title: Isolated liver transplantation in children with cystic fibrosis--an Australian experience.
abstract: CF liver disease is an uncommon indication for pediatric LT. Determining optimal timing and type (isolated liver versus multi-organ) of transplantation for those with severe liver disease can be challenging and involves consideration of the extent of liver disease (PHT, synthetic dysfunction) and extrahepatic factors such as pulmonary function. We present the experience of isolated LT for CF at our center. Eight children received one allograft each (3.9% of all grafts). One- and four-yr survivals are both 75%. The two deaths occurred within the first two months after LT, and in both cases, invasive fungal infections were implicated, one following treatment for acute severe rejection. All had significant PHT, and six had synthetic dysfunction. All had roux-en Y biliary anastomoses and none developed long-term biliary complications. Seven had pulmonary colonization with Pseudomonas aeruginosa and six with fungus at time of transplantation. Mean pre-LT FEV1 was 80% (range 59-116%) predicted, and lung function post-LT was stable. Isolated LT in children with CF is successful in those with relatively preserved pulmonary function, which does not appear to deteriorate as a consequence. Roux-en Y biliary anastomosis and antifungal prophylaxis should be a part of management of these patients.
authors.affiliation: The Childrens Hospital at Westmead, Sydney, NSW, Australia.
authors.forename: Scott
authors.initials: S
authors.lastname: Nightingale
authors.forename: Edward V
authors.initials: EV
authors.lastname: O'Loughlin
authors.forename: Stuart F A
authors.initials: SF
authors.lastname: Dorney
authors.forename: Albert
authors.initials: A
authors.lastname: Shun
authors.forename: Deborah J
authors.initials: DJ
authors.lastname: Verran
authors.forename: Simone I
authors.initials: SI
authors.lastname: Strasser
authors.forename: Geoffrey W
authors.initials: GW
authors.lastname: McCaughan
authors.forename: Vicki
authors.initials: V
authors.lastname: Jermyn
authors.forename: Peter
authors.initials: P
authors.lastname: Van Asperen
authors.forename: Kevin J
authors.initials: KJ
authors.lastname: Gaskin
authors.forename: Michael O
authors.initials: MO
authors.lastname: Stormon
mesh_terms: D000293:Adolescent; D000935:Antifungal Agents / Q000627:therapeutic use; D001315:Australia; D002648:Child; D003550:Cystic Fibrosis / Q000150:complications* / Q000601:surgery; D005260:Female; D006084:Graft Rejection / Q000517:prevention & control; D006801:Humans; D006975:Hypertension, Portal / Q000601:surgery; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008107:Liver Diseases / Q000209:etiology / Q000601:surgery*; D016031:Liver Transplantation / Q000379:methods; D008297:Male; D012189:Retrospective Studies
chemical_list: D000935:Antifungal Agents; D007166:Immunosuppressive Agents