Cystic fibrosis medical therapy: Difference between revisions
Jump to navigation
Jump to search
| Line 5: | Line 5: | ||
==Overview== | ==Overview== | ||
Medical treatments for patients with cystic fibrosis has targeted following consequences of the defect such as GI and pulmonary [[mucus]] plugging and infection. Treatment include [[Mucolytic agent|mucolytic agents]] ([[dornase alfa]], N-acetyl-L-cysteine), airway surface [[rehydration]] (hypertonic [[Saline (medicine)|saline]], [[Osmosis|osmotic]] agents), [[Antimicrobial|anti-infective agents]] (for [[prophylaxis]], eradication of early infection and suppression of chronic infection), [[Anti inflammatory medications|anti-inflammatory agents]] ([[Non-steroidal anti-inflammatory drug|NSAIDs]], inhaled [[Corticosteroid|corticosteroids]], [[Leukotriene B4 receptor|LTB4 receptor]] antagonists and [[Azithromycin]]) and potentiators of [[Cystic fibrosis transmembrane conductance regulator|CFTR protein]] defect. | Medical treatments for patients with cystic fibrosis has targeted following consequences of the defect such as GI and pulmonary [[mucus]] plugging and infection. Treatment include [[Mucolytic agent|mucolytic agents]] ([[dornase alfa]], [[Acetylcysteine|N-acetyl-L-cysteine]]), [[airway]] surface [[rehydration]] ([[hypertonic]] [[Saline (medicine)|saline]], [[Osmosis|osmotic]] agents), [[Antimicrobial|anti-infective agents]] (for [[prophylaxis]], eradication of early [[infection]] and suppression of chronic [[infection]]), [[Anti inflammatory medications|anti-inflammatory agents]] ([[Non-steroidal anti-inflammatory drug|NSAIDs]], inhaled [[Corticosteroid|corticosteroids]], [[Leukotriene B4 receptor|LTB4 receptor]] [[Receptor antagonist|antagonists]] and [[Azithromycin]]) and potentiators of [[Cystic fibrosis transmembrane conductance regulator|CFTR protein]] defect. | ||
==Medical Therapy== | ==Medical Therapy== | ||
* Treatment for cystic fibrosis has targeted following consequences of the defect such as GI and pulmonary [[mucus]] plugging and [[infection]]. | * Treatment for cystic fibrosis has targeted following consequences of the defect such as [[Gastrointestinal tract|GI]] and pulmonary [[mucus]] plugging and [[infection]]. | ||
* Medical treatments for patients with cystic fibrosis are include:<ref name="pmid19393104">{{cite journal |vauthors=Ratjen FA |title=Cystic fibrosis: pathogenesis and future treatment strategies |journal=Respir Care |volume=54 |issue=5 |pages=595–605 |year=2009 |pmid=19393104 |doi= |url=}}</ref><ref name="pmid27347364">{{cite journal |vauthors=Edmondson C, Davies JC |title=Current and future treatment options for cystic fibrosis lung disease: latest evidence and clinical implications |journal=Ther Adv Chronic Dis |volume=7 |issue=3 |pages=170–83 |year=2016 |pmid=27347364 |pmc=4907071 |doi=10.1177/2040622316641352 |url=}}</ref><ref name="pmid22093951">{{cite journal |vauthors=Konstan MW, Ratjen F |title=Effect of dornase alfa on inflammation and lung function: potential role in the early treatment of cystic fibrosis |journal=J. Cyst. Fibros. |volume=11 |issue=2 |pages=78–83 |year=2012 |pmid=22093951 |pmc=4090757 |doi=10.1016/j.jcf.2011.10.003 |url=}}</ref> | * Medical treatments for patients with cystic fibrosis are include:<ref name="pmid19393104">{{cite journal |vauthors=Ratjen FA |title=Cystic fibrosis: pathogenesis and future treatment strategies |journal=Respir Care |volume=54 |issue=5 |pages=595–605 |year=2009 |pmid=19393104 |doi= |url=}}</ref><ref name="pmid27347364">{{cite journal |vauthors=Edmondson C, Davies JC |title=Current and future treatment options for cystic fibrosis lung disease: latest evidence and clinical implications |journal=Ther Adv Chronic Dis |volume=7 |issue=3 |pages=170–83 |year=2016 |pmid=27347364 |pmc=4907071 |doi=10.1177/2040622316641352 |url=}}</ref><ref name="pmid22093951">{{cite journal |vauthors=Konstan MW, Ratjen F |title=Effect of dornase alfa on inflammation and lung function: potential role in the early treatment of cystic fibrosis |journal=J. Cyst. Fibros. |volume=11 |issue=2 |pages=78–83 |year=2012 |pmid=22093951 |pmc=4090757 |doi=10.1016/j.jcf.2011.10.003 |url=}}</ref> | ||
{| class="wikitable" | {| class="wikitable" | ||
| Line 19: | Line 19: | ||
| rowspan="2" |[[Mucolytic agent|Mucolytic agents]] | | rowspan="2" |[[Mucolytic agent|Mucolytic agents]] | ||
|[[Dornase alfa]] | |[[Dornase alfa]] | ||
|Cleave the extracellular [[DNA]] and aid airway clearance | |Cleave the [[extracellular]] [[DNA]] and aid [[airway]] clearance | ||
|- | |- | ||
|N-acetyl-L-cysteine | |[[Acetylcysteine|N-acetyl-L-cysteine]] | ||
|Also increase levels of the intracellular [[antioxidant]] [[glutathione]] (GSH) that protect against the [[neutrophil]]-driven tissue damage | |Also increase levels of the [[intracellular]] [[antioxidant]] [[glutathione]] (GSH) that protect against the [[neutrophil]]-driven tissue damage | ||
|- | |- | ||
| rowspan="3" |Airway surface [[rehydration]] | | rowspan="3" |[[Airway]] surface [[rehydration]] | ||
|Hypertonic [[Saline (medicine)|saline]] | |[[Hypertonic]] [[Saline (medicine)|saline]] | ||
|As it may cause [[bronchoconstriction]], it is commonly used with an [[bronchodilator]] | |As it may cause [[bronchoconstriction]], it is commonly used with an [[bronchodilator]] | ||
|- | |- | ||
|[[Osmosis|Osmotic]] agents | |[[Osmosis|Osmotic]] agents | ||
|[[Mannitol]] is a nonabsorbable sugar [[alcohol]] which provides an [[Osmosis|osmotic]] gradient on the airway surface | |[[Mannitol]] is a nonabsorbable [[sugar]] [[alcohol]] which provides an [[Osmosis|osmotic]] gradient on the [[airway]] surface | ||
|- | |- | ||
|Correction of ion transport | |Correction of [[ion]] transport | ||
| | | | ||
|- | |- | ||
| Line 38: | Line 38: | ||
|Anti-[[Staphylococcus|staphylococcal]] [[Antibiotic|antibiotics]] (such as [[flucloxacillin]]) until ~3 years of age is recommended to reduce the incidence of [[Staphylococcus aureus|methicillin-susceptible ''S. aureus'']] ([[Staphylococcus aureus|MSSA]]) | |Anti-[[Staphylococcus|staphylococcal]] [[Antibiotic|antibiotics]] (such as [[flucloxacillin]]) until ~3 years of age is recommended to reduce the incidence of [[Staphylococcus aureus|methicillin-susceptible ''S. aureus'']] ([[Staphylococcus aureus|MSSA]]) | ||
|- | |- | ||
|Eradication of early infection | |Eradication of early [[infection]] | ||
|If [[Pseudomonas aeruginosa|P. aeruginosa]] not detected and treated aggressively, this [[gram-negative]], [[Opportunistic infection|opportunistic bacterium]] will become chronic. | |If [[Pseudomonas aeruginosa|P. aeruginosa]] not detected and treated aggressively, this [[gram-negative]], [[Opportunistic infection|opportunistic bacterium]] will become chronic. | ||
* North America: inhaled [[tobramycin]] | * North America: inhaled [[tobramycin]] | ||
* Europe: multicentre trial is currently assessing whether IV or oral [[Antibiotic|antibiotics]] are superior + nebulized [[colistin]] | * Europe: multicentre trial is currently assessing whether IV or oral [[Antibiotic|antibiotics]] are superior + nebulized [[colistin]] | ||
|- | |- | ||
|Suppression of chronic infection | |Suppression of chronic [[infection]] | ||
|The most commonly used nebulized [[Antibiotic|antibiotics]] against [[Pseudomonas aeruginosa|P. aeruginosa]] are [[tobramycin]], [[colistin]] and [[aztreonam]]. | |The most commonly used nebulized [[Antibiotic|antibiotics]] against [[Pseudomonas aeruginosa|P. aeruginosa]] are [[tobramycin]], [[colistin]] and [[aztreonam]]. | ||
|- | |- | ||
Revision as of 18:49, 9 March 2018
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shaghayegh Habibi, M.D.[2]
|
Cystic fibrosis Microchapters |
|
Diagnosis |
|---|
|
Treatment |
|
Case Studies |
|
Cystic fibrosis medical therapy On the Web |
|
American Roentgen Ray Society Images of Cystic fibrosis medical therapy |
|
Risk calculators and risk factors for Cystic fibrosis medical therapy |
Overview
Medical treatments for patients with cystic fibrosis has targeted following consequences of the defect such as GI and pulmonary mucus plugging and infection. Treatment include mucolytic agents (dornase alfa, N-acetyl-L-cysteine), airway surface rehydration (hypertonic saline, osmotic agents), anti-infective agents (for prophylaxis, eradication of early infection and suppression of chronic infection), anti-inflammatory agents (NSAIDs, inhaled corticosteroids, LTB4 receptor antagonists and Azithromycin) and potentiators of CFTR protein defect.
Medical Therapy
- Treatment for cystic fibrosis has targeted following consequences of the defect such as GI and pulmonary mucus plugging and infection.
- Medical treatments for patients with cystic fibrosis are include:[1][2][3]
| Medical treatment in patients with Cystic fibrosis | ||
|---|---|---|
| Category | Approaches | Explanation |
| Mucolytic agents | Dornase alfa | Cleave the extracellular DNA and aid airway clearance |
| N-acetyl-L-cysteine | Also increase levels of the intracellular antioxidant glutathione (GSH) that protect against the neutrophil-driven tissue damage | |
| Airway surface rehydration | Hypertonic saline | As it may cause bronchoconstriction, it is commonly used with an bronchodilator |
| Osmotic agents | Mannitol is a nonabsorbable sugar alcohol which provides an osmotic gradient on the airway surface | |
| Correction of ion transport | ||
| Anti-infective agents | Prophylaxis | Anti-staphylococcal antibiotics (such as flucloxacillin) until ~3 years of age is recommended to reduce the incidence of methicillin-susceptible S. aureus (MSSA) |
| Eradication of early infection | If P. aeruginosa not detected and treated aggressively, this gram-negative, opportunistic bacterium will become chronic.
| |
| Suppression of chronic infection | The most commonly used nebulized antibiotics against P. aeruginosa are tobramycin, colistin and aztreonam. | |
| Acute exacerbations | Pulmonary exacerbations are treated with oral or IV antibiotics depending on severity. | |
| Anti-Inflammatory agents | Nonsteroidal anti-inflammatory agents (NSAIDs) | Ibuprofen showed some benefit in young patients with mild disease in high doses. |
| Inhaled corticosteroids | ||
| LTB4 receptor antagonists | Leukotriene B4 (LTB4) is produced by macrophages and PMNs in response to infection and plays a significant role in inflammatory response. | |
| Azithromycin | ||
| CFTR protein defect | Potentiators | Enhance the activity of the CFTR channel if it is correctly located.
The most significant advance in the treatment of CF over the last few years has been the development of Ivacaftor (Ivacaftor increases the time the CFTR channel is open) |
| Correctors and combination therapy | lumicaftor/ivacaftor | |
References
- ↑ Ratjen FA (2009). "Cystic fibrosis: pathogenesis and future treatment strategies". Respir Care. 54 (5): 595–605. PMID 19393104.
- ↑ Edmondson C, Davies JC (2016). "Current and future treatment options for cystic fibrosis lung disease: latest evidence and clinical implications". Ther Adv Chronic Dis. 7 (3): 170–83. doi:10.1177/2040622316641352. PMC 4907071. PMID 27347364.
- ↑ Konstan MW, Ratjen F (2012). "Effect of dornase alfa on inflammation and lung function: potential role in the early treatment of cystic fibrosis". J. Cyst. Fibros. 11 (2): 78–83. doi:10.1016/j.jcf.2011.10.003. PMC 4090757. PMID 22093951.