https://scholars.lib.ntu.edu.tw/handle/123456789/514122
標題: | Cyclic pamidronate infusion for neonatal-onset Osteogenesis imperfecta | 作者: | Lin C.-H. YIN-HSIU CHIEN STEVEN SHINN-FORNG PENG Tsai W.-Y. YI-CHING TUNG CHENG-TING LEE Chien C.-C. WUH-LIANG HWU NI-CHUNG LEE |
公開日期: | 2014 | 卷: | 55 | 期: | 4 | 起(迄)頁: | 306-311 | 來源出版物: | Pediatrics and Neonatology | 摘要: | Background Patients with severe osteogenesis imperfecta (OI; MIM number 259420) suffer from low bone mass, fractures, and bone pain since birth, and have poor prognosis. This study assessed the outcome of patients with severe OI who were treated with cyclic pamidronate prior to the age of 1 year. Methods The six patients, who had bone fractures either in utero or in their 1st month of life, were treated with cyclic pamidronate from a mean age of 2.8 months. Results All the patients tolerated the infusion, except for having transient hypocalcemia at the first infusion. Decreases in irritability and improvements in feeding were observed 2-3 months after the first infusion. All patients showed a rapid increase in bone mineral density over the first 2 years. Fractures occurred at a rate of 0.6/year. At a mean age of 6.4 years, five patients with no interruption in treatment had normal ambulatory function, but they were short in height. Conclusion Patients with neonatal OI can have a favorable outcome when treated with cyclic pamidronate infusions early in life. ? 2014, Taiwan Pediatric Association. Published by Elsevier Taiwan LLC. All rights reserved. |
URI: | https://scholars.lib.ntu.edu.tw/handle/123456789/514122 | ISSN: | 1875-9572 | DOI: | 10.1016/j.pedneo.2013.12.001 | SDG/關鍵字: | calcium; calcium carbonate; colecalciferol; cyclic pamidronate; pamidronic acid; unclassified drug; bisphosphonic acid derivative; pamidronic acid; article; bone density; bone mineralization; bone strength; calcium blood level; childhood disease; clinical article; clinical feature; disease severity; drug half life; drug infusion; feeding behavior; female; fracture; functional assessment; human; hypocalcemia; irritability; male; medical record; newborn; osteogenesis imperfecta; outcome assessment; retrospective study; treatment duration; chemically induced; child; complication; drug administration; drug effects; Fractures, Bone; hypocalcemia; infant; intravenous drug administration; osteogenesis imperfecta; preschool child; Bone Density; Child; Child, Preschool; Diphosphonates; Drug Administration Schedule; Female; Fractures, Bone; Humans; Hypocalcemia; Infant; Infusions, Intravenous; Male; Osteogenesis Imperfecta |
顯示於: | 醫學院附設醫院 (臺大醫院) |
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