Experience with GH Therapy in a Patient with Osteogenesis Imperfecta
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概要
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Osteogenesis imperfecta (OI) is a heterogeneous inheritable disorder of the connective tissue with bone fragility. Growth deficiency and bone fracture are cardinal features of OI. We evaluated the effect of hGH treatment on growth promotion, final height and bone fracture in a girl with type I OI. At the age of 10 years, when she was 114.1 cm (-3.4 SD) tall, hGH treatment was started because the standard criteria of GH deficiency were satisfied. No episode of bone fracture had been noted prior to GH treatment, but she suffered bone fractures three times in the first two years' treatment for GH. Because of this, salmon calcitonin was added from the age of 12 years, but she suffered from bone fractures during the further GH treatment. GH treatment was terminated at the age of 17 years. Since being taken off GH therapy, one bone fracture has occurred. Her height ended up at 147.0 cm (-2.2 SD) at the age of 18 years. These findings suggest that GH treatment in a patient with type I OI seems to be effective in promoting growth, but increased frequency of bone fractures during GH treatment warrants careful selection of a patient who merits GH therapy.
- 日本小児内分泌学会の論文
日本小児内分泌学会 | 論文
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