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| | __NOTOC__ |
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| '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' | | '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' |
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| {{Infobox_Disease |
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| | Name = Cystic fibrosis
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| | Image = Cystic fibrosis.jpg
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| | Caption = Lung: Gross; Cystic fibrosis <br> <small> [http://www.peir.net Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology] </small>
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| | DiseasesDB = 3347
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| | ICD10 = {{ICD10|E|84||e|70}}
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| | ICD9 = {{ICD9|277}}
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| | ICDO =
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| | OMIM = 219700
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| | MedlinePlus = 000107
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| | eMedicineSubj =
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| | eMedicineTopic =
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| | MeshID = D003550
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| }}
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| {{Cystic fibrosis}} | | {{Cystic fibrosis}} |
| {{CMG}}; '''Associate Editor(s)-In-Chief:''' {{CZ}} | | {{CMG}}; '''Associate Editor(s)-In-Chief:''' {{SHH}} |
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| | {{SK}} CF; mucoviscoidosis; mucoviscidosis |
| ==[[Cystic fibrosis overview|Overview]]== | | ==[[Cystic fibrosis overview|Overview]]== |
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| ==[[Cystic fibrosis historical perspective|Historical Perspective]]== | | ==[[Cystic fibrosis historical perspective|Historical Perspective]]== |
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| | ==[[Cystic fibrosis classification|Classification]]== |
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| ==[[Cystic fibrosis pathophysiology|Pathophysiology]]== | | ==[[Cystic fibrosis pathophysiology|Pathophysiology]]== |
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| ==[[Cystic fibrosis epidemiology and demographics|Epidemiology & Demographics]]== | | ==[[Cystic fibrosis causes|Causes]]== |
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| | ==[[Cystic fibrosis differential diagnosis|Differentiating Cystic fibrosis from other Diseases]]== |
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| | ==[[Cystic fibrosis epidemiology and demographics|Epidemiology and Demographics]]== |
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| ==[[Cystic fibrosis risk factors|Risk Factors]]== | | ==[[Cystic fibrosis risk factors|Risk Factors]]== |
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| ==[[Cystic fibrosis screening|Screening]]== | | ==[[Cystic fibrosis screening|Screening]]== |
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| ==[[Cystic fibrosis causes|Causes]]==
| | ==[[Cystic fibrosis natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
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| ==[[Cystic fibrosis differential diagnosis|Differentiating Cystic fibrosis]]==
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| ==[[Cystic fibrosis natural history|Complications & Prognosis]]== | |
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| ==Diagnosis== | | ==Diagnosis== |
| [[Cystic fibrosis history and symptoms|History and Symptoms]] | [[Cystic fibrosis physical examination|Physical Examination]] | [[Cystic fibrosis staging|Staging]] | [[Cystic fibrosis laboratory tests|Laboratory tests]] | [[Cystic fibrosis electrocardiogram|Electrocardiogram]] | [[Cystic fibrosis x ray|X Rays]] | [[Cystic fibrosis CT|CT]] | [[Cystic fibrosis MRI|MRI]] [[Cystic fibrosis echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Cystic fibrosis other imaging findings|Other images]] | [[Cystic fibrosis other diagnostic studies|Alternative diagnostics]] | | [[Cystic fibrosis diagnostic study of choice|Diagnostic Study of Choice]] | [[Cystic fibrosis history and symptoms|History and Symptoms]] | [[Cystic fibrosis physical examination|Physical Examination]] | [[Cystic fibrosis laboratory findings|Laboratory Findings]] | [[Cystic fibrosis electrocardiogram|Electrocardiogram]] | [[Cystic fibrosis chest x ray|Chest X Ray]] | [[Cystic fibrosis echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Cystic fibrosis CT|CT]] | [[Cystic fibrosis MRI|MRI]] | [[Cystic fibrosis other imaging findings|Other Imaging Findings]] | [[Cystic fibrosis other diagnostic studies|Other Diagnostic Studies]] |
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| ==Treatment== | | ==Treatment== |
| [[Cystic fibrosis medical therapy|Medical therapy]] | [[Cystic fibrosis surgery|Surgical options]] | [[Cystic fibrosis primary prevention|Primary prevention]] | [[Cystic fibrosis secondary prevention|Secondary prevention]] | [[Cystic fibrosis cost-effectiveness of therapy|Financial costs]] | [[Cystic fibrosis future or investigational therapies|Future therapies]] | | [[Cystic fibrosis medical therapy|Medical Therapy]] | [[Cystic fibrosis surgery|Surgery]] | [[Cystic fibrosis primary prevention|Primary Prevention]] | [[Cystic fibrosis secondary prevention|Secondary Prevention]] | [[Cystic fibrosis cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Cystic fibrosis future or investigational therapies|Future or Investigational Therapies]] |
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| ==Diagnosis and monitoring==
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| ===The role of chronic infection in lung disease===
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| 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 ([[biofilm]]s) 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.<ref name="Saiman">Saiman L. ''Microbiology of early CF lung disease.'' Paediatr Respir Rev. 2004;5 Suppl A:S367-9. PMID 14980298</ref>
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| 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 complex|Burkholderia cepacia]]'') dominates. Once within the lungs, these bacteria adapt to the environment and develop [[antibiotic resistance|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.<ref name="Saiman" />
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| One way in which infection has spread is by passage between different individuals with CF.<ref>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</ref> In the past, people with CF often participated in summer "CF Camps" and other recreational gatherings.<ref>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</ref><ref>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</ref> Hospitals grouped patients with CF into common areas and routine equipment (such as [[nebulizer]]s)<ref>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</ref> was not sterilized between individual patients.<ref>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</ref> 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.<ref>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</ref> Often, patients with particularly damaging bacteria will attend clinics on different days and in different buildings than those without these infections.
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| ==See also==
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| *[[List of cystic fibrosis organizations]]
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| *[[Dor Yeshorim]]
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| ==References==
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| {{Reflist|2}}
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| ==External links== | | ==Case Studies== |
| <!-- please don't unilaterally delete links without discussion -->
| | :[[Cystic fibrosis case study one|Case #1]] [[Category:Gastroenterology]] [[Category:Pediatrics]] [[Category:Pulmonology]] |
| *{{dmoz|Health/Conditions_and_Diseases/Genetic_Disorders/Cystic_Fibrosis/}}
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| *[http://www.cfww.org/ Cystic Fibrosis Worldwide]
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| *[http://www.cff.org Cystic Fibrosis Foundation]
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| *[http://www.gfmer.ch/genetic_diseases_v2/gendis_detail_list.php?cat3=90 Cystic fibrosis pictures (Univ Geneva, Switzerland)]
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| * {{GeneTests|cf}}
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| *[http://rarediseasesnetwork.epi.usf.edu/gdmcc/learnmore/index.htm#cf Rare Diseases Clinical Research Network]
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| *[http://genecards.org/cgi-bin/cardsearch.pl?search=cystic+fibrosis&search_type=kwd&mini=yes&speed=fast#results Search GeneCards for genes envolved in Cystic Fibrosis]
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| {{SIB}}
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| {{Other metabolic pathology}}
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| {{Link FA|es}}
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| [[ar:تليف كيسي]]
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| [[ca:Fibrosi quística]]
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| [[cs:Cystická fibróza]]
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| [[cy:Ffibrosis systig]]
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| [[da:Cystisk fibrose]]
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| [[de:Mukoviszidose]]
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| [[et:Tsüstiline fibroos]]
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| [[es:Fibrosis quística]]
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| [[eu:Fibrosi kistiko]]
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| [[fa:فیبروز کیستیک]]
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| [[fr:Mucoviscidose]]
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| [[it:Fibrosi cistica]]
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| [[he:סיסטיק פיברוזיס]]
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| [[hu:Cisztás fibrózis]]
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| [[nl:Taaislijmziekte]]
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| [[ja:嚢胞性線維症]]
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| [[no:Cystisk fibrose]]
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| [[nn:Cystisk fibrose]]
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| [[pl:Mukowiscydoza]]
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| [[pt:Fibrose cística]] | |
| [[ru:Муковисцидоз]] | |
| [[sq:Fibroza kistike]]
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| [[simple:Cystic fibrosis]]
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| [[sk:Cystická fibróza]]
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| [[sr:Цистична фиброза]]
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| [[fi:Kystinen fibroosi]]
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| [[sv:Cystisk fibros]]
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| [[uk:Кістозний фіброз]]
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| [[zh:囊腫性纖維化]] | |
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