Familiární idiopatická intersticiální pneumonice - případ jedné rodiny
| Title in English | Familial idiopathic interstitial pneumonia - a case of one family |
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| Authors | |
| Year of publication | 2013 |
| Type | Article in Periodical |
| Magazine / Source | Studia pneumologica et phthiseologica |
| MU Faculty or unit | |
| Citation | |
| Field | Pneumology |
| Keywords | familial idiopathic intersticial pneumonia; familial idiopathic pulmonary fibrosis; sporadic idiophatic pulmonary fibrosis; surfactant protein |
| Description | 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. |