Pulmonary Involvement in Niemann-Pick Disease: A State-of-the-Art Review.
von Ranke FM, Pereira Freitas HM, Mançano AD, Rodrigues RS, Hochhegger B, Escuissato D, Araujo Neto CA, da Silva TK, Marchiori E.
von Ranke FM, et al.
Lung. 2016 Aug;194(4):511-8. doi: 10.1007/s00408-016-9893-0. Epub 2016 May 10.
Lung. 2016.
PMID: 27164983
Review.
Progression of respiratory disease is slow, but inexorable, due to the accumulation of Niemann-Pick cells in the alveolar septa, bronchial walls, and pleura, potentially leading to a progressively worsening restrictive pattern on pulmonary function testing. ...The most com …
Progression of respiratory disease is slow, but inexorable, due to the accumulation of Niemann-Pick cells in the alveolar septa, bron …