Clinical Application of Liver MR Imaging in Wilson's Disease

被引:19
作者
Cheon, Jung-Eun [1 ,2 ]
Kim, In-One [1 ,2 ]
Seo, Jeong Kee [3 ]
Ko, Jae Sung [3 ]
Lee, Jeong Min [1 ,2 ]
Shin, Cheong-Il [1 ,2 ]
Kim, Woo Sun [1 ,2 ]
Yeon, Kyung Mo [1 ,2 ]
机构
[1] Seoul Natl Univ, Coll Med, Dept Radiol, Seoul 110744, South Korea
[2] SNUMRC, Inst Radiat Med, Seoul 110744, South Korea
[3] Seoul Natl Univ, Coll Med, Dept Pediat, Seoul 110744, South Korea
关键词
Hepatolenticular degeneration; Liver; Magnetic resonance (MR); GENE; DIAGNOSIS; CIRRHOSIS; ATPASE;
D O I
10.3348/kjr.2010.11.6.665
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
Objective: To determine whether there is a correlation between liver MR findings and the clinical manifestations and severity of liver dysfunction in patients with Wilson's disease. Materials and Methods: Two radiologists retrospectively evaluated MR images of the liver in 50 patients with Wilson's disease. The Institutional Review Board approved this retrospective study and informed consent was waived. MR images were evaluated with a focus on hepatic contour abnormalities and the presence of intrahepatic nodules. By using Fisher's exact test, MR findings were compared with clinical presentations (neurological and non-neurological) and hepatic dysfunction, which was categorized by the Child-Pugh classification system (A, B and C). Follow-up MR images were available for 17 patients. Results: Contour abnormalities of the liver and intrahepatic nodules were observed in 31 patients (62%) and 25 patients (50%), respectively. Each MR finding showed a statistically significant difference (p < 0.05) among the three groups of Child-Pugh classifications (A, n = 36; B, n = 5; C, n = 9), except for splenomegaly (p = 0.243). The mean age of the patients with positive MR findings was higher than that of patients with negative MR findings. For patients with Child-Pugh class A (n = 36) with neurological presentation, intrahepatic nodules, surface nodularity, and gallbladder fossa widening were more common. Intrahepatic nodules were improved (n = 8, 47%), stationary (n = 5, 29%), or aggravated (n = 4, 24%) on follow-up MR images. Conclusion: MR imaging demonstrates the contour abnormalities and parenchymal nodules of the liver in more than half of the patients with Wilson's disease, which correlates with the severity of hepatic dysfunction and clinical manifestations.
引用
收藏
页码:665 / 672
页数:8
相关论文
共 23 条
[11]   HEPATIC MORPHOLOGY AND HISTOCHEMISTRY OF WILSONS-DISEASE PRESENTING AS FULMINANT HEPATIC-FAILURE - A STUDY OF 11 CASES [J].
DAVIES, SE ;
WILLIAMS, R ;
PORTMANN, B .
HISTOPATHOLOGY, 1989, 15 (04) :385-394
[12]   Diagnosis and phenotypic classification of Wilson disease [J].
Ferenci, P ;
Caca, K ;
Loudianos, G ;
Mieli-Vergani, G ;
Tanner, S ;
Sternlieb, I ;
Schilsky, M ;
Cox, D ;
Berr, F .
LIVER INTERNATIONAL, 2003, 23 (03) :139-142
[13]   Wilson disease [J].
Gitlin, JD .
GASTROENTEROLOGY, 2003, 125 (06) :1868-1877
[14]   Expanded gallbladder fossa: Simple MR imaging sign of cirrhosis [J].
Ito, K ;
Mitchell, DG ;
Gabata, T ;
Hussain, SM .
RADIOLOGY, 1999, 211 (03) :723-726
[15]  
Kim TJ, 2006, AM J NEURORADIOL, V27, P1373
[16]   Unusual liver MR findings of Wilson's disease in an asymptomatic 2-year-old girl [J].
Ko, SF ;
Lee, TY ;
Ng, SH ;
Lin, JW ;
Cheng, YF .
ABDOMINAL IMAGING, 1998, 23 (01) :56-59
[17]   Regression of nodular liver lesions in Wilson's disease [J].
Kozic, D. ;
Svetel, M. ;
Petrovic, I. ;
Sener, R. N. ;
Kostic, V. S. .
ACTA RADIOLOGICA, 2006, 47 (07) :624-627
[18]   DIFFUSE DISEASE OF THE LIVER - RADIOLOGIC-PATHOLOGICAL CORRELATION [J].
MERGO, PJ ;
ROS, PR ;
BUETOW, PC ;
BUCK, JL .
RADIOGRAPHICS, 1994, 14 (06) :1291-1307
[19]   Clinical presentation, diagnosis and long-term outcome of Wilson's disease: a cohort study [J].
Merle, U. ;
Schaefer, M. ;
Ferenci, P. ;
Stremmel, W. .
GUT, 2007, 56 (01) :115-120
[20]   Genotype-phenotype correlations for a wide spectrum of mutations in the Wilson disease gene (ATP7B) [J].
Panagiotakaki, E ;
Tzetis, M ;
Manolaki, N ;
Loudianos, G ;
Papatheodorou, A ;
Manesis, E ;
Nousia-Arvanitakis, S ;
Syriopoulou, V ;
Kanavakis, E .
AMERICAN JOURNAL OF MEDICAL GENETICS PART A, 2004, 131A (02) :168-173