HHH syndrome (hyperornithinaemia, hyperammonaemia, homocitrullinuria), with fulminant hepatitis-like presentation

@article{Fecarotta2006HHHS,
  title={HHH syndrome (hyperornithinaemia, hyperammonaemia, homocitrullinuria), with fulminant hepatitis-like presentation},
  author={Simona Fecarotta and Giancarlo Parenti and Pietro Vajro and Alfredo Zuppaldi and Roberto Della Casa and Massimo Carbone and Antonio Correra and Giancarlo Torre and Silvia Riva and Carlo Dionisi-Vici and Filippo Maria Santorelli and Generoso Andria},
  journal={Journal of Inherited Metabolic Disease},
  year={2006},
  volume={29},
  pages={186-189}
}
We report a 3-year-old Italian patient with the hyperornithinaemia, hyperammonaemia, homocitrullinuria (HHH) syndrome who presented with neurological deterioration after an intercurrent infection. Hyperammonaemia, coagulopathy and moderate hypertransaminasaemia were detected on hospital admission. Severe hepatocellular necrosis with hypertransaminasaemia (aspartate aminotransferase 20 000 UI/L, alanine aminotransferase 18 400 UI/L) and coagulopathy (PT < 5%) rapidly developed within few days… CONTINUE READING
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