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ABSTRACT

Background: Osteogenesis Imperfecta (OI) or brittle bone disease is a genetically determined congenital

osteoporosis characterized by weakness and fragility of al bones of the body, with resultant frequent
pathological fractures. The effective treatment nowadays for OI is intravenous bisphosphonate especially
pamidronat which giving a progressive functional outcome and decreasing fracture events on OI cases. The aim
of this study is to see how intravenous biphosponate decreasing the symptoms and the fracture events of OI
patient.

Case study: Sy, a 24 months old girl, BW 7,2 kg and BL 70 cm, has been diagnosed with Osteogenesis

Imperfecta type III since she was 4 months old. Since she was born she has an abnormality on her feet, the feet
bend like crossing sitting and also her hands, and has a blue sclera. She always cried and looked painful without
any cause. No history of trauma. After has been diagnosed with OI type III, she has intravenous disodium
pamidronat (Pamisol) 0.5 mg/kgBW/time every three months.
The observation began on the sixth course of pamidronat (2009), since these periods there was only one new
fracture. According to her parents since the treatment of pamidronat begins the complain had been drecreasing
and looked less painful and less crying. She also can move her four extrimities and began to try new movement.
There had been six fracture events happened since the treatment began, from 2009 until 2014. There was two
fracture events of tibia fibula sinistra, one fracture of femur sinistra, and three fracture events of femur dextra.
It all had been performed with immobilization splinting with spalk, immobilization with skin traction,
immobilization with cast, and open reduction with internal fixation.
During first year observation she had a progressivity of her disease, which was signed by there was a new
fracture when she was 24 months old and 35 months old, there was a deformity on the old fracture, the left leg
became bowing extremity, abnormal growth development with short stature and the gross motoric was delayed.
The absence of the pain and decreasing fracture events caused the increasing of mobility, which affected to her
skeletal system development. It was contributed to quality of life of the OI patient. The lack of fracture event in
the other side increased the injury risk, which caused by lots of mobility that showed to the effect of the therapy.
And now when she is 6 years old, the gross motoric is fine.

Conclusion: the intravenous bisphosphonate therapy showing that the therapy improving the OI patient

quality of life by decreasing the pain and the fracture events, even though at the first year observation she had
an abnormal growth development with short stature and the gross motoric was delayed. Through the therapy
cycles she has been capable of moving without any pain and improvement of her gross motoric. But these
situations affected to the increasing the injury risk of fracture events on the later years.
Keyword: Osteogenesis Imperfecta, OI, fracture events, delayed of gross motoric