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Paediatric porto-sinusoidal vascular disease: Two different clinical phenotypes with subtle histological differences.

Angelo Di GiorgioLorenza MatarazzoAurelio SonzogniEmanuele NicastroAndrea PietrobattistaMara CananziPaola GaioMarco SciveresGrazia Di LeoRaffaele IorioAntonio MarsegliaGreta CarioliGiuseppe MaggioreMaria GuidoLorenzo D'Antiga
Published in: Liver international : official journal of the International Association for the Study of the Liver (2023)
Paediatric patients with PSVD present with two different clinical phenotypes, one characterised by PH and one by chronic elevation of transaminases without PH. PSVD should be included among the conditions causing isolated hypertransaminasaemia. On histology, the differences between the two groups are subtle. Medium-term outcome is favourable in patients without PH; progression of the disease is observed in those with PH.
Keyphrases
  • end stage renal disease
  • emergency department
  • chronic kidney disease
  • intensive care unit
  • ejection fraction
  • newly diagnosed
  • prognostic factors
  • preterm infants
  • patient reported outcomes