title: Venoocclusive liver disease (VOD) as a complication of Wilms' tumour management in the series of consecutive 206 patients.
abstract: In 4 years (1993-1996) 206 pts. with nephroblastoma were treated. All children were treated according to SIOP 93-01 protocol. Overall survival was 92%. In 27 cases hepatotoxic events occurred. In 10 cases, venoocclusive liver disease (VOD) was diagnosed. VOD is a syndrome associated with hepatomegaly, sudden weight gain or ascites and jaundice. It results from damage to the endothelium of hepatic venules and necrosis of central hepatocytes with subsequent proliferation of fibrous tissue and occlusion of the central hepatic veins. Dactinomycin is one of the drugs considered responsible for its development. Mean age of VOD patients was 4 yrs, however 3 of them were below 1 yr. In all cases, VOD occurred during postoperative chemotherapy (mean 16 th week of treatment). All patients received dactinomycin and vincristine. Five children with right kidney tumors underwent post-operative abdominal irradiation. Main VOD symptoms were hepatomegaly and ascites (80%). Hypertransaminasaemia, as well as, on ultrasound, gallbladder wall thickening and/or free abdominal fluid were observed. Median VOD duration was 27 days and its course was usually temporary and self-limiting. However, in 2 cases recurrent VOD episodes were noted. All children received supportive treatment only. In 6 cases, VOD resulted in chemotherapy delay or drug reductions, while in 4 others chemotherapy was completed preliminarily. Nevertheless it did not affect patients' outcome overall survival in VOD group was 90%.
  
  
  CONCLUSIONS
  Total 5% VOD frequency is similar to other reports. Infants and children receiving abdominal irradiation seem to be at special risk of VOD development.
authors.affiliation: Department of Paediatric Surgery, Medical University of Gdańsk, Poland. pczaud@amedec.amg.gda.pl
authors.forename: P
authors.initials: P
authors.lastname: Czauderna
authors.forename: K
authors.initials: K
authors.lastname: Katski
authors.forename: J
authors.initials: J
authors.lastname: Kowalczyk
authors.forename: A
authors.initials: A
authors.lastname: Kurylak
authors.forename: B
authors.initials: B
authors.lastname: Lopatka
authors.forename: G
authors.initials: G
authors.lastname: Skotnicka-Klonowicz
authors.forename: K
authors.initials: K
authors.lastname: Sawicz-Birkowska
authors.forename: J
authors.initials: J
authors.lastname: Godziński
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects* / Q000627:therapeutic use; D002423:Cause of Death; D056486:Chemical and Drug Induced Liver Injury / Q000209:etiology* / Q000401:mortality; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006504:Hepatic Veno-Occlusive Disease / Q000139:chemically induced* / Q000175:diagnosis / Q000401:mortality; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007680:Kidney Neoplasms / Q000188:drug therapy* / Q000401:mortality; D008130:Lomustine / Q000009:adverse effects* / Q000627:therapeutic use; D008297:Male; D015996:Survival Rate; D014750:Vincristine / Q000009:adverse effects* / Q000627:therapeutic use; D009396:Wilms Tumor / Q000188:drug therapy* / Q000401:mortality
chemical_list: D014750:Vincristine; D008130:Lomustine