Non-activated protein C rescue treatment in Wilms tumour associated hepatic sinusoidal obstructive syndrome.

Abstract:

:Hepatic sinusoidal obstructive syndrome (HSOS) is a frequent complication in patients undergoing haematopoietic stem cell transplant (HSCT), and more rarely, in paediatric patients receiving conventional chemotherapy for solid tumours. Its diagnosis relies on a combination of clinical signs and symptoms such as hepatomegaly, jaundice, weight gain and fluid retention. HSOS treatment is primarily based on supportive care and anti-fibrinolytic agents. Here we report two patients affected by Wilms tumour who developed life-threatening HSOS that failed to respond to conventional treatment. Both patients recovered after receiving aggressive supportive treatment that included administration of non-activated protein C (Ceprotin®--Baxter).

journal_name

Pediatr Blood Cancer

journal_title

Pediatric blood & cancer

authors

De Leonardis F,Koronica R,Bruno SD,Santoro N

doi

10.1002/pbc.24859

subject

Has Abstract

pub_date

2014-05-01 00:00:00

pages

940-1

issue

5

eissn

1545-5009

issn

1545-5017

journal_volume

61

pub_type

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