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Jun 26, 2021 · Familial pulmonary fibrosis (FPF) represents a unique subgroup of patients in which at least one other relative is also affected. Patients with FPF exhibit a wide range of pulmonary fibrosis phenotypes, although idiopathic pulmonary fibrosis is the most common subtype.
- David Zhang, Chad A. Newton
- 2021
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Jan 21, 2005 · Goal 1. Briefly describe the clinical characteristics of pulmonary fibrosis. Goal 2. Review genetic causes of pulmonary fibrosis. Goal 3. Review the differential diagnosis of pulmonary fibrosis. Goal 4. Provide an evaluation strategy to identify the genetic cause of pulmonary fibrosis in a proband. Goal 5.
Jan 14, 2023 · The primary objective is determining the diagnostic value of parameters for detection of early FPF; the secondary objectives are determining the optimal timing of the screening interval and gaining insight into the natural history of early FPF.
- 10.3390/jcm12020674
- 2023/01
- J Clin Med. 2023 Jan; 12(2): 674.
Familial pulmonary fibrosis (FPF) is defined as interstitial lung involvement in at least two members of the same biological family. Pathogenesis of FPF involves background of genetic risk factors further modified by environmental exposures and aging.
Patients with FPF exhibit a wide range of pul-monary brosis phenotypes, although idiopathic pulmonary brosis is the most common sub-fi fi type. Despite variable disease manifestations, patients with FPF experience worse survival compared with their counterparts with the sporadic disease form.