title: Pulmonary arterial hypertension in childhood: an unsual presentation with fibrosing mediastinitis.
abstract: Acquired stenosis of normally connected pulmonary veins is a rare condition in children, usually associated with mediastinal processes. It may present later with a less specific clinical picture, symptoms and signs mimicking chronic lung disease. Fibrosing mediastinitis is a rarer disorder of unknown etiology, although several suspected causes such as granulomatous diseases, characterized by fibrous tissue proliferation within the mediastinum, leading to respiratory and cardiac failure by bronchial obstruction or pulmonary hypertension.
authors.affiliation: University Hospital of Parma. elisabetta.teopompi@studenti.unipr.it.
authors.forename: Elisabetta
authors.initials: E
authors.lastname: Teopompi
authors.forename: Bertrand
authors.initials: B
authors.lastname: Tchana
authors.forename: Alfredo
authors.initials: A
authors.lastname: Chetta
authors.forename: Marina
authors.initials: M
authors.lastname: Aiello
authors.forename: Marta
authors.initials: M
authors.lastname: Sgrignoli
authors.forename: Nicola
authors.initials: N
authors.lastname: Carano
authors.forename: Aldo
authors.initials: A
authors.lastname: Agnetti
mesh_terms: D000293:Adolescent; D006328:Cardiac Catheterization / Q000379:methods*; D003937:Diagnosis, Differential; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D008297:Male; D008480:Mediastinitis / Q000150:complications* / Q000175:diagnosis; D011669:Pulmonary Wedge Pressure; D012598:Sclerosis / Q000150:complications* / Q000175:diagnosis; D014057:Tomography, X-Ray Computed / Q000379:methods*