Literature DB >> 7955712

[Spontaneous pneumothorax in Duchenne muscular dystrophy].

T Yamamoto1, M Kawai.   

Abstract

We retrospectively studied an incidence and clinical features of spontaneous pneumothorax (SPT) in Duchenne muscular Dystrophy (DMD) from chest roentgenograms and clinical records of our 50 inpatients, comparing those in severely mentally and physically disabled patients. Nine out of 50 (18%) DMD patients experienced SPT, and their chest roentgenograms showed no evidence of underlying pulmonary disease such as bulla, bleb, pneumonia. They were all asymptomatic. Clinical course was well, however, relapse occurred in 4 cases. The onset age was 20 or more in 7 patients (78%), and the incidence of SPT in patients aged 20 or more was 31%, which was 7.4 times higher than that of younger patients. Body weight at the onset of SPT was all below 30 kg, tended to be lower than that of SPT negative cases. Some patients experienced SPT after having lost their weight. SPT occurred much more frequently on the right side (right only, 7 cases; left only, 0; bilateral, 2 cases). Thoracic deformity and artificial ventilation were not major risk factors. We examined chest roentgenograms of 41 severely mentally and physically disabled inpatients, age, sex, and body weight matched and found no SPT. We conclude that SPT is frequent and asymptomatic in DMD and the major risk factors of SPT are age (above 20 years old) and body weight (below 30 kg), while thoracic deformity, and artificial ventilation are not essential.

Entities:  

Mesh:

Year:  1994        PMID: 7955712

Source DB:  PubMed          Journal:  Rinsho Shinkeigaku        ISSN: 0009-918X


  3 in total

Review 1.  Cardiopulmonary support in duchenne muscular dystrophy.

Authors:  Josef Finsterer
Journal:  Lung       Date:  2006 Jul-Aug       Impact factor: 2.584

2.  Pneumothoraces in collagen VI-related dystrophy: a case series and recommendations for management.

Authors:  Kristin L Fraser; Scott Wong; A Reghan Foley; Sameer Chhibber; Carsten G Bönnemann; Daniel J Lesser; Carla Grosmann; Anne Rutkowski
Journal:  ERJ Open Res       Date:  2017-06-23

3.  X-linked myotubular myopathy and recurrent spontaneous pneumothorax: A new phenotype?

Authors:  Per-Ole Carstens; Eva Maria Christina Schwaibold; Katharina Schregel; Carolin D Obermaier; Arne Wrede; Sabrina Zechel; Silke Pauli; Jens Schmidt
Journal:  Neurol Genet       Date:  2019-04-26
  3 in total

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