Clinical application of stem/stromal cells in cystic fibrosis

Abstract

Cystic fibrosis (CF) is a detrimental hereditary disease with a pathophysiology characterized by thick, sticky mucus in the airways that promotes a proinflammatory and infection-prone environment. While treatment of the disease has drastically improved, affected individuals still have a lower quality of life and increased mortality due to the complexity of the irregulation of the lung environment. One of the major innovative approaches that is currently being investigated for the treatment of the CF pathological phenotype has been cell therapy. With an abundance of sources, a variety of stem cells have been considered as treatment options to improve CF lung function. Current in vitro and in vivo research continue to illustrate the beneficial effects of stem cells for CF via reduction of chronic inflammation and infection, lung epithelium correction, and promotion of mucociliary correction. As a result, stem cell therapy for CF has begun its integration into the first phases of clinical trials. Despite this progress, stem cell therapy faces significant hurdles in its translation to the clinic due to lack of understanding and control of the stem cells. The continuing understanding of the interaction between stem cells and the CF environment, cellular and molecular, is crucial to the future success of stem cell therapy for CF.