Literature DB >> 24906240

[Current care strategies for Duchenne muscular dystrophy].

A Fujak1, G Haaker, J Funk.   

Abstract

BACKGROUND: The medical care of patients with Duchenne muscular dystrophy (DMD) is an interdisciplinary and multifaceted task. The vast majority of those affected show a nearly constant course which is reflected in a corresponding stage-oriented treatment concept. Although there is still no causal therapy available for DMD, the course and in particular the quality of life of patients can be decisively improved by established medical practices. THERAPEUTIC STRATEGIES: The orthopedic problems of DMD patients include contractures of the upper and lower extremities as well as sitting instability due to progressive scoliosis with pelvic imbalance. The orthopedic treatment incorporates conservative measures, such as physiotherapy, provision of orthotic devices and wheelchairs as well as surgery to resolve contractures of the lower extremities and surgical stabilization of the spine. Furthermore, in these patients orthopedic surgeons and trauma surgeons are confronted with the treatment and prophylaxis of fractures induced by osteoporosis. An early onset of glucocorticoid therapy markedly delays the loss of motor abilities. TREATMENT ASPECTS: An important aspect in the care of DMD patients is the timely prophylaxis and treatment of respiratory insufficiency with regular sessions of breathing therapy, learning breathing and coughing techniques and the sufficiently early start of non-invasive mechanically assisted ventilation. Of similar relevance are also the early recognition and cardioprotective treatment of cardiomyopathy.
CONCLUSION: The orthopedic surgeon accompanies the patient and family through all stages of the disease and must be appropriately informed on current management and treatment strategies even outside the limits of the personal field of specialization.

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Year:  2014        PMID: 24906240     DOI: 10.1007/s00132-013-2217-7

Source DB:  PubMed          Journal:  Orthopade        ISSN: 0085-4530            Impact factor:   1.004


  44 in total

1.  Duchenne muscular dystrophy: prolongation of life by noninvasive ventilation and mechanically assisted coughing.

Authors:  Elia Gomez-Merino; John R Bach
Journal:  Am J Phys Med Rehabil       Date:  2002-06       Impact factor: 2.159

Review 2.  Cardiomyopathy of Duchenne muscular dystrophy: current understanding and future directions.

Authors:  Christopher F Spurney
Journal:  Muscle Nerve       Date:  2011-07       Impact factor: 3.217

3.  Platelet function deficiency in Duchenne muscular dystrophy.

Authors:  J Forst; R Forst; H Leithe; N Maurin
Journal:  Neuromuscul Disord       Date:  1998-02       Impact factor: 4.296

4.  Adult life with Duchenne muscular dystrophy: observations among an emerging and unforeseen patient population.

Authors:  Jes Rahbek; Birgit Werge; Anny Madsen; John Marquardt; Birgit Fynbo Steffensen; Joergen Jeppesen
Journal:  Pediatr Rehabil       Date:  2005 Jan-Mar

5.  Contrasting effects of steroids and angiotensin-converting-enzyme inhibitors in a mouse model of dystrophin-deficient cardiomyopathy.

Authors:  Ralf Bauer; Volker Straub; Alison Blain; Kate Bushby; Guy A MacGowan
Journal:  Eur J Heart Fail       Date:  2009-02-20       Impact factor: 15.534

6.  Osteopontin promotes fibrosis in dystrophic mouse muscle by modulating immune cell subsets and intramuscular TGF-beta.

Authors:  Sylvia A Vetrone; Encarnacion Montecino-Rodriguez; Elena Kudryashova; Irina Kramerova; Eric P Hoffman; Scot D Liu; M Carrie Miceli; Melissa J Spencer
Journal:  J Clin Invest       Date:  2009-05-18       Impact factor: 14.808

7.  Abnormal calcium homeostasis in Duchenne muscular dystrophy myotubes contracting in vitro.

Authors:  N Imbert; C Cognard; G Duport; C Guillou; G Raymond
Journal:  Cell Calcium       Date:  1995-09       Impact factor: 6.817

8.  Autonomic function in patients with Duchenne muscular dystrophy.

Authors:  Miki Inoue; Kazuhiro Mori; Yasunobu Hayabuchi; Katsunori Tatara; Shoji Kagami
Journal:  Pediatr Int       Date:  2009-02       Impact factor: 1.524

9.  Re-examination of the electrocardiogram in boys with Duchenne muscular dystrophy and correlation with its dilated cardiomyopathy.

Authors:  Philip T Thrush; Hugh D Allen; Laurence Viollet; Jerry R Mendell
Journal:  Am J Cardiol       Date:  2008-10-30       Impact factor: 2.778

Review 10.  Therapeutic advances in muscular dystrophy.

Authors:  Doris G Leung; Kathryn R Wagner
Journal:  Ann Neurol       Date:  2013-09       Impact factor: 10.422

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  1 in total

1.  Congenital fiber-type disproportion in an ambulatory rehabilitation setting : A case report.

Authors:  Stefano Palma; Mohammad Keilani; Tim Hasenoehrl; Clemens Ambrozy; Sara Dana; Thomas Sycha; Richard Crevenna
Journal:  Wien Med Wochenschr       Date:  2017-07-25
  1 in total

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