Combination therapy bisphosphonates and rhGH for children with osteogenesis imperfecta
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1
University of Medicine and Pharmacy 'Gr.T.Popa' Iasi, Department of Endocrinology, Romania
Introduction: Osteogenesis Imperfecta (OI) is a group of genetic disease with a wide spectrum of severity, ranging from very mild bone fragility to lethal forms. Patients with OI type I A have a mild phenotype with normal or near-normal height. The addition of recombinant human growth hormone (rhGH) to ongoing treatment with bisphosphonates can increase measures of BMD and growth.{BR}Objectives: We studied growth rate, bone density and bone metabolism in two patients affected by type IA O.I. and growth delay.{BR}Material and methods: Eight children (six girls and two boys) with OI type IA were treated with Risedronate. Among them two girls with mean age 6.5 years presented growth delay. These girls were treated with rhGH at a dose 0.2 mg/kg/week for two years. Auxologic data were measured every 3 months, bone age was determined at the start and at every six months. At every 3 months IGF 1, osteocalcin, alkalinphosphatase, calcium and phosphorus levels and urinary hydroxyproline and calcium levels were determined. Bone mineral density (BMD) measurements were made at the start and repeated at 12 and 24 months at the lumbar spine and whole body by DXA. {BR}Results: After 24 months, linear growth velocity increased significantly: from 4.5 cm/yr. to 8 cm/yr. in the first year and to 8.5 cm/yr. in the second year. DXA-BMD at lumbar spine increased significantly from 0.359 g/cm2 to 0.464 g/ cm2 (+29 %) in the first year and at 0.505 g/cm2 (+9%) in the second one. Whole body - bone density improved +36% in the first year and 4.4% after second year. Serum osteocalcin levels increased significantly after rhGH treatment from 1.9±0.8 nmol/L at 3.9±0.9 nmol/L after the second year.{BR}Conclusion: rhGH treatment addition at bisphosphonates in O.I. type I, increases significantly the rate of linear growth velocity. The bone turnover increases also and the bone mineral density in lumbar spine and whole body increases; the fracture risk does not increase. More children should be treated with rhGH to see the long term benefits of this treatment in final adult height and quality of OI bone.
Keywords:
Bones,
Bone Research
Conference:
2011 joint meeting of the Bone Research Society & the British Orthopaedic Research Society, Cambridge, United Kingdom, 27 Jun - 29 Jun, 2011.
Presentation Type:
Poster
Topic:
Abstracts
Citation:
Galesanu
C,
Lisnic
N,
Florescu
A,
Loghin
A,
Zaharia
V and
Apostu
L
(2011). Combination therapy bisphosphonates and rhGH for children with osteogenesis imperfecta.
Front. Endocrinol.
Conference Abstract:
2011 joint meeting of the Bone Research Society & the British Orthopaedic Research Society.
doi: 10.3389/conf.fendo.2011.02.00018
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Received:
30 Sep 2011;
Published Online:
30 Sep 2011.
*
Correspondence:
Prof. C Galesanu, University of Medicine and Pharmacy 'Gr.T.Popa' Iasi, Department of Endocrinology, Romania, comigalesanu@yahoo.co.uk