{"id":910,"date":"2012-07-27T13:11:08","date_gmt":"2012-07-27T11:11:08","guid":{"rendered":"http:\/\/slanedeti.sk\/cms\/cysticka-fibroza-klinicke-prejavy-a-liecba-2\/201207"},"modified":"2014-04-07T16:16:13","modified_gmt":"2014-04-07T15:16:13","slug":"cysticka-fibroza-klinicke-prejavy-a-liecba","status":"publish","type":"post","link":"https:\/\/slanedeti.sk\/cms\/cysticka-fibroza-klinicke-prejavy-a-liecba\/910","title":{"rendered":"Cystick\u00e1 fibr\u00f3za: Klinick\u00e9 prejavy a lie\u010dba"},"content":{"rendered":"

Po\u0161kodenie respira\u010dn\u00e9ho syst\u00e9mu je hlavnou pr\u00ed\u010dinou morbidity a mortality u pacientov s cystickou fibr\u00f3zou (CF). Klinick\u00e9 prejavy ochorenia s\u00fa ve\u013emi pestr\u00e9. Z h\u013eadiska rozmanitosti a intenzity zah\u0155\u0148aj\u00fa \u0161irok\u00fa \u0161k\u00e1lu obrazov, ktor\u00e9 m\u00f4\u017eu by\u0165 u ka\u017ed\u00e9ho chor\u00e9ho osobit\u00e9 a od ktor\u00fdch z\u00e1vis\u00ed aj individu\u00e1lny lie\u010debn\u00fd pr\u00edstup.<\/p>\n

Horn\u00e9 d\u00fdchacie cesty<\/strong><\/h4>\n

Ochorenie postihuje nielen doln\u00e9, ale spravidla aj horn\u00e9 d\u00fdchacie cesty. Ve\u013emi \u010dast\u00e1 je chronick\u00e1 rinit\u00edda a postihnutie pr\u00ednosov\u00fdch dut\u00edn vo forme ak\u00fatneho alebo chronick\u00e9ho z\u00e1palu (sinusit\u00edda). Niektor\u00ed pacienti trpia tie\u017e v\u00fdskytom nosov\u00fdch polypov, ktor\u00e9 si pr\u00edle\u017eitostne vy\u017eaduj\u00fa prevent\u00edvne chirurgick\u00e9 odstr\u00e1nenie s cie\u013eom prevencie nepriechodnosti nosa.<\/p>\n

Doln\u00e9 d\u00fdchacie cesty –\u00a0 bronchopulmon\u00e1lne ochorenia
\n<\/strong><\/h4>\n

V d\u00fdchac\u00edch cest\u00e1ch pacientov s CF nemus\u00ed prebieha\u0165 chronick\u00fd z\u00e1palov\u00fd proces ihne\u010f po naroden\u00ed. V r\u00e1mci mut\u00e1cii CFTR, pr\u00edpadne in\u00fdch modifikuj\u00facich g\u00e9nov a vplyvov sa postupne proces rozv\u00edja.<\/p>\n

V predisponovanom prostred\u00ed m\u00f4\u017ee najsk\u00f4r vznikn\u00fa\u0165 prim\u00e1rna ak\u00fatna infekcia, ktor\u00e1 spust\u00ed kask\u00e1du inflama\u010dn\u00fdch procesov a u\u013eah\u010d\u00ed vznik \u010fal\u0161\u00edch infekci\u00ed, pr\u00edpadne koloniz\u00e1cie niektor\u00fdm z patog\u00e9nov.<\/p>\n

Aj v\u00edrusov\u00e1 infekcia zvy\u0161uje n\u00e1chylnos\u0165 k vzniku z\u00e1va\u017enej\u0161ieho bakteri\u00e1lneho z\u00e1palu, \u010di k inici\u00e1lnej infekcii alebo k exacerb\u00e1cii. Doch\u00e1dza ku kumul\u00e1cii z\u00e1palov\u00fdch elementov a zv\u00fd\u0161enej tvorbe z\u00e1palov\u00fdch cytok\u00ednov, ktor\u00e9 v\u0161ak nie s\u00fa schopn\u00e9 dostato\u010dne efekt\u00edvne likvidova\u0165 a eliminova\u0165 vyvol\u00e1vaj\u00face
\nmikroorganizmy.<\/p>\n

Mikroorganizmy doln\u00fdch d\u00fdchac\u00edch ciest pri CF
\n<\/strong><\/h4>\n

Staphylococcus aureus<\/strong> – t\u00e1to bakt\u00e9ria sa naj\u010dastej\u0161ie v sp\u00fate pacientov s CF dok\u00e1\u017ee ako prv\u00e1. Zvy\u010dajne sa jedn\u00e1 o bakt\u00e9riu s relat\u00edvne dobrou citlivos\u0165ou na antibiotik\u00e1, ale m\u00f4\u017ee sa objavi\u0165 aj nozokomi\u00e1lny kme\u0148 MRSA (meticil\u00edn rezistentn\u00fd S. aureus), ktor\u00fd predstavuje z\u00e1va\u017en\u00fd probl\u00e9m a jeho v\u00fdskyt si vy\u017eaduje \u0161peci\u00e1lne epidemiologick\u00e9 a hygienick\u00e9 opatrenia.<\/p>\n

\"Staphylococcus<\/div>\n

Haemophillus influenzae<\/strong> sa v sp\u00fate objavuje ojedinele a niekedy b\u00fdva pr\u00ed\u010dinou ak\u00fatnych exacerb\u00e1cii .<\/p>\n

\"Haemophillus<\/div>\n

Pseudomonas aeruginosa<\/strong> je naj\u010dastej\u0161ia bakt\u00e9ria izolovan\u00e1 zo sp\u00fata det\u00ed aj dospel\u00fdch pacientov CF. Pri zisten\u00ed pr\u00edtomnosti tejto bakt\u00e9rie je nevyhnutn\u00e9 pok\u00fasi\u0165 sa o eradik\u00e1ciu, preto\u017ee chronick\u00e1
\nP. aeruginosa<\/strong> infekcia je spojen\u00e1 s hor\u0161ou progn\u00f3zou, hor\u0161\u00edm klinick\u00fdm stavom, ni\u017e\u0161\u00edmi funk\u010dn\u00fdmi parametrami p\u013e\u00fac, hmotnos\u0165ou a \u010dastej\u0161\u00edmi hospitaliz\u00e1ciami.<\/p>\n

Burkholderia cepacia complex<\/strong> (Bcc) vystupuje st\u00e1le viac do popredia. Nie je z\u00e1va\u017en\u00e1 svojou frekvenciou, ale poly- a\u017e multirezistenciou na antibiotik\u00e1. Niektor\u00e9 kmene Bcc s\u00fa sp\u00e1jan\u00e9 s vy\u0161\u0161\u00edm v\u00fdskytom nepriazniv\u00fdch priebehov ochorenia, r\u00fdchlou progresivitou poklesu p\u013e\u00facnych funkcii, av\u0161ak in\u00ed pacienti s t\u00fdm ist\u00fdm kme\u0148om m\u00f4\u017eu by\u0165 dlhodobo stabilizovan\u00ed. \u0160\u00edrenie sa uskuto\u010d\u0148uje najm\u00e4 prenosom z pacienta na pacienta pri hospitaliz\u00e1cii a osobn\u00fdch kontaktoch. Izol\u00e1cia sa ukazuje by\u0165 najefekt\u00edvnej\u0161\u00edm sp\u00f4sobom, ktor\u00fd zabra\u0148uje riziku prenosu.<\/p>\n

\"Burkholderia<\/div>\n

Stenotrophomonas maltophilia<\/strong> sa u pacientov s CF objavuje zv\u00e4\u010d\u0161a ojedinele a predpoklad\u00e1 sa, \u017ee hlavnou pr\u00ed\u010dinou v\u00fdskytu je selek\u010dn\u00fd tlak antibiot\u00edk. Vyskytuje sa najm\u00e4 vo vlhkom prostred\u00ed a m\u00f4\u017ee kolonizova\u0165 napr. inhala\u010dn\u00e9 pom\u00f4cky.<\/p>\n

V\u00edrusy<\/strong>, ako napr. v\u00edrus chr\u00edpky, RS v\u00edrus a mnoh\u00e9 \u010fal\u0161ie s\u00fa v\u00fdznamn\u00e9 najm\u00e4 ako inici\u00e1tory v\u00e1\u017enej\u0161\u00edch bakteri\u00e1lnych exacerb\u00e1ci\u00ed. Oslaben\u00edm lok\u00e1lnej aj celkovej imunity u\u013eah\u010duj\u00fa vznik koloniz\u00e1cie spom\u00ednan\u00fdmi bakt\u00e9riami. Opakovan\u00e9 v\u00edrusov\u00e9 infekcie s\u00fa spojen\u00e9 so zhor\u0161ovan\u00edm stavu bronchopulmon\u00e1ln\u00e9ho syst\u00e9mu. Z tohto d\u00f4vodu s\u00fa d\u00f4le\u017eit\u00e9 najm\u00e4 prevent\u00edvne epidemiologick\u00e9 opatrenia, vr\u00e1tane dostupn\u00fdch o\u010dkovan\u00ed.<\/p>\n

Kvasinkov\u00e9 mikroorganizmy<\/strong> sa z mykotick\u00fdch infekcii u pacientov s CF nach\u00e1dzaj\u00fa naj\u010dastej\u0161ie. Pozit\u00edvna kultiv\u00e1cia je najm\u00e4 u pacientov pou\u017e\u00edvaj\u00facich \u0161irokospektr\u00e1lne antibiotik\u00e1. Pre pos\u00fadenie klinickej z\u00e1va\u017enosti kultiva\u010dn\u00e9ho n\u00e1lezu je d\u00f4le\u017eit\u00e1 aj kvantifik\u00e1cia kultiva\u010dn\u00e9ho n\u00e1lezu a pr\u00edpadne mikroskopick\u00fd n\u00e1lez pseudoh\u00fdf (vl\u00e1knit\u00fdch foriem), ktor\u00e9 maj\u00fa vy\u0161\u0161\u00ed sklon k invazivite.<\/p>\n

Vl\u00e1knit\u00e9 huby rodu Aspergilus<\/strong> sa \u010dasto vyskytuj\u00fa v d\u00fdchac\u00edch cest\u00e1ch pacientov s CF, najm\u00e4 Aspergillus fumigatus, ktor\u00fd patr\u00ed k najroz\u0161\u00edrenej\u0161\u00edm v \u017eivotnom prostred\u00ed. Aspergilus m\u00f4\u017ee p\u00f4sobi\u0165 ako alerg\u00e9n a vyvola\u0165 hypersenzit\u00edvnu reakciu, zn\u00e1mu ako alergick\u00e1 bronchopulmon\u00e1lna aspergil\u00f3za (ABPA)<\/p>\n

\"Aspergilus\"<\/div>\n

Klinick\u00e9 prejavy
\n<\/strong><\/h4>\n

Ka\u0161e\u013e je zvy\u010dajne prv\u00fdm a nesporne naj\u010dastej\u0161\u00edm prejavom ochorenia. Spo\u010diatku b\u00fdva ob\u010dasn\u00fd, pripom\u00ednaj\u00faci ak\u00fatny respira\u010dn\u00fd infekt, pri\u010dom najv\u00e4\u010d\u0161iu intenzitu m\u00e1va r\u00e1no po prebuden\u00ed. Ka\u0161e\u013e \u010dasto sprev\u00e1dza sipenie a piskoty, osobitne u det\u00ed. Obdobia sprev\u00e1dzan\u00e9 ka\u0161\u013eom s\u00fa nielen \u010d\u00edm \u010falej t\u00fdm \u010dastej\u0161ie, no predl\u017euj\u00fa sa a maj\u00fa tendenciu pretrv\u00e1va\u0165 dlh\u0161ie ne\u017e pri be\u017enej infekcii d\u00fdchac\u00edch ciest.<\/p>\n

S progresiou ochorenia sa men\u00ed aj charakter ka\u0161\u013ea, ktor\u00fd je viac produkt\u00edvny s vyka\u0161liavan\u00edm hojn\u00e9ho hnisav\u00e9ho sp\u00fata, \u010dasto sfarben\u00e9ho do zelena. Priebeh ochorenia je \u010dasto preru\u0161ovan\u00fd ak\u00fatnymi stavmi, spojen\u00fdmi so zv\u00fdraznen\u00edm ka\u0161\u013ea a expektor\u00e1cie, \u010do b\u00fdva spravidla sprev\u00e1dzan\u00e9 zv\u00fdraznenou d\u00fdchavicou, nechutenstvom, chudnut\u00edm, retard\u00e1ciou rastu a zhor\u0161ovan\u00edm ukazovate\u013eov funkcie p\u013e\u00fac.<\/p>\n

Ob\u0161trukcia d\u00fdchac\u00edch ciest sa pri spirometrickom vy\u0161etren\u00edm prejavuje ako ob\u0161truk\u010dn\u00e1 ventila\u010dn\u00e1 porucha so zv\u00fd\u0161en\u00fdmi odpormi v d\u00fdchac\u00edch cest\u00e1ch. Vznik\u00e1 spo\u010diatku ako d\u00f4sledok upchatia priedu\u0161iek tuh\u00fdm visk\u00f3znym sekr\u00e9tom, v neskor\u0161om \u0161t\u00e1diu ako d\u00f4sledok de\u0161trukcie d\u00fdchac\u00edch ciest.<\/p>\n

\"201207271310_kompletna_spirometria\"<\/div>\n

P\u013e\u00facna hypertenzia sa prejavuje pri zhor\u0161ovan\u00ed p\u013e\u00facneho postihnutia, ke\u010f p\u013e\u00faca nie s\u00fa schopn\u00e9 zabezpe\u010di\u0165 adekv\u00e1tnu v\u00fdmenu d\u00fdchac\u00edch plynov a doch\u00e1dza k poklesu arteri\u00e1lneho parci\u00e1lneho tlaku kysl\u00edka, \u010do m\u00f4\u017ee vies\u0165 a\u017e k vzniku chronick\u00e9ho p\u013e\u00facneho srdca.<\/p>\n

Aspexia – vonkaj\u0161\u00edmi ne\u0161pecifick\u00fdmi prejavmi chronick\u00e9ho p\u013e\u00facneho ochorenia m\u00f4\u017eu by\u0165 pali\u010dkovit\u00e9 prsty, s\u00fadkovit\u00e9 postavenie hrudn\u00edka. U pacientov s CF je ve\u013emi d\u00f4le\u017eit\u00fd stav v\u00fd\u017eivy, ktor\u00fd ovplyv\u0148uje celkov\u00fd priebeh ochorenia. Chudnutie a retard\u00e1cia rastu predstavuj\u00fa progres ochorenia Boles\u0165 na hrudn\u00edku m\u00f4\u017ee sprev\u00e1dza\u0165 mno\u017estvo komplik\u00e1ci\u00ed objavuj\u00facu\u00edch sa pri CF (napr. pneumotorax, v\u00fdpotok, atelekt\u00e1za, abces p\u013e\u00fac, pneum\u00f3nia, svalov\u00e1 boles\u0165, frakt\u00fara rebra sp\u00f4soben\u00e1 ka\u0161\u013eom, ezofagit\u00edda…), ktor\u00e9 si pre svoju z\u00e1va\u017enos\u0165 vy\u017eaduj\u00fa r\u00fdchlu diagnostiku.<\/p>\n

Jednou z naj\u010dastej\u0161\u00edch komplik\u00e1cii u pacientov s CF s\u00fa hemopt\u00fdzy. M\u00f4\u017eu sa objavi\u0165 v miernej forme, ako pr\u00edmes krvi v sp\u00fate a vz\u00e1cnej\u0161ie mas\u00edvne vyka\u0161liavanie krvi zvy\u010dajne pri ak\u00fatnej exacerb\u00e1cii chronickej respira\u010dnej infekcie.<\/p>\n

Diagnostika<\/strong><\/h4>\n

Pod\u013ea Eur\u00f3pskeho konsenzu \u0161tandardnej starostlivosti o CF pacientov je pri podozren\u00ed na cystick\u00fa fibr\u00f3zu potrebn\u00e9 kompletn\u00e9 anamnestick\u00e9 a fyzik\u00e1lne vy\u0161etrenie pacienta, vy\u0161etrenie chloridov v pote, vy\u0161etrenie genotypu a pr\u00edtomnosti pankreatickej insuficiencie.<\/p>\n

Ak je diagn\u00f3za potvrden\u00e1, v r\u00e1mci inici\u00e1lneho vy\u0161etrenia je nutn\u00e9 realizova\u0165 vy\u0161etrenie arteriov\u00fdch krvn\u00fdch plynov, funk\u010dn\u00e9 vy\u0161etrenie p\u013e\u00fac vr\u00e1tane bronchodilata\u010dn\u00fdch testov pri ob\u0161trukcii d\u00fdchac\u00edch ciest.<\/p>\n

Zo zobrazovac\u00edch vy\u0161etreni\u00ed sa realizuj\u00fa RTG hrudn\u00edka a HRCT hrudn\u00edka, ktor\u00e9 umo\u017e\u0148uje zobrazi\u0165 reverzibiln\u00e9 zmeny (zhrubnutie stien bronchov, muk\u00f3zne z\u00e1tky, infiltr\u00e1ty, atelekt\u00e1zu…) ireverzibiln\u00e9 zmeny (bronchiekt\u00e1zie, fibrotiz\u00e1cia, sekul\u00e1cie, cysty, emfyz\u00e9m).<\/p>\n

Labor\u00e1torne sa vy\u0161etruje krvn\u00fd obraz, hemokoagula\u010dn\u00e9 parametre, biochemick\u00e9 parametre, vr\u00e1tane z\u00e1palov\u00fdch markerov ako CRP, IgG, pod\u013ea mo\u017enosti hladiny v tukoch rozpustn\u00fdch vitam\u00ednov (najm\u00e4 A a D), mno\u017estvo celkov\u00e1ch IgE, ale aj \u0161pecifick\u00e9 aspergilov\u00e9 IgE a precipit\u00edny v r\u00e1mci diagnostiky ABPA. K z\u00e1kladn\u00fdm diagnostick\u00fdm vy\u0161etreniam patr\u00ed aj mikroskopick\u00e9 a kultiva\u010dn\u00e9 vy\u0161etrenie sp\u00fata.<\/p>\n

Echokartdiografick\u00e9 vy\u0161etrenie orienta\u010dne posudzuje ve\u013ekos\u0165 tlaku v p\u013e\u00facnej art\u00e9rii. Diagnostikovan\u00ed pacienti s\u00fa na\u010falej sledovan\u00ed v CF centr\u00e1ch. Frekvencia n\u00e1v\u0161tev je individu\u00e1lna, ale mala by by\u0165 realizovan\u00e1 v trojmesa\u010dn\u00fdch intervaloch.<\/p>\n

Lie\u010dba
\n<\/strong><\/h4>\n

Prevencia minimalizuje progresiu ochorenia, preto je d\u00f4le\u017eit\u00e9 predch\u00e1dza\u0165 nebezpe\u010dn\u00fdm n\u00e1kazam a rizik\u00e1m prenosu bakt\u00e9rii a to dodr\u017eiavan\u00edm odpor\u00fa\u010dan\u00e9ho hygienick\u00e9ho \u0161tandardu pacientmi, dodr\u017eiavan\u00edm hygienicko-epidemiologick\u00fdch opatren\u00ed v zdravotn\u00edckych zariadeniach a \u00fa\u010dinnou prevenciou je aj o\u010dkovanie (napr. be\u017en\u00e9 o\u010dkovania u det\u00ed, hepatit\u00edda, v\u00edrus chr\u00edpky, Haemophillus influenze, Streptococcus pneumoniae, v \u0161t\u00e1diu sk\u00fa\u0161ania je vakc\u00edna proti Pseudomonas a.)<\/p>\n

Rehabilit\u00e1cia je jedn\u00fdm z prv\u00fdch krokov lie\u010dby a del\u00ed sa na techniky u\u013eah\u010duj\u00face o\u010dis\u0165ovanie bronchov a na kondi\u010dn\u00e9 cvi\u010denia. Odstra\u0148ovan\u00edm hnisav\u00e9ho sekr\u00e9tu z d\u00fdchac\u00edch ciest sa zni\u017euje bronchi\u00e1lna ob\u0161trukcia, odpor d\u00fdchacich ciest, zlep\u0161uj\u00fa sa funk\u010dn\u00e9 parametre a odstra\u0148uje \u017eivn\u00e1 p\u00f4da pre bakt\u00e9rie. Na podporu tejto techniky mo\u017eno pou\u017ei\u0165 jednoduch\u00e9 pom\u00f4cky ako s\u00fa Flutter, R-C Cornet, Acapella, PEP maska alebo technicky n\u00e1ro\u010dnej\u0161\u00ed pr\u00edstroj The Vest. Vhodn\u00fdm kondi\u010dn\u00fdm cvi\u010den\u00edm sa zvy\u0161uje kardiorespira\u010dn\u00e1 kond\u00edcia, odolnos\u0165 dychov\u00fdch svalov, zn\u00ed\u017eenie pocitu dychovej nedostato\u010dnosti, podporuje sa mukocili\u00e1rny kl\u00edrens sp\u00fata, zvy\u0161uje svalov\u00e1 masa a svalov\u00e1 sila.<\/p>\n

Je jednozna\u010dne potvrden\u00e1 skuto\u010dnos\u0165, \u017ee pravideln\u00e9 denne kondi\u010dn\u00e9 cvi\u010denie predl\u017euje \u017eivot pacientov, ale zlep\u0161uje aj kvalitu \u017eivota a funk\u010dn\u00e9 parametre p\u013e\u00fac.<\/p>\n

Lieky
\n<\/strong><\/h4>\n

Bronchodilata\u010dn\u00e9 lieky zlep\u0161uj\u00fa p\u013e\u00facne funkcie u pacientov s CF a m\u00f4\u017eu zni\u017eova\u0165 frekvenciu ATB lie\u010dby. D\u00f4le\u017eit\u00fd v\u00fdznam m\u00e1 tie\u017e ich pod\u00e1vanie pred fyzioterapiou, zlep\u0161uj\u00fa mukocili\u00e1rny kl\u00edrens d\u00fdchac\u00edch ciest.<\/p>\n

Mukolytik\u00e1 s\u00fa d\u00f4le\u017eitou skupinou liekov, ktor\u00e9 u\u013eah\u010duj\u00fa o\u010dis\u0165ovanie d\u00fdchac\u00edch ciest od hust\u00e9ho visk\u00f3zneho hlienu. Jedn\u00fdm z prelomov\u00fdch mukolyt\u00edk je inhala\u010dne aplikovan\u00e1 rekombinantn\u00e1 \u013eudsk\u00e1 DNAza (alfa dorn\u00e1za – Pulmozyme). \u010eal\u0161\u00edm \u00fa\u010dinn\u00fdm mukolytikom je hypertonick\u00fd roztok NaCl, N-acetylcystein (Solmucol, ACC) a najnov\u0161ie inhala\u010dn\u00fd manitol (Bronchitol).<\/p>\n

Imunomodul\u00e1tory a protiz\u00e1palov\u00e9 lieky maj\u00fa ve\u013ek\u00fd v\u00fdznam pri potl\u00e1\u010dan\u00ed chronick\u00e9ho z\u00e1palu, ktor\u00fd je najz\u00e1va\u017enej\u0161\u00edm d\u00f4vodom de\u0161trukcie d\u00fdchac\u00edch ciest pri CF. T\u00fato skupinu liekov predstavuj\u00fa napr. makrolidy, kortikosteroidy, nesteroidn\u00e9 antiflogistik\u00e1 (ibuprof\u00e9n). Ich nev\u00fdhodou je, \u017ee dlhodob\u00e9 u\u017e\u00edvanie m\u00f4\u017ee nies\u0165 riziko ne\u017eiad\u00facich \u00fa\u010dinkov.<\/p>\n

Antibiotik\u00e1 s\u00fa neodmyslite\u013enou s\u00fa\u010das\u0165ou lie\u010dby CF a sp\u00f4sob ich podania z\u00e1vis\u00ed od kultiva\u010dne zistenej bakt\u00e9rie a v\u00fdber sa riadi citlivos\u0165ou na antibiotik\u00e1, alebo potvrden\u00fdm synergick\u00fdm \u00fa\u010dinkom zvolen\u00fdch antibiot\u00edk Oxygenoterapia – lie\u010dba kysl\u00edkom m\u00f4\u017ee by\u0165 pod\u00e1van\u00e1 pacientom do\u010dasne pri zhor\u0161en\u00ed stavu, alebo pri poklese satur\u00e1cie art\u00e9riovej krvi. Ak ned\u00f4jde k normaliz\u00e1cii satur\u00e1cie mal by by\u0165 pacient nastaven\u00fd na dlhodob\u00fa dom\u00e1cu oxygenoterapiu v trvan\u00ed aspo\u0148 16 hod\u00edn denne.<\/p>\n

Transplant\u00e1cia p\u013e\u00fac je posledn\u00e1 mo\u017enos\u0165 rie\u0161enia z\u00e1va\u017en\u00e9ho p\u013e\u00facneho po\u0161kodenia pri CF. K transplant\u00e1cii je pacient indikovan\u00fd ak spln\u00ed ur\u010dit\u00e9 krit\u00e9ria, ku ktor\u00fdm patr\u00ed napr. pokles FEV1 pod 30%, mas\u00edvne hemopt\u00fdzy, r\u00fdchla progresia respira\u010dnej insuficiencie, zv\u00fd\u0161en\u00e1 frekvencia hospitaliz\u00e1cii, prekro\u010denie stanoven\u00fdch hodn\u00f4t O2 a CO2. D\u00f4le\u017eit\u00fdm predpokladom \u00faspe\u0161nej transplant\u00e1cie a potransplanta\u010dn\u00e9ho priebehu je dobr\u00e1 fyzick\u00e1 kond\u00edcia pacienta.<\/p>\n

N\u00e1dejou do bud\u00facnosti pri lie\u010dbe CF je g\u00e9nov\u00e1 terapia, na ktorej pracuj\u00fa vedci-odborn\u00edci a h\u013eadaj\u00fa sp\u00f4soby prenosu opraven\u00e9ho g\u00e9nu CFTR do bunky. To by malo pacientov zbavi\u0165 choroby a jej pr\u00edznakov.<\/p>\n

Zdroj: \u010derpan\u00e9 z odbornej pr\u00e1ce MUDr. Branislava Remi\u0161a
\nKlinika pneumol\u00f3gie a ftizeol\u00f3gie I. SZU, Univerzitn\u00e1 nemocnica Bratislava<\/em><\/p>\n","protected":false},"excerpt":{"rendered":"

Poškodenie respiračného systému je hlavnou príčinou morbidity a mortality u pacientov s cystickou fibrózou (CF). Klinické prejavy ochorenia sú veľmi pestré. Z hľadiska rozmanitosti a intenzity zahŕňajú širokú škálu obrazov, ktoré môžu byť u každého chorého osobité a od ktorých … Continued<\/a><\/p>\n","protected":false},"author":6,"featured_media":1130,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"kt_blocks_editor_width":"","footnotes":""},"categories":[253],"tags":[260,33],"class_list":["post-910","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-o-cystickej-fibroze","tag-klinicke-prejavy","tag-liecba"],"yoast_head":"\nCystick\u00e1 fibr\u00f3za: Klinick\u00e9 prejavy a lie\u010dba - Slan\u00e9 deti<\/title>\n<meta name=\"description\" content=\"Klinick\u00e9 prejavy cystickej fibr\u00f3zy, diagnostika a lie\u010dba spolu s popisom mikroorganizmov doln\u00fdch d\u00fdchac\u00edch ciest.\" \/>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/slanedeti.sk\/cms\/cysticka-fibroza-klinicke-prejavy-a-liecba\/910\" \/>\n<meta property=\"og:locale\" content=\"sk_SK\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Cystick\u00e1 fibr\u00f3za: Klinick\u00e9 prejavy a lie\u010dba - 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