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Pulmonary Type B Niemann-Pick Disease Successfully Treated with Lung Transplantation
BACKGROUND: Niemann-Pick Disease (NPD) type B is a rare autosomal recessive disease characterised by hepatosplenomegaly and pulmonary disease, highlighted by preserved volumes and diminished diffusion capacity of the lung for carbon monoxide (DLCO) on pulmonary function tests (PFTs). There is no cur...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Hindawi
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6601489/ https://www.ncbi.nlm.nih.gov/pubmed/31316859 http://dx.doi.org/10.1155/2019/9431751 |