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Slímseigjusjúkdómur (cystic fibrosis) : meingerð, greining og meðferð : yfirlitsgrein

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Authors
Brynja Jónsdóttir
Hörður Bergsteinsson
Ólafur Baldursson
Issue Date
2008-12-01

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Other Titles
Cystic fibrosis--review
Citation
Læknablaðið 2008, 94(12):831-7
Abstract
The purpose of this paper is to give a brief overview of cystic fibrosis; its pathogenesis, diagnosis, treatment and prognosis. Cystic fibrosis is an autosomal recessive disorder, which is caused by a mutation in the CFTR protein, a chloride channel in epithelial cell membranes. More than 1500 mutations are known. The incidence is 1/2.000-3.000 in nations of European origin. The CFTR mutation influences the secretion and absorption by epithelium in various organs. The consequences are different depending on the organ, but there is a global tendency for obstruction of secretory glands. The primary organs affected are the respiratory tract, pancreas, gastrointestinal tract and sweat glands. The disease is most often diagnosed during the first months of life, with a common presentation of salty tasting sweat, failure to thrive and diverse faecal problems. Possible diagnostic tools are sweat test and DNA testing. Respiratory symptoms cause most morbidity, with chronic infections and an exaggerated inflammatory response. Abnormal water and electrolyte composition leads to thicker respiratory secretions compared to that of healthy individuals. The interaction of pathogens with the epithelium causes S. aureus, and later P. aeuruginosa, to transform into a mucoid form which is much more difficult to eradicate with antibiotics, making them a significant part of the disease burden of cystic fibrosis. The main respiratory medications are antibiotics, bronchodilators, mucolytic agents and anti-inflammatory agents. 90% of cystic fibrosis patients have pancreas insufficiency which is treated with pancreas enzymes. A good nutritional status is a necessary basis for any further treatment. The prognosis of cystic fibrosis patients has improved greatly over the last few decades in parallel with increased knowledge, and the average survival is currently 37 years in the United States.
Slímseigjusjúkdómur er arfgengur víkjandi sjúkdómur sem orsakast af stökkbreytingu í CFTR geninu, en próteinafurð þess myndar jónagöng sem stýra salt- og vökvabúskap í þekjufrumum. Yfir 1500 stökkbreytingar eru þekktar. Tíðni sjúkdómsins er 1/2.000-3.000 í evrópskum þjóðum. Galli í CFTR hefur áhrif á seyti þekjuvefs og upptöku um hann í ýmsum líffærum. Áhrifin eru mismunandi eftir því hvaða líffæri á í hlut, en teppa í seyti útkirtla er sameiginlegt vandamál. Helstu líffæri sem verða fyrir áhrifum eru öndunarfæri, bris, meltingarfæri og svitakirtlar. Sjúkdómurinn greinist oftast á fyrstu mánuðum lífs og eru vanþrif, saltur sviti og hægðabreytingar vegna vanmeltingar á fitu algeng birtingarmynd. Til að greina sjúkdóminn er mögulegt að gera svitapróf, erfðaefnispróf og fleira. Öndunarfærasjúkdómur er oftast alvarlegasti hluti sjúkdómsins, með langvinnum sýkingum með ýktu bólgusvari auk þess sem lungnaslím er þykkara og öðruvísi samsett en í heilbrigðum. Samspil sýkla við þekjuvef veldur því að S.aureus og síðar P.aeuruginosa breytast í slímmyndandi form sem erfitt er að uppræta og gerir þessar tegundir því helstu skaðvaldana. Helstu lyf við öndunarfæraeinkennum eru sýklalyf, berkjuvíkkandi lyf, lyf sem auka seyti loftvega og bólgueyðandi lyf. 90% sjúklinga hafa vanstarfsemi briss sem er meðhöndlað með brisensímum. Gott næringarástand er mikilvæg forsenda allrar meðferðar. Horfur sjúklinga hafa batnað mikið undanfarna áratugi með vaxandi þekkingu og er meðalaldur nú 37 ár í Bandaríkjunum.
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