Rall Susanne, Grimm Tiemo
Abteilung für Medizinische Genetik, Universität Tübingen, Germany.
Acta Myol. 2012 Oct;31(2):117-20.
To determine the survival in a population of German patients with Duchenne muscular dystrophy.
Information about 94 patients born between 1970 and 1980 was obtained by telephone interviews and questionnaires. In addition to age of death or actual age during the investigation, data concerning clinical course and medical interventions were collected.
67 patients with molecularly confirmed diagnoses had a median survival of 24.0 years. Patients without molecular confirmation (clinical diagnosis only) had a chance of 67 % to reach that age. Grouping of our patient cohort according to the year of death (before and after 2000), ventilation was recognized as main intervention affecting survival with ventilated reaching a median survival of 27.0 years. For those without ventilation it was 19.0 years.
our study provides survival data for a cohort of DMD patients in Germany stratified by year of death. Median survival was 24.0 years in patients confirmed by molecular testing. Ventilated patients had a median survival of 27 years. We consider this piece of information helpful in the medical care of DMD patients.
确定德国杜氏肌营养不良患者群体的生存率。
通过电话访谈和问卷调查获取了94例出生于1970年至1980年之间患者的信息。除了死亡年龄或调查期间的实际年龄外，还收集了有关临床病程和医疗干预的数据。
67例经分子确诊的患者中位生存期为24.0岁。未经分子确诊（仅临床诊断）的患者达到该年龄的几率为67%。根据死亡年份（2000年之前和之后）对我们的患者队列进行分组，通气被认为是影响生存的主要干预措施，接受通气治疗的患者中位生存期为27.0岁。未接受通气治疗的患者为19.0岁。
我们的研究提供了按死亡年份分层的德国DMD患者队列的生存数据。经分子检测确诊的患者中位生存期为24.0岁。接受通气治疗的患者中位生存期为27岁。我们认为这一信息对DMD患者的医疗护理有帮助。
