Matches in SemOpenAlex for { <https://semopenalex.org/work/W3023355860> ?p ?o ?g. }
- W3023355860 endingPage "104024" @default.
- W3023355860 startingPage "104024" @default.
- W3023355860 abstract "At least 10% of patients with pulmonary fibrosis, whether idiopathic or secondary, present heritable pulmonary fibrosis suspected on familial aggregation of pulmonary fibrosis, specific syndromes or early age of diagnosis. Approximately 30% of those patients have an identified mutation mostly in telomere related genes (TRG) more rarely in surfactant homeostasis or other genes. TRG mutation may be associated with hematological and hepatic diseases that may worsen after lung transplantation requiring a specific care and adapted immunosuppression. Surfactant genes mutations are usually associated with ground-glass opacities and cysts on CT scan and may improve with steroids, hydroxychloroquine or azithromycin. Moreover relatives should benefit from a genetic analysis associated with a clinical evaluation according to the gene involved. Genetics of pulmonary fibrosis raise specific problems from diagnosis, therapy or genetic counseling varying from one gene to another." @default.
- W3023355860 created "2020-05-13" @default.
- W3023355860 creator A5028173975 @default.
- W3023355860 creator A5042208385 @default.
- W3023355860 creator A5072347403 @default.
- W3023355860 creator A5075038951 @default.
- W3023355860 creator A5085204876 @default.
- W3023355860 creator A5087236365 @default.
- W3023355860 date "2020-06-01" @default.
- W3023355860 modified "2023-10-18" @default.
- W3023355860 title "Impact of genetic factors on fibrosing interstitial lung diseases. Incidence and clinical presentation in adults" @default.
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