Pneumothorax pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Feham Tariq, MD [2]
Overview
The exact pathogenesis of [disease name] is not fully understood.
OR
It is thought that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].
OR
[Pathogen name] is usually transmitted via the [transmission route] route to the human host.
OR
Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
OR
[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
OR
The progression to [disease name] usually involves the [molecular pathway].
OR
The pathophysiology of [disease/malignancy] depends on the histological subtype.
Pathophysiology
Anatomy and physiology of the thoracic cavity
- Thoracic cavity is defined as the space inside the chest that contains the heart,lungs, and several major blood vessels.
- On either side of the cavity, a pleural membrane covers the outside surface of the lung (visceral pleura) and also lines the inside of the chest wall (parietal pleura).
- The two layers are separated by a small amount of lubricating serous fluid known as the pleural fluid.
- The lungs are fully inflated within the cavity as the pressure inside the airways is higher than the pressure inside the pleural space.
- The inhaled air does not enter the pleural space as there is no natural connections between them as well as the pressure of gases in the blood stream is too low for them to be forced into the pleural space.
- Air can enter the pleural space through the following mechanisms:
- Damage to the pleura or the chest wall
- Damage to the lung itself
- Microorganisms in the pleural space
Pathogenesis
- The pathophysiology of pneumothorax depends on the underlying disease causing it.
Primary spontaneous pneumothorax
- The most common underlying pathology of primary sponataneous pneumothorax is an apical subpleural bleb (small air-filled lesions under the pleural surface).[1]
- In additon, smoking causes inflammation and obstruction of the small airways, which is responsible for the increased risk of primary sponataneous pneumothorax in smokers.
Genetics
Genetic assocaiation
The genetic association of primary sponatneous pneumothorax is as follows:[2][3][4][5][6][7]
- Primary spontaneous pneumothorax can result as a mutation in the FLCN (folliculin) gene.
- This gene codes for a protein called folliculin.
- It is produced by the cells lining the alveoli of the lung.
- Folliculin is found in the connective tissue cells that allow the lungs to contract and expand while breathing.
- It plays a role in repairing the lung tissue after damage.
- Nonsense mutation in the folliculin gene results in isolated familial sponataneous primary pneumothorax.[8]
- Altered folliculin protein can trigger the inflammatory process within the lung tissue that can alter and damage the tissue, resulting in blebs formation.
Associated Conditions
Gross Pathology
- On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
Microscopic Pathology
- On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
References
- ↑ Yazkan R, Han S (2010). "Pathophysiology, clinical evaluation and treatment options of spontaneous pneumothorax". Tuberk Toraks. 58 (3): 334–43. PMID 21038147.
- ↑ Chiu HT, Garcia CK (2006). "Familial spontaneous pneumothorax". Curr Opin Pulm Med. 12 (4): 268–72. doi:10.1097/01.mcp.0000230630.73139.f0. PMID 16825879.
- ↑ Bintcliffe O, Maskell N (2014). "Spontaneous pneumothorax". BMJ. 348: g2928. doi:10.1136/bmj.g2928. PMID 24812003.
- ↑ Wakai A (2008). "Spontaneous pneumothorax". BMJ Clin Evid. 2008. PMC 2907964. PMID 19450320.
- ↑ Wakai AP (2011). "Spontaneous pneumothorax". BMJ Clin Evid. 2011. PMC 3275306. PMID 21477390.
- ↑ Andrivet P, Djedaini K, Teboul JL, Brochard L, Dreyfuss D (1995). "Spontaneous pneumothorax. Comparison of thoracic drainage vs immediate or delayed needle aspiration". Chest. 108 (2): 335–9. PMID 7634863.
- ↑ Lippert HL, Lund O, Blegvad S, Larsen HV (1991). "Independent risk factors for cumulative recurrence rate after first spontaneous pneumothorax". Eur Respir J. 4 (3): 324–31. PMID 1864347.
- ↑ Graham RB, Nolasco M, Peterlin B, Garcia CK (2005). "Nonsense mutations in folliculin presenting as isolated familial spontaneous pneumothorax in adults". Am J Respir Crit Care Med. 172 (1): 39–44. doi:10.1164/rccm.200501-143OC. PMID 15805188.