CHEST Guidelines-Primary-Ciliary-Dyskinesia-and-the-Heart

Primary-Ciliary-Dyskinesia-and-the-Heart_chest

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The article discusses two main studies published in the November 2014 issue of CHEST journal. The first study examines the role of Hepatocyte Growth Factor (HGF) and Vascular Endothelial Growth Factor (VEGF) in chronic obstructive pulmonary disease (COPD). It finds that while HGF concentrations are elevated in the peripheral airways of normal smokers, they are reduced in COPD patients, suggesting a potential role in preventing fibrosis. The study proposes therapeutic strategies for COPD, including HGF protein therapy or gene therapy via stem cells, despite known associations of HGF with non-small-cell lung cancer—a common risk in COPD patients.<br /><br />The second editorial focuses on primary ciliary dyskinesia (PCD) and its association with congenital heart disease (CHD) and heterotaxy, a condition involving abnormal organ arrangement. The article interests in the potential overlap between PCD and CHD, as a significant number of patients with heterotaxic CHD exhibit signs of respiratory ciliary dysfunction linked to PCD. This suggests screening for CHD in PCD patients, and vice versa, to enhance early diagnosis and treatment. It critiques the current understanding and diagnostic approaches, acknowledging limitations such as the low incidence of PCD and the complexity of testing, but highlighting the potential of genetic testing to clarify this overlap.<br /><br />Together, these articles highlight areas for further research and potential therapeutic advances. The first study suggests exploring HGF pathways for preventing fibrosis in COPD, while the second encourages heightened diagnostic efforts to understand the genetic links between PCD and CHD, with the ultimate aim of improving patient outcomes through early intervention.

Keywords

COPD

HGF

VEGF

fibrosis

gene therapy

PCD

CHD

heterotaxy

genetic testing

early diagnosis