Author/Authors :
Mungan, Neslihan Önenli Çukurova Üniversitesi - Tıp Fakültesi - Çocuk Metabolizma Bilim Dalı, Turkey , Gürbüz, Fatih Çukurova Üniversitesi - Tıp Fakültesi - Çocuk Endokrinolojisi Bilim Dalı, Turkey , Mengen, Eda Çukurova Üniversitesi - Tıp Fakültesi - Çocuk Endokrinolojisi Bilim Dalı, Turkey , Özgür, Özden Çukurova Üniversitesi - Tıp Fakültesi - Çocuk Sağlığı ve Hastalıkları Anabilim Dalı, Turkey , Topaloğlu, Ali Kemal Çukurova Üniversitesi - Tıp Fakültesi - Çocuk Endokrinolojisi Bilim Dalı, Turkey , Yüksel, Bilgin Çukurova Üniversitesi - Tıp Fakültesi - Çocuk Endokrinolojisi Bilim Dalı, Turkey
Abstract :
Purpose: Osteogenesis imperfecta is an inherited disorder of connective tissue. Children with this condition suffer from recurrent fractures, deformities, osteoporosis and pain. Over the recent years, pamidronate became the standard treatment choice. However the optimal dose and interval have not been defined yet. The main of this study was to compare of two different pamidronate regime.Materials and Methods: 12 patients aged 42.3 ± 37.4 months were studied. At the beginning patients had received pamidronate infusion at a dose of 1.5 mg/kg/day once, every two months with duration of 23.5 ± 9.0 months (first protocol), than switched to a dose of 1mg/kg/day for three consecutive days, every three months with duration of 18.5 ± 5.1 months (second protocol). The bone mineral density Z-score was evaluated yearly.Results: Annual fracture rate decreased from 6.3 ± 5.5 to 1.1 ± 1.3 (p=0.001) in the first and from 1.1 ± 1.3 to 0.0 ± 0.0 (p 0.001) in the second protocol. Bone mineral density Z-scores increased from -3.9 ± -1.4 to -2.5 ± -1.3 (p 0.05) in the first, and from -2.5 ± -1.3 ± -1.2 ± -1.1 (p 0.05) in the second protocol.Conclusion: Our study demonstrated that higher yearly doses in 3 consecutive day administration of pamidronate did not provide any additional beneficial effects. Furthermore, higher doses of treatment and longer duration of hospitalization led to the loss of school hours and work hours of parents and was more costly.
