Bmc Med
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Idiopathic pulmonary fibrosis, the most common form of idiopathic interstitial pneumonia, is characterized by progressive, irreversible scarring of the lung parenchyma. Idiopathic pulmonary fibrosis has a poor prognosis, and there are no medical therapies available that have been shown to improve survival. It is usually sporadic, but there is evidence of familial clustering of pulmonary fibrosis, suggesting a genetic basis for this disease. More recently, studies have confirmed that specific genetic variants are associated with both familial and sporadic forms of pulmonary fibrosis. ⋯ Although there are common and rare genetic variants that have been associated with the risk of developing pulmonary fibrosis, the genotyping of patients is not a generally accepted strategy. Better understanding of the interplay between genetic risk and environmental exposure is likely needed to inform both treatment and disease prevention. Several identified disease-associated genetic variants have implications for disease progression and survival, but systematic studies of known genetic variants and their influence on therapeutic efficacy are lacking. Future investigations should focus on understanding phenotypic differences between patients carrying different risk alleles, and clinical studies should be designed to control for the influence of different genetic risk variants on patient outcomes. Inherited genetic factors play a significant role in the risk of developing pulmonary fibrosis. Future studies will be needed to characterize patient phenotypes and to understand how these genetic factors will influence clinical decision-making for both diagnosis and treatment of idiopathic pulmonary fibrosis.
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The aim of this opinion article is to understand to what extent idiopathic pulmonary fibrosis (IPF) can be considered, in its clinical and pathogenic features, similar to cancer. Indeed, IPF has common risk factors with cancer, a low survival, and, most importantly, epigenetic and genetic alterations, abnormal expression of microRNAs, cellular and molecular aberrances, and the activation of similar signalling pathways. ⋯ The pathogenic link between the two diseases may have a number of practical consequences. It may improve our understanding of IPF drawing on cancer biology knowledge. In addition, the recognition of similar pathogenic pathways may also encourage the use of cancer drugs for the treatment of IPF. Nintedanib, an inhibitor of tyrosine kinase receptors initially developed for cancer, has been recently approved for the treatment of IPF thanks to the observation that these receptors are also abnormally activated in IPF. The vision of IPF as a cancer-like disease may improve our understanding of the pathogenesis of this disease also opening new scenarios for repositioning cancer drugs for IPF. In addition, it may increase the level of awareness towards this dreadful disease at the public, political, and healthcare level.
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Patient-centredness is an accepted term and is perceived by healthcare professionals to be morally and ethically desirable. We are motivated by the belief that this approach will improve the patient-professional experience of the decision-making process and improve health outcomes. We acknowledge that patients, either as participants or as co-investigators, have positive contributions to make to research. ⋯ A conservative approach appears to have been taken to the inclusion of generic symptom or quality of life measures as PRO endpoints. Thus, the impact of new drugs on the quality of life of research participants demonstrates only minimal benefit. It is time to refocus on a patient-centred approach with regards to the co-investigator role, PRO development, and research participants.
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Idiopathic pulmonary fibrosis (IPF) is the prototype of a large and heterogeneous group of pulmonary disorders, mainly chronic and progressive, usually known as interstitial lung disease (ILD). Over the last few decades, IPF has been increasingly recognized as a major unmet medical need in respiratory medicine and has become the focus of intense research activity. ⋯ For all these reasons, missing a diagnosis of IPF is not acceptable anymore and there is a need for spreading the knowledge about IPF across various specialties of medicine globally. In this context, this article collection in BMC Medicine contributes to the ultimate goal of early identification and better management of patients with ILD, and IPF in particular, worldwide.
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Hepatocellular carcinoma (HCC), the most prevalent form of liver cancer, is difficult to diagnose and has limited treatment options with a low survival rate. Aside from a few key risk factors, such as hepatitis, high alcohol consumption, smoking, obesity, and diabetes, there is incomplete etiologic understanding of the disease and little progress in identification of early risk biomarkers. ⋯ Our results show clear metabolic alterations from early stages of HCC development with application for better etiologic understanding, prevention, and early detection of this increasingly common cancer.