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| ==Overview== | | ==Overview== |
| '''Cystic fibrosis''' (also known as '''CF''', '''mucoviscoidosis''', or '''mucoviscidosis''') is a [[Genetic disorder|hereditary disease]] that affects the exocrine (mucus) glands of the lungs, liver, pancreas, and intestines, causing progressive disability due to multisystem failure.<ref name=CDCCF1> Cystic Fibrosis. Centers for Disease Prevention and Control (1995). https://www.cdc.gov/excite/ScienceAmbassador/ambassador_pgm/lessonplans/high_school/Am%20I%20a%20Carrier%20for%20Cystic%20Fibrosis/Cystic_Fibrosis_Fact_Sheet.pdf Accessed on July 28, 2016 </ref>
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| Thick mucus production, as well as a less competent [[immune system]], results in frequent [[pneumonia|lung infections]]. Diminished secretion of pancreatic enzymes is the main cause of [[failure to thrive|poor growth]], [[Steatorrhea|fatty diarrhea]] and deficiency in fat-soluble vitamins. Males can be [[infertility|infertile]] due to the condition [[CBAVD|congenital bilateral absence of the vas deferens]]. Often, symptoms of CF appear in infancy and childhood. [[Meconium ileus]] is a typical finding in newborn babies with CF.
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| Individuals with cystic fibrosis can be diagnosed prior to birth by [[genetic testing]]. [[Guthrie test|Newborn screening tests]] are increasingly common and effective. The diagnosis of CF may be confirmed if high levels of salt are found during a [[sweat test]], although some false positives may occur.
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| There is no cure for CF, and most individuals with cystic fibrosis die young: many in their 20s and 30s from lung failure. However, with the continuous introduction of many new treatments, the life expectancy of a person with CF is increasing to ages as high as 40 or 50. [[Lung transplantation]] is often necessary as CF worsens.
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| Cystic fibrosis is one of the most common life-shortening, childhood-onset inherited diseases. In the United States, 1 in 3900 children is born with CF<ref>[http://cfri.org/framesfaq2.htm About Cystic Fibrosis<!-- Bot generated title -->]</ref>. It is most common among Europeans and Ashkenazi Jews; one in twenty-two people of European descent are [[Genetic carrier|carriers]] of one gene for CF, making it the most common genetic disease in these populations. Ireland has the highest rate of CF carriers in the world (1 in 19).
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| CF is caused by a [[mutation]] in a [[gene]] called the [[CFTR (gene)|cystic fibrosis transmembrane conductance regulator]] (CFTR). The [[cystic fibrosis transmembrane conductance regulator|product of this gene]] is a chloride ion channel important in creating sweat, [[digestion|digestive]] juices, and [[mucus]]. Although most people without CF have two working copies (alleles) of the CFTR gene, only one is needed to prevent cystic fibrosis. CF develops when neither allele can produce a functional CFTR protein. Therefore, CF is considered an [[Autosome|autosomal]] [[recessive gene|recessive]] disease.
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| ==Historical Perspective== | | ==Historical Perspective== |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
.
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Hereditary pancreatitis from Other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications, and Prognosis
Diagnosis
Diagnostic Criteria
History and Symptoms
Physical Examination
Laboratory Findings
Electrocardiogram
X-ray
Ultrasound
CT scan
MRI
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Primary Prevention
Secondary Prevention
References
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