Title: Budd-Chiari syndrome as presenting symptom of hepatic sarcoidosis in a child, with recurrence after liver transplantation
Authors: Van Brusselen, Daan ×
Janssen, Carl E I
Scott, Christiaan
Bevers, Nanja
Roskams, Tania
Wouters, Carine
van damme-lombaerts, Rita #
Issue Date: Mar-2012
Publisher: Wiley-Blackwell Publishing, Inc.
Series Title: Pediatric transplantation vol:16 issue:2 pages:E58-E62
Article number: 10.1111/j.1399-3046.2011.01535.x
Abstract: Van Brusselen D, Janssen CEI, Scott C, Bevers N, Roskams T, Wouters C, Van Damme-Lombaerts R. Budd-Chiari syndrome as presenting symptom of hepatic sarcoidosis in a child, with recurrence after liver transplantation. Pediatr Transplantation 2011: 00: 000-000. © 2011 John Wiley & Sons A/S. Abstract:  A seven-yr-old boy presented with a severe Budd-Chiari syndrome, complicated by recurrent thrombosis of several successive TIPSs. Because of liver failure secondary to venous outflow tract obstruction and deterioration of his general condition, an emergency liver transplantation was performed. Steroids were discontinued three months after transplantation, and maintenance immunosuppressive therapy consisted of tacrolimus and azathioprine. Seven years later, this patient presented symptoms of recurrence of venous outflow obstruction in the transplant liver, comparable to the initial event. Histopathology of the liver revealed diffuse granulomatous inflammation with confluent non-caseating granulomas compressing the centrolobular veins. Extensive investigations excluded infections, immune deficiency, and systemic vasculitides. After treatment with a high dose of corticosteroids, the granulomas in the allograft disappeared completely. We report the first case of hepatic sarcoidosis, presenting with venous outflow obstruction and recurring after liver transplantation, in a child.
ISSN: 1397-3142
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Laboratory of Pediatric Immunology
Translational Cell & Tissue Research
Section Child - Miscellaneous (-)
× corresponding author
# (joint) last author

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