Risk Factors for Death of Patients with Cystic Fibrosis Awaiting Lung Transplantation

Abstract

We identified risk factors for waiting list mortality that could impact on transplant listing and allocation guidelines.

Pulmonary vascular dysfunction in end-stage cystic fibrosis: role of NF- B and endothelin-1

“…The fact that CF is associated with a strong impact on Ppa and right ventricle function had long been demonstrated [6][7][8], bearing a strongly negative prognostic value [10,12]. Their exact frequency is not known.…”

“…First, CF is a widely distributed chronic obstructive lung disease in which PH and right-sided cardiac failure have long been described [6][7][8][9]. Although PH seems to be relatively rare in this context, it is recognised as a very significant clinical issue with a heavy impact on the prognosis [10][11][12].…”

The Analysis of Cystic Fibrosis in the Federation of Bosnia and Herzegovina

“…It is possible to act on intracellular events and encourage, correct or potentiate the synthesis of mutated CFTR-a (Ivacafor, Lumocaftor, Ataluran), (15,16,17). In the fi nal stage lung disease is available and lung transplantation, individuals with CF must have both lungs replaced because the remaining lung might contain bacteria that could infect the transplanted lung (18). Although the lifespan moved from childhood to adult age, patients are still faced with an abundance of therapeutic needs, and their doctors with a new pathology.…”

Lung Transplantation for Cystic Fibrosis

“…They concluded that ''patients with CF should be referred for transplantation at a time when based on their rate of decline in lung function, the expected time for their percent predicted FEV 1 to reach 20% equals the average local waiting time for donor lungs, or when the baseline percent predicted FEV 1 reaches 30%, whichever comes first.'' Finally, a retrospective cohort study of 343 patients listed for lung transplantation at four academic medical centers was used to generate a multivariate model of waiting list mortality (18 3; 95% CI, 1.3-4.1) were at higher risk. The authors concluded that ''on the basis of these data, the presence of hypercapnia in the setting of better lung function and the requirement for nutritional interventions are additional factors that should be considered in developing future models to be used in allocating organs to the CF population'' (18).…”

“…Finally, a retrospective cohort study of 343 patients listed for lung transplantation at four academic medical centers was used to generate a multivariate model of waiting list mortality (18 3; 95% CI, 1.3-4.1) were at higher risk. The authors concluded that ''on the basis of these data, the presence of hypercapnia in the setting of better lung function and the requirement for nutritional interventions are additional factors that should be considered in developing future models to be used in allocating organs to the CF population'' (18).…”