, especially in patients with quantitative collagen synthesis defects.
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