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Respiratory disease
Published in Kaji Sritharan, Jonathan Rohrer, Alexandra C Rankin, Sachi Sivananthan, Essential Notes for Medical and Surgical Finals, 2021
Kaji Sritharan, Jonathan Rohrer, Alexandra C Rankin, Sachi Sivananthan
Cause often unknown: ‘idiopathic pulmonary fibrosis’ (IPF). Other interstitial pneumonias associated with connective tissue disease (rheumatoid arthritis, SLE, etc.), chronic active hepatitis, ulcerative colitis, lymphoma. IPF has poor prognosis with a mean life expectancy of three years and responds poorly to immunosuppression. The other interstitial pneumonias have variable but better prognosis than IPF.
Unusual Inherited Pulmonary Diseases Which Provide Clues to Pulmonary Physiology and Function
Published in Stephen D. Litwin, Genetic Determinants of Pulmonary Disease, 2020
Thomas Κ. C. King, Robert A. Norum
Many other diseases of known or unknown etiology are associated with fibrosis of the lungs similar to that in idiopathic pulmonary fibrosis. The pathologic feature of fibrosis in the lungs is, therefore, nonspecific. Among diseases of unknown cause, progressive systemic sclerosis [29] and rheumatoid arthritis [16] when associated with pulmonary fibrosis, are apt to be histologically indistinguishable from the idiopathic syndrome. Thus, it has recently been suggested that idiopathic pulmonary fibrosis be classified into two categories: those associated with a collagen-vascular disorder and those unassociated with any changes in other organs [30].
The Roles of Lung Biopsy in Interstital Lung Diseases in Children
Published in Lourdes R. Laraya-Cuasay, Walter T. Hughes, Interstitial Lung Diseases in Children, 2019
Many different terms have been used to classify idiopathic pulmonary fibrosis. In 1944, Hamman and Rich first described an entity of acute, rapidly progressing interstitial pneumonia in adults that usually terminated in death within months.67 This entity was first described in children by Bradley in 1956, and since then numerous pediatric cases have been reported.68 In 1974, Liebow proposed a classification of interstitial pneumonitis based on six histologic variants.69 These are often grouped under the term cryptogenic fibrosing alveolitis, which includes the classification of usual interstitial pneumonia (UIP) and desquamative interstitial pneumonia (DIP). Originally these were thought to represent different entities; however, many investigators now feel that these are merely stages of the same disease process.70 DIP refers to early idiopathic pulmonary fibrosis and UIP refers to a later stage of the same disease.70
Sorafenib for hepatocellular carcinoma: potential molecular targets and resistance mechanisms
Published in Journal of Chemotherapy, 2022
Idiopathic pulmonary fibrosis is a grave regenerative and irrevocable disease with only a few treatment options. It is accepted that the EMT is responsible for the cellular origin of fibroblast build-up in reaction to injury to lung tissues. In the pathogenesis of this disease, and EMT is induced by the transforming growth factor-b (TGF-b) signaling. EMT induced by TGF-b1 is suppressed by sorafenib in alveolar epithelial cells and concurrently decreases the collagen synthesis in fibroblasts and cell proliferation [24]. Sorafenib mediated activation and initiation of apoptosis in hepatoma cells, but spare primary hepatocytes. Partial hepatectomy (PH) and an isograft HCC transplantation model in mice were applied to confirm the mechanism. Implanted Hepa1-6 cells produce small tumour foci that show apoptosis but not the neighboring healthy hepatocytes, and apoptosis was not induced after PH. Sorafenib fleetingly repressed cell cycle progression that led to mitotic catastrophe and increased non-apoptotic liver injury during regeneration. Regenerating livers after PH were entirely sheltered from apoptosis [25].
Radiation induced apoptosis and pulmonary fibrosis: curcumin an effective intervention?
Published in International Journal of Radiation Biology, 2020
Shilpa Johnson, Sadiya B. Shaikh, Fatheema Muneesa, Barki Rashmi, Yashodhar P. Bhandary
ALI leads to the progression of PF. Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal lung disease that leads to the irreversible distortion of the lung’s architecture and is characterized by fibroblast proliferation and ECM remodeling. Although the pathogenetic mechanisms remain unclear, literature suggests that fibrosis is caused by chronic inflammatory process, resulting in the end-stage fibrotic scar in the lungs (Selman et al. 2001). Patients affected by idiopathic pulmonary fibrosis are usually between 50 and 70 years of age (Johnston et al. 1997). The estimated incidence of IPF is 7 cases per 100,000 for women and 10 cases per 100,000 for men, per year. There is an increase in the incidence, prevalence, and death rate with age (Coultas et al. 1994).
Novel management strategies for idiopathic pulmonary fibrosis
Published in Expert Review of Respiratory Medicine, 2018
Kareem Ahmad, Steven D. Nathan
Idiopathic pulmonary fibrosis is a chronic and progressively debilitating fibrotic lung disease. It has a male predominance and typically effects the elderly population. There is an association with prior smoking history, but no definitive causal relation has been determined. The disease presents with gradually increasing dyspnea, often accompanied by a dry irritating cough. On physical examination, patients classically have coarse, ‘Velcro®’-type crackles on auscultation with these tending to have a predilection for the lung bases. Pulmonary function testing typically reveals a restrictive ventilatory limitation and reduced diffusing capacity, but normal lung volumes are not uncommon, especially when there is an element of accompanying emphysema [1].