Pulmonary alveolar proteinosis: a long way to correct diagnosis: problems of diagnostics and therapy in routine practice.

Autor:	Ilkovich YM; Pulmonology Clinic of Pavlov State Medical University, St. Petersburg., Ariel BM; St. Petersburg City Mortem Bureau, St. Petersburg Institute of Phthysiopulmonology, St. Petersburg, Russia arielboris@rambler.ru., Novikova LN; Pulmonology Clinic of Pavlov State Medical University, St. Petersburg., Bazhanov AA; Pulmonology Clinic of Pavlov State Medical University, St. Petersburg., Dvorakovskaya IV; Pulmonology Clinic of Pavlov State Medical University, St. Petersburg., Ilkovich MM; Pulmonology Clinic of Pavlov State Medical University, St. Petersburg.
Jazyk:	angličtina
Zdroj:	Annals of clinical and laboratory science [Ann Clin Lab Sci] 2014 Fall; Vol. 44 (4), pp. 405-9.
Abstrakt:	Pulmonary Alveolar Proteinosis is a rare disease with unknown etiology that is due to an abnormal surfactant metabolism. Retrospective analysis of a consecutive series of 68 patients with PAP who were seen at Pulmonology Clinic of Pavlov State Medical University from 1977-2013 was performed. The mean time since first abnormalities were found until diagnosis establishment was 34 months. During that time, most patients were treated with antibiotics, tuberculostatics, glucocorticoids, and immunosupressants. The most effective PAP treatment method was found to be whole lung lavage procedure: 82% of patients showed improvement after its implementation. Delayed diagnosis and incorrect administration of antibiotics, tuberculostatics etc. reduce the probability of a long symptom-free period after lung lavage and spontaneous resolution of the disease. (© 2014 by the Association of Clinical Scientists, Inc.)
Databáze:	MEDLINE
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