Forst R, Krönchen-Kaufmann A, Forst J
Orthopädische Klinik, RTWH Aachen.
Klin Padiatr. 1991 Jan-Feb;203(1):24-7. doi: 10.1055/s-2007-1025394.
So far there is no causal treatment for Duchenne muscular dystrophy up to now, it has been proven, however, that its course can be considerably improved by an early contracture-prophylactic operation of both lower limbs--mostly between age 4 and 6 years--as well as by a surgical stabilization of the spine before any progressive scoliosis appears, that is at the very beginning of the wheel-chair stage: Walking and standing ability can be prolonged for several years and a significant scoliosis can be avoided. A decisive prolongation of life can be achieved by treating the fatal respiratory insufficiency with timely started mechanical ventilation. Our first experiences have shown, that there is no proven justification for a reserved attitude against early lower limb surgery in view of modern anaesthesia. Malignant hyperthermia-reaction and hyperkalaemia are the known anaesthetic rise factors of operations in Duchenne muscular dystrophy. However, we could exclude virtually these rises by choosing the right anaesthesia and by a comprehensive monitoring routine. If need be, malignant hyperthermia can be treated effectively by using the obligatory antidote (Dantrolene). In view of an expected causal treatment in future, early detection of Duchenne muscular dystrophy by newborn screening ("CK-Test") as well as a comprehensive stage-depending treatment programme (early surgery/mechanical ventilation) are nowadays of outstanding importance.
到目前为止,杜氏肌营养不良症尚无病因治疗方法,但已证实,通过对双下肢进行早期挛缩预防性手术(大多在4至6岁之间)以及在脊柱出现任何进行性脊柱侧弯之前(即在轮椅阶段刚开始时)进行脊柱手术稳定,可以显著改善其病程:行走和站立能力可延长数年,且可避免严重脊柱侧弯。通过及时开始机械通气治疗致命的呼吸功能不全,可实现寿命的决定性延长。我们的初步经验表明,鉴于现代麻醉技术,没有确凿的理由对早期下肢手术持保留态度。恶性高热反应和高钾血症是杜氏肌营养不良症手术已知的麻醉风险因素。然而,我们通过选择合适的麻醉方法和全面的监测程序,几乎可以排除这些风险。如有必要,可使用特效解毒剂(丹曲林)有效治疗恶性高热。鉴于未来有望进行病因治疗,如今通过新生儿筛查(“CK检测”)早期发现杜氏肌营养不良症以及制定全面的分阶段治疗方案(早期手术/机械通气)至关重要。