Cystic fibrosis
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| Cystic fibrosis | |
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| Lung: Gross; Cystic fibrosis Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology | |
| ICD-10 | E84 |
| ICD-9 | 277 |
| OMIM | 219700 |
| DiseasesDB | 3347 |
| MedlinePlus | 000107 |
| MeSH | D003550 |
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Cystic fibrosis Microchapters |
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Diagnosis |
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Cystic fibrosis On the Web |
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American Roentgen Ray Society Images of Cystic fibrosis |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]
Overview
Historical Perspective
Pathophysiology
Epidemiology & Demographics
Risk Factors
Screening
Causes
Differentiating Cystic fibrosis
Complications & Prognosis
Diagnosis
History and Symptoms | Physical Examination | Staging | Laboratory tests | Electrocardiogram | X Rays | CT | MRI Echocardiography or Ultrasound | Other images | Alternative diagnostics
Treatment
Medical therapy | Surgical options | Primary prevention | Secondary prevention | Financial costs | Future therapies
Diagnosis and monitoring
The role of chronic infection in lung disease
The lungs of individuals with cystic fibrosis are colonized and infected by bacteria from an early age. These bacteria, which often spread amongst individuals with CF, thrive in the altered mucus, which collects in the small airways of the lungs. This mucus encourages the development of bacterial microenvironments (biofilms) that are difficult for immune cells (and antibiotics) to penetrate. The lungs respond to repeated damage by thick secretions and chronic infections by gradually remodeling the lower airways (bronchiectasis), making infection even more difficult to eradicate.[1]
Over time, both the types of bacteria and their individual characteristics change in individuals with CF. In the initial stage, common bacteria such as Staphylococcus aureus and Hemophilus influenzae colonize and infect the lungs. Eventually, however, Pseudomonas aeruginosa (and sometimes Burkholderia cepacia) dominates. Once within the lungs, these bacteria adapt to the environment and develop resistance to commonly used antibiotics. Pseudomonas can develop special characteristics that allow the formation of large colonies, known as "mucoid" Pseudomonas and rarely seen in people that do not have CF.[1]
One way in which infection has spread is by passage between different individuals with CF.[2] In the past, people with CF often participated in summer "CF Camps" and other recreational gatherings.[3][4] Hospitals grouped patients with CF into common areas and routine equipment (such as nebulizers)[5] was not sterilized between individual patients.[6] This led to transmission of more dangerous strains of bacteria among groups of patients. As a result, individuals with CF are routinely isolated from one another in the healthcare setting and healthcare providers are encouraged to wear gowns and gloves when examining patients with CF in order to limit the spread of virulent bacterial strains.[7] Often, patients with particularly damaging bacteria will attend clinics on different days and in different buildings than those without these infections.
See also
References
- ↑ 1.0 1.1 Saiman L. Microbiology of early CF lung disease. Paediatr Respir Rev. 2004;5 Suppl A:S367-9. PMID 14980298
- ↑ Tummler B, Koopmann U, Grothues D, Weissbrodt H, Steinkamp G, von der Hardt H. Nosocomial acquisition of Pseudomonas aeruginosa by cystic fibrosis patients. J Clin Microbiol. 1991 Jun;29(6):1265–7. PMID 1907611
- ↑ Centers for Disease Control and Prevention (CDC). Pseudomonas cepacia at summer camps for persons with cystic fibrosis. MMWR Morb Mortal Wkly Rep. 1993 Jun 18;42(23):456-9. PMID 7684813
- ↑ Pegues DA, Carson LA, Tablan OC, FitzSimmons SC, Roman SB, Miller JM, Jarvis WR.Acquisition of Pseudomonas cepacia at summer camps for patients with cystic fibrosis. Summer Camp Study Group. J Pediatr. 1994 May;124(5 Pt 1):694–702. PMID 7513755
- ↑ Pankhurst CL, Philpott-Howard J. The environmental risk factors associated with medical and dental equipment in the transmission of Burkholderia (Pseudomonas) cepacia in cystic fibrosis patients. J Hosp Infect. 1996 Apr;32(4):249-55. PMID 8744509
- ↑ Jones AM, Govan JR, Doherty CJ, Dodd ME, Isalska BJ, Stanbridge TN, Webb AK. Identification of airborne dissemination of epidemic multiresistant strains of Pseudomonas aeruginosa at a CF centre during a cross infection outbreak. Thorax. 2003 Jun;58(6):525-7. PMID 12775867
- ↑ Hoiby N. Isolation and treatment of cystic fibrosis patients with lung infections caused by Pseudomonas (Burkholderia) cepacia and multiresistant Pseudomonas aeruginosa. Neth J Med. 1995 Jun;46(6):280-7. PMID 7643943
External links
- Template:Dmoz
- Cystic Fibrosis Worldwide
- Cystic Fibrosis Foundation
- Cystic fibrosis pictures (Univ Geneva, Switzerland)
- cf at NIH/UW GeneTests
- Rare Diseases Clinical Research Network
- Search GeneCards for genes envolved in Cystic Fibrosis
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