Mannaa Mohannad M, Kalra Maninder, Wong Brenda, Cohen Aliza P, Amin Raouf S
Division of Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, USA.
J Clin Neuromuscul Dis. 2009 Mar;10(3):85-9. doi: 10.1097/CND.0b013e318190310f.
Medical and technological advances over the past 2 decades have resulted in improved patient care for children with spinal muscular atrophy (SMA). The objective of the present study was to describe changes in the life expectancy of pediatric patients with SMA over time and to compare these findings with previously reported survival patterns.
Medical records of all patients diagnosed with SMA over a 16-year period (1989-2005) at Cincinnati Children's Hospital Medical Center were reviewed. Data pertaining to date of birth, type of SMA, medical and surgical interventions, pulmonary complications, and date of death were obtained.
Kaplan-Meier survival analyses showed a significant improvement in survival probabilities in the severest form of SMA.
We found a positive trend in the survival of patients with severe SMA. Although we cannot attribute this trend to any single factor, it is likely that advances in pulmonary care and aggressive nutritional support have played a significant role.
过去20年里医学和技术的进步使脊髓性肌萎缩症（SMA）患儿的护理得到改善。本研究的目的是描述小儿SMA患者的预期寿命随时间的变化，并将这些结果与先前报道的生存模式进行比较。
回顾了辛辛那提儿童医院医疗中心在16年期间（1989 - 2005年）所有诊断为SMA的患者的病历。获取了有关出生日期、SMA类型、医疗和外科干预、肺部并发症及死亡日期的数据。
Kaplan-Meier生存分析显示，最严重形式的SMA患者的生存概率有显著改善。
我们发现重度SMA患者的生存呈积极趋势。虽然我们不能将这一趋势归因于任何单一因素，但肺部护理的进步和积极的营养支持可能发挥了重要作用。
