Uras Ismail, Uras Nurdan, Karadag Ahmet, Yavuz Osman Yuksel, Atalar Hakan
Fatih University, Faculty of Medicine, Department of Orthopaedics, Ankara, Turkey.
Korean J Radiol. 2005 Jan-Mar;6(1):52-4. doi: 10.3348/kjr.2005.6.1.52.
Hypophosphatasia is a clinically heterogeneous inheritable disorder characterized by defective bone mineralization and the deficiency of serum and tissue liver/bone/kidney alkaline phosphatase activities. Due to the mineralization defect of the bones, various skeletal findings can be radiologically observed in hypophosphatasia. Bowing and Bowdler spurs of long bones are the characteristic findings. The Bowdler spurs reported on in the previous pertinent literature were observed in the perinatal aged patients and these lesions have rarely involved adolescents. We herein report on a 14-year-old girl with fibular Bowdler spurs.
低磷性骨软化症是一种临床异质性的遗传性疾病,其特征为骨矿化缺陷以及血清和组织肝/骨/肾碱性磷酸酶活性缺乏。由于骨骼的矿化缺陷,在低磷性骨软化症中可通过放射学观察到各种骨骼表现。长骨的弓形弯曲和鲍德勒骨棘是其特征性表现。先前相关文献报道的鲍德勒骨棘见于围产期患者,这些病变很少累及青少年。我们在此报告一例患有腓骨鲍德勒骨棘的14岁女孩。