"1213-810X" . . "CZ - \u010Cesk\u00E1 republika" . "RIV/00216224:14110/13:00070492" . "Binkov\u00E1, Ilona" . . "Doubkov\u00E1, Martina" . "Famili\u00E1rn\u00ED idiopatick\u00E1 interstici\u00E1ln\u00ED pneumonie (IIP) je definov\u00E1na jako onemocn\u011Bn\u00ED, kter\u00E9 postihuje 2 nebo v\u00EDce \u010Dlen\u016F t\u00E9\u017Ee rodiny a m\u00E1 klinick\u00E9 a histologick\u00E9 znaky interstici\u00E1ln\u00ED pneumonie. Zp\u016Fsob d\u011Bdi\u010Dnosti nenmoci nen\u00ED pln\u011B rozpozn\u00E1n,ale zd\u00E1 se, \u017Ee se jedn\u00E1 o autosom\u00E1ln\u011B dominantn\u00ED onemocn\u011Bn\u00ED s ne\u00FAplnou penetrac\u00ED. Nej\u010Dast\u011Bj\u0161\u00EDm fenotypem u rodin\u00E9ho v\u00FDskytu IIP je fenotyp IPF/UIP (idiopatick\u00E1 plicn\u00ED fibr\u00F3za obbvykl\u00E9ho typu)- P\u0159esn\u00E1 prevence famili\u00E1rn\u00ED IIP nen\u00ED rovn\u011B\u017E zn\u00E1ma. Podle sou\u010Dasn\u00FDch poznatk\u016F se odhaduje, \u017Ee tvo\u0159\u00ED 0,5 - 3,7 % v\u0161ech p\u0159\u00EDpad\u016F IPF. V na\u0161\u00ED pr\u00E1ci popisujeme dva p\u0159\u00EDpady IIP - u matky a syna. Ka\u017Ed\u00FD z nich m\u00E1 jinou formu a jin\u00FD rozsah interstci\u00E1ln\u00ED pneumonie." . . . "Famili\u00E1rn\u00ED idiopatick\u00E1 interstici\u00E1ln\u00ED pneumonie (IIP) je definov\u00E1na jako onemocn\u011Bn\u00ED, kter\u00E9 postihuje 2 nebo v\u00EDce \u010Dlen\u016F t\u00E9\u017Ee rodiny a m\u00E1 klinick\u00E9 a histologick\u00E9 znaky interstici\u00E1ln\u00ED pneumonie. Zp\u016Fsob d\u011Bdi\u010Dnosti nenmoci nen\u00ED pln\u011B rozpozn\u00E1n,ale zd\u00E1 se, \u017Ee se jedn\u00E1 o autosom\u00E1ln\u011B dominantn\u00ED onemocn\u011Bn\u00ED s ne\u00FAplnou penetrac\u00ED. Nej\u010Dast\u011Bj\u0161\u00EDm fenotypem u rodin\u00E9ho v\u00FDskytu IIP je fenotyp IPF/UIP (idiopatick\u00E1 plicn\u00ED fibr\u00F3za obbvykl\u00E9ho typu)- P\u0159esn\u00E1 prevence famili\u00E1rn\u00ED IIP nen\u00ED rovn\u011B\u017E zn\u00E1ma. Podle sou\u010Dasn\u00FDch poznatk\u016F se odhaduje, \u017Ee tvo\u0159\u00ED 0,5 - 3,7 % v\u0161ech p\u0159\u00EDpad\u016F IPF. V na\u0161\u00ED pr\u00E1ci popisujeme dva p\u0159\u00EDpady IIP - u matky a syna. Ka\u017Ed\u00FD z nich m\u00E1 jinou formu a jin\u00FD rozsah interstci\u00E1ln\u00ED pneumonie."@cs . "74623" . "Familial idiopathic interstitial pneumonia - a case of one family"@en . "2"^^ . "3"^^ . "familial idiopathic intersticial pneumonia; familial idiopathic pulmonary fibrosis; sporadic idiophatic pulmonary fibrosis; surfactant protein"@en . . . . "[4C77D20C9059]" . "Famili\u00E1rn\u00ED idiopatick\u00E1 interstici\u00E1ln\u00ED pneumonice - p\u0159\u00EDpad jedn\u00E9 rodiny"@cs . . "73" . . "5"^^ . "Famili\u00E1rn\u00ED idiopatick\u00E1 interstici\u00E1ln\u00ED pneumonice - p\u0159\u00EDpad jedn\u00E9 rodiny"@cs . "Sk\u0159i\u010Dkov\u00E1, Jana" . "Famili\u00E1rn\u00ED idiopatick\u00E1 interstici\u00E1ln\u00ED pneumonice - p\u0159\u00EDpad jedn\u00E9 rodiny" . "Studia pneumologica et phthiseologica" . "Familial idiopathic interstitial pneumonia - a case of one family"@en . . "5" . "Familial idiopathic intersticial pneumonia (IIP) is defined as disease observed in 2 or more members of the same family. The inheriatance pattern of IIP si not clear, but it seems to be autosomal dominant with incomplete penetrance. The most frequent phenotyp in familial IIP is idiopathic pulmonary fibrosis / usual interstitital pneumonia (IPF/UIP). Accurate epidemiological data are unavailable ( a mother and son). Each patient had a different form, extent and outcome of IIP."@en . . . . . "Famili\u00E1rn\u00ED idiopatick\u00E1 interstici\u00E1ln\u00ED pneumonice - p\u0159\u00EDpad jedn\u00E9 rodiny" . . . "I" . . "RIV/00216224:14110/13:00070492!RIV14-MSM-14110___" . "14110" .