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Hepatic imaging with computed tomography of chronic tyrosinaemia type 1

D. Macvicar, MRCP, FRCR C. Dicks-Mireaux, MRCP, FRCR J. V. Leonard, PhD, FRCP ^* and D. G. D. Wight, FRCPath

Department of Radiology, The Hospital for Sick Children, Great Ormond Street, London ^* Department of Child Health, Institute of Child Health, London Department of Pathology, Addenbrooke's Hospital, Cambridge

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.

Received for publication December 1, 1989. Revision received March 1, 1990.