摘要
目的观察原发性高磷酸酶血症患儿骨骼X线异常表现。方法分析21例原发性高磷酸酶血症患儿骨骼异常X线表现。结果双侧长管状骨骨干增粗、对称性弯曲(21/21,100%),皮质增厚分层(14/21,66.67%),骨干骨皮质变薄、骨质吸收并髓腔增宽呈多囊状(7/21,33.33%),骨密度增高(17/21,80.95%)或减低(4/21,19.05%),并见多发骨折(5/21,23.81%)。掌、指骨(13/13,100%)和跖、趾骨呈"长方框"状(12/12,100%),掌、指骨(5/13,38.46%),跖、趾骨(3/12,25.00%)梭形增粗。颅骨见多发圆形骨质吸收区(12/15,80.00%),颅板增厚呈磨玻璃样(13/15,86.67%),异常增大(11/13,84.62%),颅底骨增厚硬化(13/13,100%)及牙槽骨骨硬板骨质疏松或消失(8/13,61.54%)。胸腰椎塌陷变扁(18/18,100%)或呈"夹心椎"(16/18,88.89%);骨盆骨质软化、髋臼内陷、呈小骨盆腔变形(5/5,100%);锁骨、肩胛骨增粗、膨大(7/12,58.33%),肋骨增宽、皮质与髓腔界限不清(8/12,66.67%),肋间隙变窄(5/12,41.67%),可见肋骨骨折(1/12,8.33%)。结论高磷酸酶血症患儿骨骼异常X线表现有一定特征。
Objective To explore the abnormal radiographic findings of skeletons in children with idiopathic hyperphos- phatasia. Methods Imaging data of 21 children with idiopathic hyperphosphatasia were analyzed, and the features of skele- tons in X-ray films were observed. Results Long bones showed diaphysis thickened and bended symmetrically （21/21, 100%）, the cortex thickened with stratification （14/21, 66.67 %） or thinned with bone absorption and medullary cavity broadening as multiple cystic （7/21, 33.33%）, the bone density enhanced （17/21, 80.95%） or reduced （4/21, 19.05%）. In addition, multiple fractures of long bones occurred in 5 cases （5/21, 23.81%）. The short bones showed ＂frame-like＂ sign in metacarpus/hand phalanx （13/13, 100%） and metatarsus/foot phalanx （12/12, 100%）, fusiform thickening in metacarpus/hand phalanx （5/13, 38.46%） and metatarsus/foot phalanx （3/12, 25.00%）. The skulls displayed multiple bone absorption areas （12/15, 80.00 % ） and enlarged abnormally （11/13, 84.62 % ）, the skull plates thickened as ground- glass （13/15, 86.67 %）, the cranial basis thickened and hardened （13/13, 100 %）, and the alveolar bone plate became os- teoporotic or disappeared （8/13, 61.54%）. Thoracic and the lumbar vertebras collapsed （18/18, 100%） or showed as rug- ger-jersey spine （16/18, 88.89%）. Osteomalacia of the pelvis, invagination of the acetabulum and deformation of pelvic ca- nal were observed （5/5, 100%）. The clavicle and the scapula thickened and enlarged （7/12, 58.33 % ）, the ribs broadened with unclear boundary of cortex and medullary cavity （8/12, 66.67%）, and the intercostal space was narrowed （5/12, 41.67%）. Rib fraction occurred in 1 case （1/12, 8.33%）. Conclusion The radiographic findings of tubular bones, skull and spine have certain characteristics in children with idiopathic hyperphosphatasia.
出处
《中国医学影像技术》
CSCD
北大核心
2014年第4期583-586,共4页
Chinese Journal of Medical Imaging Technology
关键词
高磷酸酶血症
X线
骨骼
Hyperphosphatasia
X-rays
Skeleton
