HEPATIC IMAGING WITH COMPUTED-TOMOGRAPHY OF CHRONIC TYROSINEMIA TYPE-1

被引：8

作者：

MACVICAR, D

DICKSMIREAUX, C

LEONARD, JV

WIGHT, DGD

机构：

[1] HOSP SICK CHILDREN,DEPT DIAGNOST RADIOL,GREAT ORMOND ST,LONDON WC1N 3JH,ENGLAND

[2] INST CHILD HLTH,DEPT CHILD HLTH,LONDON WC1N 1EH,ENGLAND

[3] ADDENBROOKES HOSP,DEPT PATHOL,CAMBRIDGE CB2 2QQ,ENGLAND

来源：

BRITISH JOURNAL OF RADIOLOGY | 1990年 / 63卷 / 752期

关键词：

D O I：

10.1259/0007-1285-63-752-605

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：

Tyrosinaemia type 1 (fumaryl acetoacetase deficiency, hepato-renal tyrosinaemia) is a rare inborn error of metabolism which, in its chronic form, leads to cirrhosis in early childhood and subsequent development of hepatocellular carcinoma in a high proportion of cases. Imaging with computed tomography has an important role in assessing the progress of the liver disease and may be helpful in timing liver transplantation. The radiological features of seven cases are described and the implications discussed.

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页码：605 / 608

页数：4

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