Supine volume drop and diaphragmatic function in adults with Pompe disease.

نویسندگان

  • Hélène Prigent
  • David Orlikowski
  • Pascal Laforêt
  • Nadège Letilly
  • Line Falaize
  • Nadine Pellegrini
  • Djillali Annane
  • Jean-Claude Raphael
  • Frédéric Lofaso
چکیده

The onset of the adult form of Pompe disease, also known as acid maltase deficiency or glycogen storage disease type II, is a slow progressive muscular disorder in which little correlation exists between respiratory function and locomotor function [1]. The greater degree of respiratory dysfunction, compared with mobility loss, is mainly ascribable to the predominant and progressive involvement of the diaphragm [1].

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عنوان ژورنال:
  • The European respiratory journal

دوره 39 6  شماره 

صفحات  -

تاریخ انتشار 2012