scholarly journals Primary interstitial lung disease specific to childhood due to pulmonary surfactant protein anomalies

2020 ◽  
Author(s):  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Pulmonary Surfactant ◽  
Surfactant Protein ◽  
Disease Specific ◽  
Pulmonary Surfactant Protein

scholarly journals Evaluation of usefulness in surfactant protein D as a predictor of mortality in myositis-associated interstitial lung disease

PLoS ONE ◽  
2020 ◽  
Vol 15 (6) ◽  
pp. e0234523
Author(s):  
Shinjiro Kaieda ◽  
Takahisa Gono ◽  
Kenichi Masui ◽  
Naoshi Nishina ◽  
Shinji Sato ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Surfactant Protein ◽  
Surfactant Protein D

scholarly journals Interaction of Pulmonary Surfactant Protein SP-A with DPPC/Egg-PG Bilayers

2003 ◽  
Vol 85 (4) ◽  
pp. 2397-2405 ◽  
Author(s):  
Michael R. Morrow ◽  
Nidal Abu-Libdeh ◽  
June Stewart ◽  
Kevin M.W. Keough
Keyword(s):  
Pulmonary Surfactant ◽  
Surfactant Protein ◽  
Pulmonary Surfactant Protein

scholarly journals Molecular structure of pulmonary surfactant protein D (SP-D).

1994 ◽  
Vol 269 (25) ◽  
pp. 17311-17319 ◽  
Author(s):  
E. Crouch ◽  
A. Persson ◽  
D. Chang ◽  
J. Heuser
Keyword(s):  
Molecular Structure ◽  
Pulmonary Surfactant ◽  
Surfactant Protein ◽  
Surfactant Protein D ◽  
Pulmonary Surfactant Protein

Clinical Characteristics and Genetic Analysis of Interstitial Lung Disease in Chinese Children (Genes and Interstitial Lung Disease)

2021 ◽  
Author(s):  
Mei-Xia Huang ◽  
Lu Qin ◽  
Fei-Zhou Zhang ◽  
Lei Wu ◽  
Jia-Hui Yu ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Clinical Characteristics ◽  
Clinical Manifestations ◽  
Failure To Thrive ◽  
Surfactant Protein ◽  
Chinese Children ◽  
Common Mutation ◽  
Onset Age ◽  
Surfactant Dysfunction

Abstract BackgroundMutation in the surfactant protein C gene (SFTPC) is a cause of interstitial lung disease (ILD). Our objective was to investigate the clinical characteristics, outcome and influencing factors of ILD in Chinese children with SFTPC mutations.MethodA total of 8 Chinese children with ILD heterozygous for SFTPC mutations that were treated in our hospital from January 2014 to December 2020 were included in our study. Candidate genes responsible for surfactant dysfunction were sequenced by next-generation sequencing. The clinical and genetic data were reviewed retrospectively.ResultsThe children’s onset age was before the age of 2 years, and one case was just after birth. The most significant clinical manifestations were cough, tachypnea, hypoxemia and failure to thrive. The most common mutation was p. lle73Thr, which accounted for 87.5% (7/8) of our patients. Four patients whose onset was within 3 months, including 3 children with CMV infection, died. Conclusionp. lle73Thr mutation of SFTPC was an important and common cause of ILD in the Chinese children. The clinical manifestations of ILD associated with this mutation are not specific. The severity and outcome of the disease may be affected by factors such as onset age and viral infection.

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