Biofabrication of phenotypic pulmonary fibrosis assays

REPORT OF FOUR CASES OF FAMILIAL IDIOPATHIC PULMONARY FIBROSIS

A 25 year old male and his 46 year old aunt presented with shortness of breath and a dramatic response to steroids. The other two patients are sisters with more advanced disease. One of these responded partially to steroids, while the other died within 4 months of treatment. The genetic basis and pathogenesis are discussed.

Biofabrication of Tissue Scaffolds

Saffron Protection against Bleomycin-Induced Pulmonary Fibrosis in Rats

Background: Bleomycin-induced lung fibrosis has been accepted as an animal model for fibrosis in rats. The aim of this study was to evaluate the effects of saffron aqueous extract on this disorder paving the way for more investigation in treating idiopathic pulmonary fibrosis in human.  Methods: Male Wistar rats (250–300 gr) were instilled a single dose of bleomycin (5 mg/kg) via in...

Pulmonary Rehabilitation in Idiopathic Pulmonary Fibrosis: A Chance for a Multidisciplinary Treatment Approach

Background and Objectives: Idiopathic pulmonary fibrosis (IPF) is characterized by progressively worsening lung function, ventilation capacity, dyspnea, and finally reduced exercise intolerance. All of these have a significant negative impact on functional capacity and quality of life. In this study, we aim to evaluate the effects of pulmonary rehabilitation (PR) in IPF and assess the predictor...

Phenotypic heterogeneity in cystic fibrosis.

We have confirmed heterogeneity in CF using a different combination of primary clinical variables than those used in previous studies. Subgroupings of individuals with similar levels of sweat chloride were independent of the clustering based on level of pancreatic enzyme supplementation and degree of pulmonary involvement. Data from families with multiple CF children are consistent with the hyp...