title: 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).
authors.affiliation: Division of Paediatric Haematology-Oncology, Department of Pediatrics, Bari, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: De Leonardis
authors.forename: Roberta
authors.initials: R
authors.lastname: Koronica
authors.forename: Stefania D
authors.initials: SD
authors.lastname: Bruno
authors.forename: Nicola
authors.initials: N
authors.lastname: Santoro
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects*; D005260:Female; D005343:Fibrinolytic Agents / Q000627:therapeutic use*; D006504:Hepatic Veno-Occlusive Disease / Q000139:chemically induced / Q000188:drug therapy*; D006801:Humans; D007223:Infant; D007680:Kidney Neoplasms / Q000188:drug therapy*; D008297:Male; D011486:Protein C / Q000627:therapeutic use*; D016896:Treatment Outcome; D009396:Wilms Tumor / Q000188:drug therapy*
chemical_list: D005343:Fibrinolytic Agents; D011486:Protein C
keywords: Wilms tumour; chemotherapy; support care; veno-occlusive disease