title: [Difficulties in diagnosis of Bourneville-Pringle's diseases].
abstract: The authors present a case of Bourneville-Pringle disease in a 43 year-old female patient. The diagnosis was confirmed after 8 years from the beginning of disease. She was hospitalized several times and treated by different specialists. The diagnosis was confirmed in pulmonary department in SZChPiG in Bystra Sl. They observed multiorgan changes concerning not only the respiratory system, but also skin, kidneys, central nervous system. Medical history of patient, family data, the course of disease, histopathological changes in kidneys, specific ultrasonographic picture of the urinary system, high resolution computed tomography picture of lungs and classical computed tomography pictures of brain confirmed diagnosis. Tamoxifen was used in therapy. The improvement of quality of life and delay of natural progression was obtained. The course of disease observed during 20 years leads to ventilatory disturbances mainly obstructive type and respiratory insufficiency. The prognosis of presented patient still remains uncertain in spite of survival exceeding 20 years.
authors.affiliation: Oddział Pulmonologiczny Specjalistycznego Zespołu Chorób Płuc i Gruźlicy w Bystrej Slaskiej.
authors.forename: I
authors.initials: I
authors.lastname: Markiewicz-Bendkowska
authors.forename: A
authors.initials: A
authors.lastname: Pyź
mesh_terms: D000328:Adult; D001921:Brain / Q000000981:diagnostic imaging; D018450:Disease Progression; D005260:Female; D006801:Humans; D007668:Kidney / Q000473:pathology; D011379:Prognosis; D011788:Quality of Life; D012140:Respiratory Tract Diseases / Q000000981:diagnostic imaging; D013629:Tamoxifen / Q000627:therapeutic use; D014057:Tomography, X-Ray Computed; D014402:Tuberous Sclerosis / Q000175:diagnosis* / Q000188:drug therapy
chemical_list: D013629:Tamoxifen