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Apnea of prematurity
Acute respiratory distress syndrome
Pulmonary changes at high altitude and altitude sickness
Congenital pulmonary airway malformation
Superior vena cava syndrome
Meconium aspiration syndrome
Neonatal respiratory distress syndrome
Sudden infant death syndrome
Transient tachypnea of the newborn
Alpha 1-antitrypsin deficiency
Idiopathic pulmonary fibrosis
Restrictive lung diseases
Retropharyngeal and peritonsillar abscesses
Upper respiratory tract infection
Apnea, hypoventilation and pulmonary hypertension: Pathology review
Cystic fibrosis: Pathology review
Deep vein thrombosis and pulmonary embolism: Pathology review
Lung cancer and mesothelioma: Pathology review
Obstructive lung diseases: Pathology review
Pleural effusion, pneumothorax, hemothorax and atelectasis: Pathology review
Pneumonia: Pathology review
Respiratory distress syndrome: Pathology review
Restrictive lung diseases: Pathology review
Tuberculosis: Pathology review
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Living with IPF: Colin's story
Idiopathic Pulmonary Fibrosis (IPF)
Idiopathic pulmonary fibrosis can be broken down into idiopathic which means a disease without a known cause or mechanism, pulmonary which refers to the lungs, and fibrosis which refers to excess collagen in connective tissue, or interstitial tissue between cells, usually after tissue damage.
So idiopathic pulmonary fibrosis is the ongoing repair process of having excess collagen or scar tissue in the interstitial tissue of the lung.
What triggers the repair process is unknown, but it’s a chronic process that leads to a progressive loss of lung tissue.
Normally, gas exchange happens between the alveoli which carry air and capillaries which carry blood.
The alveoli are lined by type I and type II alveolar epithelial cells, also called type I and type II pneumocytes.
Type I pneumocytes make up the majority of cells - they’re simple squamous cells that form a nearly continuous barrier between the air and underlying connective tissue.
Type II pneumocytes are studded throughout the type I - they’re shaped like cubes, have microvilli to sweep away invading particles, and secrete surfactant, an oily mixture of proteins, phospholipids, and neutral lipids which prevent the alveoli from collapsing during exhalation.
Type II pneumocytes also have the ability to divide to make more type II pneumocytes and can also divide and become type I pneumocytes as well.
Now, between the type I and type II pneumocytes and the capillaries is interstitial tissue of the lung, which has macrophages and fibroblasts.
When the alveolar lining is damaged, type I pneumocytes release transforming growth factor beta1, which gets the type II pneumocytes to stimulate fibroblasts to proliferate and develop into myofibroblasts, which are fibroblasts with some smooth muscle cell properties.
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