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A fibrose cística (FC), denominada também de mucoviscidose, é uma doença genética do tipo autossômica recessiva que acomete principalmente crianças e. O presente estudo examinou os desafios psicológicos de adolescentes com fibrose cística (FC) no Brasil, por meio de uma entrevista semiestruturada com. View at Google Scholar; L. F. O. Honório, N. Ludwig Neto, E. Barbosa et al., “ Avaliação da triagem neonatal para fibrose cística no estado de.

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Its frequency varies in different populations and depends on the evaluative technique.

Today more CF clinics are being recognized all over the country. Int J Psychoanal [Epub ahead of print].

Cystic fibrosis

One of the most important aspects of the current approach to CF is the great heterogeneity of the disease regarding its presentation, clinical course, and prognosis. Sweat testgenetic testing [1].

Foi investigado como os adolescentes lidam com a FC, cisticx suas necessidades, e como eles vislumbram o futuro. To deliver the medication through the skin, iontophoresis is used, whereby one electrode is fibroose onto the applied medication and an electric current is passed to a separate electrode on the skin.

In addition, the evidence is increasing that genetic modifiers besides CFTR modulate the frequency and severity of the disease. The most current theory suggests that defective ion transport leads to dehydration in the airway epithelia, thickening mucus. An exception is Finlandwhere only one in 80 people carries a CF mutation.


Parenting adolescents with cystic fibrosis: Life expectancy between 42 and 50 years developed world [4]. Only two Brazilian studies involving CF patients evaluated upper airways from a microbiological point of view. Resultant hypoproteinemia may be severe enough to cause generalized edema.

Additionally hypothiocyaniteOSCN, cannot be produced by the immune defense system. These protein defects are also targets for drugs which can sometimes restore their function. In addition to the citsica problems, people with cystic fibrosis experience more heartburn[29] intestinal blockage by intussusceptionand constipation.

Whether taking antioxidants affects outcomes is unclear. Other mutations found by Alvarez and colleagues were GX 4. The Journal of Pediatrics. Thus, CF is considered an autosomal recessive disease.

Cystic fibrosis – Wikipedia

As a consequence there will be an increasing amount of research and data to be shared. The study conducted by Sakano and coworkers [ 20 ] researched the most frequent mutations in Brazil, dividing into three groups: List of people diagnosed with cystic fibrosis. To better understand the pathogenic mechanisms of nasal polyps in CF, several studies have tried to characterize the inflammatory microenvironment, cytokines, adhesion molecules, and ionic transport.

Clubbing in the fingers of a person with cystic fibrosis.

Cystic Fibrosis: Brazilian ENT Experience

There is no known cure for cystic fibrosis. Prevalence of depression and anxiety in patients with cystic fibrosis and parent caregivers: Antibiotics by mouth such as ciprofloxacin or azithromycin are given to help prevent infection or to control ongoing infection.


J Cyst Fibros, 10 2S45— Although single lung transplantation is possible in other diseases, individuals with CF must have both lungs replaced because the remaining lung might contain bacteria that could infect the transplanted lung. The lower survival rates compared to developed countries do not seem to be directly related to those genetic differences, but raise the possible existence of mutations not yet known that lead to undiagnosed patients.

Fetal and maternal outcome”. The prognosis for cystic fibrosis has improved due to earlier diagnosis through screening and better treatment and access to health care. Pseudomonas can develop special characteristics that allow the formation of large colonies, known as “mucoid” Pseudomonaswhich are rarely seen in people who do not have CF. Other mutations produce proteins that do not use energy in the form of ATP normally, do not allow chloride, iodide, and thiocyanate to cross the membrane appropriately, [46] and degrade at a faster rate than normal.