Prior-Sánchez Inmaculada, Herrera-Martínez Aura Dulcinea, Tenorio Jiménez Carmen, Molina Puerta María José, Calañas Continente Alfonso Jesús, Manzano García Gregorio, Gálvez Moreno María Ángeles
UGC Endocrinología y Nutrición. Hospital Universitario Reina Sofía, Córdoba. España..
Nutr Hosp. 2014 Dec 1;30(6):1289-94. doi: 10.3305/nh.2014.30.6.7808.
Amyotrophic Lateral Sclerosis (ALS) is a degenerative disorder that affects the pyramidal tract, producing progressive motor dysfunctions leading to paralysis. These patients can present with dysphagia, requiring nutritional support with a nasogastric tube or Percutaneous Endoscopic Gastrostomy (PEG). PEG is associated with increased survival rates. However, the timing of PEG placement remains a significant issue for clinicians.
To analyse the characteristics of ALS patients at the moment of PEG placement and their progression.
Descriptive retrospective study including patients diagnosed with ALS and PEG who were assessed during the 2005-2014 period in our hospital. Nutritional parameters and respiratory function were assessed for all patients, as well as their progression. The data was analysed using SPSS15.
37 patients were included (56.8% men, 43.2% women) with an average age of 60 at diagnosis, and an average age of 63.1 at PEG placement. 48.6% started with spinal affection and 51.4%, with bulbar affection. 43.2% of the patients received oral nutritional supplements prior to PEG placement for a mean period of 11.3 months. The mean forced vital capacity at diagnosis was 65.45±13.67%, with a negative progression up to 39.47±14.69% at the moment of PEG placement. 86.5% of patients required non-invasive positive-pressure ventilation. 86.5% presented with dysphagia, 64.9% with weight loss > 5-10% from their usual weight, 8.1% with low Body Mass Index, 27% with malnutrition and 73% with aworsened breathing function; therefore, 100% met the criteria for PEG placement according to our protocol. The period on enteral feeding was extended for 10.1 months with a mortality of 50% during the first 6 months from PEG placement.
There is evidence of a 3-year delay between diagnosis and PEG placement, with a survival rate of 50% at 6 months from PEG insertion. Further studies are required to establish whether an earlier placement might increase survival rates.
肌萎缩侧索硬化症（ALS）是一种退行性疾病，会影响锥体束，导致进行性运动功能障碍并最终发展为瘫痪。这些患者可能会出现吞咽困难，需要通过鼻胃管或经皮内镜下胃造口术（PEG）进行营养支持。PEG与生存率的提高相关。然而，PEG置入的时机对于临床医生来说仍然是一个重要问题。
分析PEG置入时ALS患者的特征及其病情进展。
描述性回顾性研究，纳入2005年至2014年期间在我院接受评估的诊断为ALS且进行了PEG的患者。对所有患者的营养参数、呼吸功能及其病情进展进行评估。使用SPSS15对数据进行分析。
共纳入37例患者（男性占56.8%，女性占43.2%），诊断时平均年龄为60岁，PEG置入时平均年龄为63.1岁。48.6%的患者起病于脊髓受累，51.4%起病于延髓受累。43.2%的患者在PEG置入前接受了口服营养补充剂，平均时间为11.3个月。诊断时平均用力肺活量为65.45±13.67%，到PEG置入时呈负向进展，降至至39.47±14.69%。86.5%的患者需要无创正压通气。86.5%的患者存在吞咽困难，64.9%的患者体重较平常体重减轻>5-10%，8.1%的患者体重指数较低，27%的患者存在营养不良，73%的患者呼吸功能恶化；因此，根据我们的方案，100%的患者符合PEG置入标准。肠内喂养期延长了10.1个月，PEG置入后头6个月内死亡率为50%。
有证据表明从诊断到PEG置入存在3年延迟，PEG置入后6个月生存率为50%。需要进一步研究以确定更早置入是否可能提高生存率。
