Lower incidence of bronchiolitis obliterans in pediatric liver-lung transplant recipients with cystic fibrosis.
BACKGROUND
Simultaneous liver-lung transplantation is an infrequent but technically feasible procedure in patients with end-stage lung disease and advanced liver disease. We characterize the outcomes of pediatric patients who underwent this procedure at our institution.


METHODS
We performed a retrospective, case-control study and reviewed the medical records of all patients referred to our transplant program from its inception. Seven patients were listed for simultaneous liver-lung transplant. The five patients who survived to transplant were matched to 13 controls who underwent isolated bilateral sequential lung transplant for underlying diagnosis, age at time of transplant, gender, and era of transplant. Outcome measures included patient and graft survival, occurrence of bronchiolitis obliterans (BO), and episodes of rejection.


RESULTS
Of the five study patients who underwent liver-lung transplant, one died of multiorgan failure 11 days after transplant compared with 9 of 13 controls who died. The median survival for the study patients was 89 months (range, 0-112 months) compared with the controls, who had a median survival of 34 months (range, 0-118 months). The remaining four patients had bronchiolitis obliterans syndrome scores of 0 compared with 5 of 13 control patients (P=0.02). The rate of acute rejection per 100 patient days was 0.012 for the study patients compared with 0.11 for the controls (P=0.025).


CONCLUSIONS
Simultaneous liver-lung transplantation is a technically feasible procedure with excellent long-term outcomes. The surviving study subjects remain free from bronchiolitis obliterans syndrome. These results suggest that the transplanted liver may bestow immunologic privilege to the lung allograft.
Faro
Albert
A
Department of Pediatrics, Washington University in St. Louis, St. Louis, MO 63110, USA. faro_a@kids.wustl.edu
Shepherd
Ross
R
Huddleston
Charles B
CB
Lowell
Jeffrey
J
Gandhi
Sanjiv
S
Nadler
Michelle
M
Sweet
Stuart C
SC
D000293:Adolescent; D001989:Bronchiolitis Obliterans / Q000453:epidemiology* / Q000209:etiology*; D016022:Case-Control Studies; D003550:Cystic Fibrosis / Q000150:complications / Q000601:surgery*; D005260:Female; D006084:Graft Rejection / Q000453:epidemiology; D006801:Humans; D015994:Incidence; D008107:Liver Diseases / Q000150:complications / Q000601:surgery*; D016031:Liver Transplantation; D016040:Lung Transplantation / Q000009:adverse effects*; D008297:Male; D009102:Multiple Organ Failure / Q000209:etiology / Q000401:mortality; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome