Familial Pulmonary Fibrosis: Genetic Features and Clinical Implications

CHEST Journal ◽  
2021 ◽  
Author(s):  
David Zhang ◽  
Chad A. Newton
Keyword(s):  
Pulmonary Fibrosis ◽  
Clinical Implications ◽  
Genetic Features ◽  
Familial Pulmonary Fibrosis

scholarly journals Idiopathic pulmonary fibrosis: unmasking cryptogenic environmental factors

2019 ◽  
Vol 53 (2) ◽  
pp. 1801699 ◽  
Author(s):  
Coralynn Sack ◽  
Ganesh Raghu
Keyword(s):  
Environmental Factors ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Treatment Modality ◽  
Unknown Origin ◽  
Clinical Implications ◽  
High Morbidity ◽  
Novel Treatment ◽  
Progression Of Disease ◽  
Fibrotic Lung Disease

Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease of unknown origin that is associated with high morbidity and mortality. In this perspective, we briefly review the current understanding of the pathophysiology of IPF and the importance of environmental triggers as a precipitant of disease. We discuss occult intrinsic and extrinsic environmental factors that affect the lung microenvironment and may contribute to the development and progression of disease. The clinical implications of this framework need to be further elucidated, because prompt identification and elimination of occult exposures may represent a novel treatment modality.

Rapidly Progressive Familial Pulmonary Fibrosis In 4 Children With An Unknown DNA Repair Defect

2010 ◽  
Author(s):  
Timothy J. Vece ◽  
Marc G. Schecter ◽  
Claire Langston ◽  
Christine K. Garcia ◽  
Megan K. Dishop ◽  
...  
Keyword(s):  
Dna Repair ◽  
Pulmonary Fibrosis ◽  
Repair Defect ◽  
Familial Pulmonary Fibrosis

scholarly journals Surfactant protein C G100S mutation causes familial pulmonary fibrosis in Japanese kindred

2011 ◽  
Vol 38 (4) ◽  
pp. 861-869 ◽  
Author(s):  
S. Ono ◽  
T. Tanaka ◽  
M. Ishida ◽  
A. Kinoshita ◽  
J. Fukuoka ◽  
...  
Keyword(s):  
Pulmonary Fibrosis ◽  
Protein C ◽  
Surfactant Protein ◽  
Surfactant Protein C ◽  
Familial Pulmonary Fibrosis

scholarly journals Familial Pulmonary Fibrosis and Hermansky-Pudlak Syndrome Rare Missense Mutations in Context

2019 ◽  
Vol 200 (2) ◽  
pp. 253-256 ◽  
Author(s):  
Robert S. Stearman ◽  
Amber R. Cornelius ◽  
Lisa R. Young ◽  
David S. Conklin ◽  
Elizabeth A. Mickler ◽  
...  
Keyword(s):  
Pulmonary Fibrosis ◽  
Missense Mutations ◽  
Hermansky Pudlak Syndrome ◽  
Familial Pulmonary Fibrosis

scholarly journals Familial pulmonary fibrosis: Clinical and radiological characteristics and progression analysis in different high resolution-CT patterns

2017 ◽  
Vol 126 ◽  
pp. 75-83 ◽  
Author(s):  
David Bennett ◽  
Maria Antonietta Mazzei ◽  
Nevada Cioffi Squitieri ◽  
Elena Bargagli ◽  
Rosa Metella Refini ◽  
...  
Keyword(s):  
High Resolution ◽  
Pulmonary Fibrosis ◽  
High Resolution Ct ◽  
Familial Pulmonary Fibrosis

Lung Transplantation for Familial Pulmonary Fibrosis

2019 ◽  
Vol 38 (4) ◽  
pp. S325-S326
Author(s):  
S.M. Lari ◽  
D.J. Ross ◽  
R. Saggar ◽  
J.P Lynch ◽  
D. Sayah ◽  
...  
Keyword(s):  
Pulmonary Fibrosis ◽  
Lung Transplantation ◽  
Familial Pulmonary Fibrosis

scholarly journals Exome sequencing links mutations in PARN and RTEL1 with familial pulmonary fibrosis and telomere shortening

2015 ◽  
Vol 47 (5) ◽  
pp. 512-517 ◽  
Author(s):  
Bridget D Stuart ◽  
Jungmin Choi ◽  
Samir Zaidi ◽  
Chao Xing ◽  
Brody Holohan ◽  
...  
Keyword(s):  
Pulmonary Fibrosis ◽  
Exome Sequencing ◽  
Telomere Shortening ◽  
Familial Pulmonary Fibrosis
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