Fujak A, Haaker G, Funk J
Orthopädische Klinik, Friedrich-Alexander-Universität Erlangen-Nürnberg, Rathsberger Str. 57, 91054, Erlangen, Deutschland,
Orthopade. 2014 Jul;43(7):636-42. doi: 10.1007/s00132-013-2217-7.
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.
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.
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.
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.
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。
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。
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。
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。