CFDB - Cystic Fibrosis DataBase

primary studies published, non RCT

Lower incidence of bronchiolitis obliterans in pediatric liver-lung transplant recipients with cystic fibrosis.

Study design (if review, criteria of inclusion for studies)

retrospective, case-control study

Participants

Seven patients 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.

Interventions

simultaneous liver-lung transplant vs isolated bilateral sequential lung transplant

Outcome measures

Outcome measures included patient and graft survival, occurrence of bronchiolitis obliterans (BO), and episodes of rejection.

Main 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).

Authors' 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.

Keywords: Lung Transplantation; non pharmacological intervention - surg; Respiratory Insufficiency; Respiratory Tract Infections; transplantation; Infection; Respiratory Tract Diseases; Liver Diseases; Gastrointestinal Diseases;