Reversibility of cirrhotic regenerative liver nodules upon NTBC treatment in a child with tyrosinaemia type I

J. Crone, D. Möslinger, O. A. Bodamer, W. Schima, W. D. Huber, E. Holme, S. Stöckler Ipsiroglu

Research output: Contribution to journalArticle

28 Scopus citations

Abstract

In a male patient with hereditary tyrosinaemia type I (HTI), NTBC [2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexandion] treatment and a diet low in phenylalanine and tyrosine were started at the age of 4 wk. At the recommended average dosage (1 mg kg-1), liver failure improved transiently. After 4 mo of treatment, with increased body weight, the dose had decreased to 0.7 mg kg-1, and diffuse cirrhotic changes in liver parenchyma and multiple nodules were visualized by ultrasonography. Multiple nodules in the liver parenchyma were differentiated from hepatocellular carcinoma by magnetic resonance imaging (MRI) using mangafodipir trisodium as a paramagnetic liver-specific contrast agent. Augmentation of NTBC dosage resulted in a decrease in serum α-fetoprotein levels and in significant regression of liver nodules on MRI. Conclusion: In HTI patients with a poor response to NTBC treatment and/or development of cirrhotic changes of liver parenchyma, augmentation of the recommended NTBC dosage may result in significant improvement of symptoms.

Original languageEnglish (US)
Pages (from-to)625-628
Number of pages4
JournalActa Paediatrica, International Journal of Paediatrics
Volume92
Issue number5
DOIs
StatePublished - May 1 2003

Keywords

  • Hepatocellular carcinoma
  • Hereditary tyrosinaemia type I
  • Liver
  • Magnetic resonance

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Fingerprint Dive into the research topics of 'Reversibility of cirrhotic regenerative liver nodules upon NTBC treatment in a child with tyrosinaemia type I'. Together they form a unique fingerprint.

  • Cite this