. . "Pediatrie pro Praxi" . "Terapeutick\u00E9 trendy cystick\u00E9 fibr\u00F3zy" . "RIV/00216208:11130/14:10293422" . "4"^^ . . "Therapeutic strategies in cystic fibrosis"@en . . "Therapeutic strategies in cystic fibrosis"@en . "ivacaftor; CFTR potentiators and correctors; treatment; standards of care; diagnosis; cystic fibrosis"@en . "Skalick\u00E1, Veronika" . "Cystic fibrosis (CF) is the most common autosomal recessive disease of the Caucasian population characterised by chronic multi-organ impairment. Although cystic fibrosis is still incurable condition, it has been shown that holistic approach to treatment that is based on multi-professional centre care provided by trained and experienced health professionals is necessary for optimal management and improved quality of life and survival of patients. Complex treatment focused on intensive airway clearance, good nutritional status, aggressive anti-infective and anti-inflammatory therapy and continuous search for complications is essential. New therapeutic strategies for CF focus on protein targeted treatment of impaired cystic fibrosis transmembrane conductance regulator (CFTR) that is responsible for exocrine secretion pathology."@en . "6" . "Terapeutick\u00E9 trendy cystick\u00E9 fibr\u00F3zy"@cs . . . . "Cystick\u00E1 fibr\u00F3za je nej\u010Dast\u011Bj\u0161\u00EDm autozom\u00E1ln\u011B recesivn\u011B d\u011Bdi\u010Dn\u00FDm onemocn\u011Bn\u00EDm kavkazsk\u00E9 rasy. Onemocn\u011Bn\u00ED m\u00E1 charakter multiorg\u00E1nov\u00E9ho posti\u017Een\u00ED. Je sice nevyl\u00E9\u010Diteln\u00E9, ale st\u00E1le v\u00EDce a l\u00E9pe l\u00E9\u010Diteln\u00E9 s postupn\u00FDm zlep\u0161ov\u00E1n\u00EDm kvality a prodlu\u017Eov\u00E1n\u00EDm d\u00E9lky \u017Eivota nemocn\u00FDch. Progn\u00F3za velmi z\u00E1vis\u00ED na v\u010Dasn\u00E9 diagnostice, multioborov\u00E9m l\u00E9\u010Debn\u00E9m p\u0159\u00EDstupu s vyu\u017Eit\u00EDm nejnov\u011Bj\u0161\u00EDch poznatk\u016F i hlubok\u00FDch zku\u0161enost\u00ED. Komplexn\u00ED l\u00E9\u010Dba je zam\u011B\u0159en\u00E1 na p\u00E9\u010Di o dobrou pr\u016Fchodnost d\u00FDchac\u00EDch cest, p\u00E9\u010Di o dobr\u00FD stav v\u00FD\u017Eivy, boj proti infekci a z\u00E1n\u011Btu a soustavn\u00E9 vyhled\u00E1v\u00E1n\u00ED a l\u00E9\u010Dbu komplikac\u00ED. Nejnov\u011Bj\u0161\u00ED terapeutick\u00E9 trendy sm\u011B\u0159uj\u00ED k l\u00E9\u010Db\u011B kauz\u00E1ln\u00ED na principu n\u00E1pravy defektn\u00EDho proteinu zodpov\u011Bdn\u00E9ho za patologick\u00E9 slo\u017Een\u00ED sekret\u016F exokrinn\u00EDch \u017El\u00E1z."@cs . "[6D3428810D34]" . "1"^^ . . "RIV/00216208:11130/14:10293422!RIV15-MSM-11130___" . "Cystick\u00E1 fibr\u00F3za je nej\u010Dast\u011Bj\u0161\u00EDm autozom\u00E1ln\u011B recesivn\u011B d\u011Bdi\u010Dn\u00FDm onemocn\u011Bn\u00EDm kavkazsk\u00E9 rasy. Onemocn\u011Bn\u00ED m\u00E1 charakter multiorg\u00E1nov\u00E9ho posti\u017Een\u00ED. Je sice nevyl\u00E9\u010Diteln\u00E9, ale st\u00E1le v\u00EDce a l\u00E9pe l\u00E9\u010Diteln\u00E9 s postupn\u00FDm zlep\u0161ov\u00E1n\u00EDm kvality a prodlu\u017Eov\u00E1n\u00EDm d\u00E9lky \u017Eivota nemocn\u00FDch. Progn\u00F3za velmi z\u00E1vis\u00ED na v\u010Dasn\u00E9 diagnostice, multioborov\u00E9m l\u00E9\u010Debn\u00E9m p\u0159\u00EDstupu s vyu\u017Eit\u00EDm nejnov\u011Bj\u0161\u00EDch poznatk\u016F i hlubok\u00FDch zku\u0161enost\u00ED. Komplexn\u00ED l\u00E9\u010Dba je zam\u011B\u0159en\u00E1 na p\u00E9\u010Di o dobrou pr\u016Fchodnost d\u00FDchac\u00EDch cest, p\u00E9\u010Di o dobr\u00FD stav v\u00FD\u017Eivy, boj proti infekci a z\u00E1n\u011Btu a soustavn\u00E9 vyhled\u00E1v\u00E1n\u00ED a l\u00E9\u010Dbu komplikac\u00ED. Nejnov\u011Bj\u0161\u00ED terapeutick\u00E9 trendy sm\u011B\u0159uj\u00ED k l\u00E9\u010Db\u011B kauz\u00E1ln\u00ED na principu n\u00E1pravy defektn\u00EDho proteinu zodpov\u011Bdn\u00E9ho za patologick\u00E9 slo\u017Een\u00ED sekret\u016F exokrinn\u00EDch \u017El\u00E1z." . "15" . . . "http://www.pediatriepropraxi.cz/pdfs/ped/2014/06/04.pdf" . . . "1"^^ . "11130" . "I" . . . "Terapeutick\u00E9 trendy cystick\u00E9 fibr\u00F3zy" . . "Terapeutick\u00E9 trendy cystick\u00E9 fibr\u00F3zy"@cs . "49934" . "1213-0494" . "CZ - \u010Cesk\u00E1 republika" . . .