Protrusio acetabuli in Marfan's syndrome

被引:10
作者
Yule, SR [1 ]
Hobson, EE
Dean, JCS
Gilbert, FJ
机构
[1] Aberdeen Royal Infirm, Dept Radiol, Aberdeen AB9 2ZB, Scotland
[2] Aberdeen Royal Infirm, Dept Med Genet, Aberdeen AB9 2ZB, Scotland
关键词
Marfan's syndrome; protrusio acetabuli;
D O I
10.1016/S0009-9260(99)91067-X
中图分类号
R8 [特种医学]; R445 [影像诊断学];
学科分类号
1002 ; 100207 ; 1009 ;
摘要
Marfan's syndrome is an autosomal dominant disorder of connective tissue, commonly involving the cardiovascular, ocular, and skeletal systems. Revised criteria for the clinical diagnosis of Marfan's syndrome regard skeletal involvement as a major criterion if at least four of eight typical skeletal manifestations are present, one of which is protrusio acetabuli, Using Kulman's method to determine the presence of protrusio, we analysed the pelvic X-rays of 15 patients with Marfan's syndrome and 15 controls, Protrusio was present in 47% (7/15) of Marfan patients, compared with 7% (1/15) of controls (P = 0.035). Using the revised criteria, the presence of protrusio would have affected the final diagnosis of Marfan's syndrome in only one patient out of 15, Therefore, we recommend that a pelvic X-ray is reserved for those cases in which the presence of protrusio will alter the final diagnosis, With regard to the radiological assessment of protrusio, in our opinion this can be performed simply and reliably using the position of the acetabular line alone.
引用
收藏
页码:95 / 97
页数:3
相关论文
共 7 条
[1]  
ARMBUSTER TG, 1978, RADIOLOGY, V128, P1
[2]  
BEIGHTON P, 1986, AM J MED GENET, V29, P581
[3]  
DePaepe A, 1996, AM J MED GENET, V62, P417, DOI 10.1002/(SICI)1096-8628(19960424)62:4<417::AID-AJMG15>3.0.CO
[4]  
2-R
[5]   MUTATIONS IN THE HUMAN GENE FOR FIBRILLIN-1 (FBN1) IN THE MARFAN-SYNDROME AND RELATED DISORDERS [J].
DIETZ, HC ;
PYERITZ, RE .
HUMAN MOLECULAR GENETICS, 1995, 4 :1799-1809
[6]   ACETABULAR PROTRUSION IN THE MARFAN-SYNDROME [J].
KUHLMAN, JE ;
SCOTT, WW ;
FISHMAN, EK ;
PYERITZ, RE ;
SIEGELMAN, SS .
RADIOLOGY, 1987, 164 (02) :415-417
[7]  
WIBERG G, 1938, ACTA CHIR SCAND S, V58, P1