title: Pulmonary alveolar lipoproteinosis in a seven-year-old girl. A follow-up over six years.
abstract: Alveolar lipoproteinosis is a rare disease in childhood, and its outcome is considered unfavourable compared to adults. We report on the spontaneous improvement of the disease over a six-year period in a child now 13 years of age. At the time of diagnosis the vital capacity was reduced to 65% of the mean predicted for her height, but deteriorated to 40% during the following year. A bronchoalveolar lavage was performed with small amounts of fluid but it did not immediately reverse the pulmonary alterations. Contrary to expectation, in the following years there was a continuous and gradual increase of the vital capacity to 70% of the mean predicted without specific therapy. This observation underlines the importance of an individual approach to prognosis in this disease and contributes to discussion of therapeutic procedures in the paediatric age group.
authors.affiliation: Children's Hospital, University of Heidelberg, Germany.
authors.forename: K
authors.initials: K
authors.lastname: Paul
authors.forename: K M
authors.initials: KM
authors.lastname: Müller
authors.forename: H C
authors.initials: HC
authors.lastname: Oppermann
authors.forename: W
authors.initials: W
authors.lastname: Nützenadel
mesh_terms: D001992:Bronchoalveolar Lavage Fluid; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D011649:Pulmonary Alveolar Proteinosis / Q000503:physiopathology / Q000628:therapy; D012075:Remission, Spontaneous; D014797:Vital Capacity