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[杜氏肌营养不良症的当前护理策略]

[Current care strategies for Duchenne muscular dystrophy].

作者信息

Fujak A, Haaker G, Funk J

机构信息

Orthopädische Klinik, Friedrich-Alexander-Universität Erlangen-Nürnberg, Rathsberger Str. 57, 91054, Erlangen, Deutschland,

出版信息

Orthopade. 2014 Jul;43(7):636-42. doi: 10.1007/s00132-013-2217-7.

DOI:10.1007/s00132-013-2217-7
PMID:24906240
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.

摘要

背景

杜氏肌营养不良症(DMD)患者的医疗护理是一项跨学科且多方面的任务。绝大多数患者病情几乎持续发展,这反映在相应的分阶段治疗理念中。尽管目前仍没有针对DMD的病因疗法,但既定的医疗实践可显著改善患者的病程,尤其是生活质量。

治疗策略

DMD患者的骨科问题包括上下肢挛缩以及因进行性脊柱侧弯伴骨盆失衡导致的坐姿不稳。骨科治疗包括保守措施,如物理治疗、提供矫形器械和轮椅,以及手术以解决下肢挛缩和脊柱手术固定。此外,在这些患者中,骨科医生和创伤外科医生还面临骨质疏松引起的骨折的治疗和预防。早期开始糖皮质激素治疗可显著延迟运动能力丧失。

治疗方面

DMD患者护理的一个重要方面是通过定期进行呼吸治疗、学习呼吸和咳嗽技巧以及足够早地开始无创机械辅助通气,及时预防和治疗呼吸功能不全。心肌病的早期识别和心脏保护治疗也具有类似的相关性。

结论

骨科医生在疾病的各个阶段陪伴患者及其家人,即使超出个人专业领域的范围,也必须充分了解当前的管理和治疗策略。

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1
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本文引用的文献

1
Therapeutic advances in muscular dystrophy.肌肉萎缩症的治疗进展。
Ann Neurol. 2013 Sep;74(3):404-11. doi: 10.1002/ana.23989.
2
Flexible intramedullary nailing for distal femoral fractures in patients with myopathies.柔性髓内钉治疗肌病患者的股骨远端骨折
J Child Orthop. 2012 Jun;6(2):119-23. doi: 10.1007/s11832-012-0399-x. Epub 2012 Apr 11.
3
Surgical treatment of Duchenne muscular dystrophy patients in Germany: the present situation.德国杜氏肌营养不良症患者的外科治疗:现状
Acta Myol. 2012 May;31(1):21-3.
4
The use of intravenous bisphosphonate therapy to treat vertebral fractures due to osteoporosis among boys with Duchenne muscular dystrophy.静脉注射双磷酸盐疗法治疗杜氏肌营养不良症男性患者骨质疏松性椎体骨折。
Osteoporos Int. 2012 Nov;23(11):2703-11. doi: 10.1007/s00198-012-1911-3.
5
Effects of steroids and angiotensin converting enzyme inhibition on circumferential strain in boys with Duchenne muscular dystrophy: a cross-sectional and longitudinal study utilizing cardiovascular magnetic resonance.利用心血管磁共振研究类固醇和血管紧张素转换酶抑制对杜氏肌营养不良症男孩周向应变的影响:一项横断面和纵向研究。
J Cardiovasc Magn Reson. 2011 Oct 19;13(1):60. doi: 10.1186/1532-429X-13-60.
6
Cardiomyopathy of Duchenne muscular dystrophy: current understanding and future directions.杜氏肌营养不良症性心肌病:当前认识与未来方向。
Muscle Nerve. 2011 Jul;44(1):8-19. doi: 10.1002/mus.22097.
7
Mechanisms inducing low bone density in Duchenne muscular dystrophy in mice and humans.诱导 Duchenne 型肌营养不良症小鼠和人类低骨密度的机制。
J Bone Miner Res. 2011 Aug;26(8):1891-903. doi: 10.1002/jbmr.410.
8
Endocrine aspects of Duchenne muscular dystrophy.杜氏肌营养不良症的内分泌方面
Neuromuscul Disord. 2011 Apr;21(4):298-303. doi: 10.1016/j.nmd.2011.02.006. Epub 2011 Feb 25.
9
Effects of glucocorticoids on growth and bone mineralization.糖皮质激素对生长和骨矿化的影响。
J Pediatr (Rio J). 2011 Jan-Feb;87(1):4-12. doi: 10.2223/JPED.2052. Epub 2011 Jan 13.
10
Patterns of left ventricular remodeling in patients with Duchenne Muscular Dystrophy: a cardiac MRI study of ventricular geometry, global function, and strain.Duchenne 型肌营养不良症患者左心室重构模式:心脏 MRI 对心室几何形状、整体功能和应变的研究。
Int J Cardiovasc Imaging. 2012 Jan;28(1):99-107. doi: 10.1007/s10554-010-9781-2. Epub 2011 Jan 8.