Abstract Ref Number = APCP853
Poster Presentation
Arnesya Ayu Pramadyani,Yun Istatik,Aman Bhakti Pulungan Rumah Sakit Pondok Indah Rumah Sakit Cipto Mangunkusumo
Background : Background Osteogenesis Imperfecta (OI) is an inherited disease causing skeletal fragility. Minimal trauma can cause fractures and deformities, causing potential problem in child’s growth and development and sometimes can be fatal. Early treatment with Zoledronic Acid can prevent complications and potential death. Aim To study the effect of Zoledronic acid as first line treatment for baby with Osteogenesis Imperfecta to prevent complications and death. Case Presentation Summary : Two-months old boy with Type 1 Osteogenesis Imperfecta came to the doctor to get treatment for the disease. The patient has history of multiple femur and humerus fractures from minimal trauma at 1 week old. The baby cried unconsolably if he moved part of his bodies. Other clinical appearances are bowing leg and blue sclera. Vitamin D level was 4 mg/dl (very low). Osteogenesis Imperfecta was confirmed by Bone Mineral Density examination. The patient was also suspected with Achondroplasia because the limbs appeared short but eventually ruled out by genetic examination. Zoledronic acid was planned to be given in 3 times, with the range between dosages are 1 and 5 months. First dosage was given 1 month after the diagnosis was confirmed. After first dosage, the baby could move more comfortably and fracture didn’t occurred even if there was minor trauma. At 4 months old, the baby started to move his head. After second dosage, the baby can hold the head up and starts to roll to right and left without crying. Vitamin D and Calcium supplementation were also given to help creating stronger bone. Learning Points/Discussion : Osteogenesis Imperfecta is a serious condition that can create a future problems and complications. Early Zoledronic Acid administration on Baby with OI can help reduce the risk of complications.
Keywords: Type 1 Osteogenesis Imperffecta Zoledrinic Acid Baby
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