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Jan 21, 2005 · FPF = familial pulmonary fibrosis; GERD = gastroesophageal reflux disease; HRCT = high-resolution computed tomography; MOI = mode of inheritance; PF = pulmonary fibrosis 1. Most often associated with pathogenic variants in TERC or TERT
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What is familial pulmonary fibrosis (FPF)?
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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.
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
Potential Results of Genetic Tests - Up to 30% of cases of FPF will have a positive test for a causative mutation in a gene implicated in FPF. In this case, family members can undergo genetic testing to determine if they have the same mutation and are at increased risk for developing FPF.
Abstract. 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.