Pleuroparenchymal fibroelastosis associated with telomerase reverse transcriptase mutations
نویسندگان
چکیده
منابع مشابه
Pleuroparenchymal fibroelastosis presenting with pneumothorax
A 47-year-old woman presented with spontaneous right side pneumothorax. Image studies showed consolidations and reticular opacities involving the pleural and subpleural regions of bilateral lungs. Wedge biopsy specimens of right upper, middle and lower lobes showed fibrosis of the visceral pleura and subpleural area in all three lobes, more significant in the upper lobe. Elastic Van Gieson stai...
متن کاملIdiopathic Pleuroparenchymal Fibroelastosis
PURPOSE OF THE REVIEW Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare fibrosing lung disease, affecting the visceral pleura and the subpleural parenchyma with an upper lobe predilection, included as a distinct clinicopathologic entity in the latest international multidisciplinary classification of the idiopathic interstitial pneumonias (IIP). We aim to summarize the current eviden...
متن کاملPleuroparenchymal fibroelastosis associated with aluminosilicate dust: a case report.
Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a recently described rare condition, characterized by pleural and subpleural parenchymal fibrosis, predominantly in the upper lobes. The clinical course of this disease is progressive and prognosis is poor, with little information regarding the etiology of IIPPFE. This report describes an IPPFE patient with convincing evidence of inhalation...
متن کاملPleuroparenchymal Fibroelastosis: Its Clinical Characteristics
Pleuroparenchymal fibroelastosis (PPFE) is a rare pulmonary fibrosis that is clinically characterized by upperlobe predominant fibrosis. PPFE is a slowly progressive disorder and its first symptom is dyspnea or dry cough. Chest pain because of pneumothorax may be the first symptom in some patients. Patients with PPFE are slender with a flat rib cage or abnormally narrowed anterior-posterior tho...
متن کاملTelomerase Reverse Transcriptase Regulates microRNAs
MicroRNAs are small non-coding RNAs that inhibit the translation of target mRNAs. In humans, most microRNAs are transcribed by RNA polymerase II as long primary transcripts and processed by sequential cleavage of the two RNase III enzymes, DROSHA and DICER, into precursor and mature microRNAs, respectively. Although the fundamental functions of microRNAs in RNA silencing have been gradually unc...
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ژورنال
عنوان ژورنال: European Respiratory Journal
سال: 2017
ISSN: 0903-1936,1399-3003
DOI: 10.1183/13993003.02022-2016