Abstract Ref Number = APCP845
OSTEOGENESIS IMPERFECTA IN CHILDREN REPORT OF THREE CASES
Mutmainnah Hamzah,Ratna Dewi Artati,Kwari Januar Satriono
Deptartemen Ilmu Kesehatan Anak FK UNHAS Dept Ilmu Kesehatan Anak FK UNHAS
Background : Osteogenesis Imperfecta (OI) is an uncommon congenital abnormality of the connective tissues causing bone fragility and some extra-skeletal features. The consequence of recurrent fractures will result in deformity, short stature, chronic pain and affect quality of life, zoledronic acid (ZA) are first line of pharmacological therapy.
Case Presentation Summary : We conducted a retrospective review of three cases of patients with osteogenesis imperfecta from the past 3 years in Wahidin Sudirohusodo Hospital. All three of our patients presented with recurrent fractures, blue sclera and deformity of lower extremites. The history of antenatal ultrasound scan in one patient was reported abnormality in lower extremites and short stature. All patients treated with intravenous ZA, the initial dose is 0,025 mg/kg and then increased to 0.05 mg/kg, every six months. One patient had side effects of fever after first drug administration, and pain in second therapy, but in the third therapy the patient had no fever and the pain was reduced. All three of the bone x-ray showed of multiple fractures, osteoporosis and deformity. Skulls x-ray in two patients showed cortex thinning and in other patients showed wormian bones. During ZA therapy no patients had recurrent fractures. Pain is gradually reduced after administration of ZA. In two patients there were no pain after the third cycle therapy and the other patient on the second cycle of therapy.
Learning Points/Discussion : • OI involves multidisciplinary management. The goals of treatment are to reduced fracture rates, improve mobility and quality of life. • Children with recurrent fractures caused by minor trauma, should be suspected with OI. • Treatment with ZA brought significant clinical improvements, reduce pain, reduce fracture incidence, and better mobility for children with OI.
Keywords: Osteogenesis Imperfecta Zelodronic Acid Children Fracture