肝豆状核变性的骨与关节X线改变(附42例报告)

X ray Features of Bone and Joint Changes in Hepatolenticular Degeneration(Report of 42 cases)

本文对42例肝豆状核变性(HLD)患者进行了骨、关节X 线分析。全组除7例骨质未发现异常外,其余35例有不同程度的骨关节变化(83.3%).结果表明,骨质密度减低、脱钙或骨质软化是HLD 常见的X 线表现,但缺乏特征性,诊断本病对应结合临床体征。此外,骨关节X线改变与年龄及临床类型有关。单纯脱钙、佝偻病主要见于肝豆状核变性型中15岁以下儿童,而骨关节退行性变则全部发生于假性硬化型中的中青年患者。

X ray analysis of bone and jointin 42 cases of hepatolenticular dege-neration(HLD)are reported in thisarticle.There were 7 cases of consi-dering normal and 35 cases of showingdifferent degree of changes(83.8%)in bone and joint radiographs.Theresults showed that the reduction ofbone density,demineralization andosteomalacia were mostly manifestat-ions of HLD.Because the finding wasnot specific to HLD,the diagnosisof the disease should be closely cor-related with the clinical picture.Inaddition,X ray changes of bone andjoint were related to age and clinicaltype.The simple demineralization andrickets were mainly seen in childrenunder 15 year old in the HLD typeand the degenerative osteoarthrosiswere all discovered in youthful andmiddle aged patients in false sclerosis type.