经皮内镜下胃造瘘术在肌萎缩侧索硬化中的应用及中医护理体会

目的探讨经皮内镜下胃造瘘术(Percutaneous Endoscopic Gastrostomy,PEG)在肌萎缩侧索硬化(Amyotrophic Lateral Sclerosis,ALS)患者中的临床应用及护理方法。方法对10例行PEG治疗的ALS患者从情志护理、辨证施膳、胃造瘘衣的使用,出院后生活调护等中医系统护理干预措施,观察其行PEG治疗后的病情及临床转归情况。结果 10例患者手术均成功,未出现与手术相关的严重并发症,PEG术后肠内营养治疗6个月后平均BMI为20.8 kg/m2。随访1年,2例患者死亡,8例存活者营养状态良好。结论行PEG后实施中医系统护理干预措施,提高ALS患者家属的照顾质量,有效地维持患者的营养状态,从而提高患者生活质量。     

经皮内镜下胃造口术在肌萎缩侧索硬化病人中的应用

目的:探讨经皮内镜下胃造口术(PEG)对于肌萎缩侧索硬化(ALS)伴有吞咽困难病人的安全性和有效性.方法:回顾性分析46例行PEG的ALS伴有吞咽困难病人的临床资料,分析PEG置管时间、成功率、并发症及处理方法.结果:本研究纳入了46例病人,男30例,女16例.平均置管时间为(8.8±1.6)min,成功率为100％,未发生置管相关并发症,置管后并发症的发生率为17.39％(8/46),包括造口感染2例,造口管堵塞2例,肺炎2例,造口管近腹壁处折断1例,造口渗漏1例.术后30 d死亡1例,死亡率为2.17％.结论:PEG操作简便、安全、有效、易于护理、并发症少等优点,适合ALS伴有吞咽困难的病人.     

胃镜下经皮胃造瘘的临床应用

1980年非手术经皮内镜下胃造瘘术（percutaneous endoscopic gastrostomy,PEG）被介绍应用于临床,其优点是操作简便、并发症少。近30年来,PEG临床应用的范围不断扩展,越来越受到重视。该项技术已在欧美、日本等国家替代外科胃造瘘，目前PEG已经成为需要长期肠内营养支持患者的首选方法[1]。     

Introducer法经皮胃镜下胃造瘘术的临床应用

经皮内镜下胃造瘘术(percutaneous endoscopic gastrostomy,PEG)是一种通过胃镜介导放置胃造瘘管进行肠内营养且无需外科手术及全身麻醉的胃造瘘术,具有操作简便、快捷、安全、创伤小、便于护理及成功率高的特点.操作仅需在胃镜室及床边麻醉下即可进行,患者易于接受.目前使用广泛的是Pull法和Push法,而采用Introducer法不多,我院2010年6月至2011年6月对16例具有适应证的患者采用Introducer 法行PEG,取得了较好的临床效果,现报道如下.     

经皮内镜下胃造瘘术在上段食管癌患者中的临床应用

目的探讨经皮内镜下胃造瘘术（percutaneous endoscopic gastrostomy,PEG）在上段食管癌患者中的临床应用。方法回顾分析2005年1月～2010年3月在本院行PEG术的25例上段食管癌患者的临床资料。结果所有患者PEG均获成功,术后患者营养状况明显好转。无手术相关死亡病例发生,25例患者中1例术后出现黑便,3例出现造瘘口局部少许渗血,2例出现造瘘管周围皮肤感染,经对症处理后均改善。结论PEG技术操作简单易行、安全,创伤小,并发症少,可避免食管上段癌患者营养状态的恶化,是上段食管癌并恶性梗阻患者行肠内营养支持治疗的可供选择的一种方法。     

经皮内镜胃造瘘术的临床应用

目的:探讨经皮穿刺内镜下胃造瘘术(percutaneous endoscopic gastrostomy,PEG)的临床应用价值以及并发症。方法:2008年5月～2011年5月,因各种原因造成的经口进食困难引起营养不良需长期营养支持的患者10例,在本院内镜中心行PEG,术后给予肠内营养。结果:所有患者成功完成内镜下胃造瘘术,平均耗时20 min。术后跟踪随访,所有患者造瘘管置入后营养迅速恢复,停止静脉补液。其中1例患者经管饲1个月后营养状态改善拔管,改为经口饮食。1例患者发生造瘘管周围皮肤感染,1例出现反流性食管炎,1例术后近期伤口少许渗血,造瘘口周围肉芽组织增生,对症处理后治愈。无消化道出血、腹膜炎等严重并发症。结论:PEG是作为胃肠减压和肠内营养替代鼻饲的一种完善和成熟的微创技术,其技术操作简单易行、安全、有效并发症少,创伤小,医疗费用低。值得临床推广应用。     

高龄危重病患者经皮内镜下胃空肠造瘘术营养支持的研究

目的 评价经皮内镜下胃空肠造瘘术( PEG/J),在高龄危重病患者临床治疗中的应用价值.方法 选择32例住院高龄患者因疾病无法经口饮食者,采用PEG/J前后自身对照的方法,比较其营养水平、肺部感染的发生率及对胃肠功能的影响.结果 32例患者PEG/J后营养状况均得到改善,术前体质量、血红蛋白、血清白蛋白平均数分别为(47.4±3.2)kg、( 86.2±4.0)g/L、(24.6±2.4)g/L,术后3个月明显提高,分别为(55.6±2.5)kg、(102.0±5.4)g/L、( 32.6±3.6) g/L,术后与术前比较,差异有统计学意义(P＜0.05).吸入性肺炎的发生次数减少,平均感染时间缩短,胃肠功能恢复.结论 PEG/J可有效改善高龄危重病患者的营养状况,减少肺部感染的发生率,改善胃肠功能,阻止多器官功能障碍的发生;且可在床边操作,值得老年病房推广.     

脓毒症休克患者血管外肺水的监测及其对预后的预测价值

目的 分析脓毒症休克患者血管外肺水(EVLW)的变化特点,探讨其与脓毒症休克预后的关系.方法 采用回顾性分析的方法,将21例脓毒症休克患者根据最终临床转归分为存活组(10例)与死亡组(11例),观察两组患者的临床特征,利用脉波指示剂连续心排血量(PiCCO)监护仪进行血流动力学监测,测定EVLW,分析其与患者预后的关系.结果 存活组入院第1、2、3天EVLW分别为(12.7±1.8)、(11 3±1.3)、(10.1±1.3)ml/kg,死亡组分别为(14.4±1.0)、(14.6±1.4)、(14.6±1.3)ml/kg,两组比较差异有统计学意义(P＜0.05);存活组随入院时间延长EVLW均明显下降(P＜0.05),死亡组无明显变化(P＞0.05).结论 脓毒症休克患者EVLW均有明显增加;EVLW的动态变化可预测患者的预后.

Abstract：

Objective To analyze the alterative characteristics of the extravascular lung water (EVLW ) in the patients with septic shock and clarify its value on the prognosis of these patients.Methods By the methods of retrospective analysis,according to the ultimate survival,21 patients with septic shock were divided into survivor group (10 cases) and non-survivor group (11 cases).The clinical features of the patients were observed and hemodynamic monitoring was made with PiCCO monitor.The EVLW was measured and the relationship between the EVLW and the prognosis of patients was analyzed.Results On the first,second and third day,EVLW was (12.7 ±1.8),(11.3 ±1.3),(10.1 ±1.3) ml/kg in survivor group,and (14.4 ± 1.0),(14.6 ± 1.4),(14.6 ±1.3) ml/kg in non-survivor group respectively,and there were statistical differences between two groups (P <0.05).However,on the second day after the intensive therapy,EVLW in survivor group dropped significantly(P＜0.05),but the non-survivor group only declined slightly,and compared with the result of the first day,there was no obvious difference (P >0.05).Conclusions The EVLW in the patients with septic shock increases significantly.The dynamic changes of the EVLW may be one of the factors for predicting the prognosis of patients with septic shock.     

经皮内窥镜引导下胃造口术的欧洲肠外肠内营养学会指南解读

经皮内窥镜引导下胃造口术(PEG)是一项简便、高效、安全的置管技术,尤其对不能经口进食患者的营养支持有不可替代的作用,应严格按照适应证、道德标准、禁忌证来选择行PEG的患者.早期开展PEG,能明显改善生活质量.

Abstract：

Percutaneous endoscopic gastrostomy (PEG) is an convenient, effective, and safe technique,and is especially useful for the nutritional support of patients who are unable to befedorally. PEG should be carefully arranged based on indications, ethical criteria, and contraindications. Early PEG can remarkably improve the quality of life.     

Analysis of the influencing factors of embryo quality and pregnancy outcomes in frozen-thawed embryo transfer cycle in polycystic ovary syndrome patients北大核心CSCD

Objective To explore the influencing factors of embryos quality during the cycle of in vitro fertilization/intracytoplasmic sperm injection (IVF/ICSI) and pregnancy outcomes of frozen-thawed embryo transfer (FET) in patients with polycystic ovary syndrome (PCOS). Methods A retrospective case-control study design was used to analyze patients who received IVF/ ICSI treatment at the Reproductive Medicine Center of Tianjin Central Obstetrics and Gynecology Hospital from January 2015 to December 2019, underwent whole embryo cryopreserved and performed the first FET. The 1233 cycles included were divided into control group (n=561) and PCOS group (n=672) according to PCOS diagnosis. The general clinical characteristics, laboratory-related indicators and pregnancy outcomes of patients between the two groups were compared, and the affecting factors of the late miscarriage rate were analyzed by multivariate logistic regression. Results 1) In terms of the general clinical characteristics between the two groups, the differences of duration of infertility [(3.95±2.01) years vs. (4.84±2.91) years, P=0.007], body mass index (BMI) [(21.96±2.52) kg/m2 vs. (23.96±3.50) kg/m2, P<0.001], basal luteinizing hormone [(4.71±2.38) mU/L vs. (8.18±5.40) mU/L, P<0.001], basal estradiol [(45.49±31.80) ng/L vs. (56.67±54.17) ng/L, P=0.032], basal testosterone [(42.80±13.45) ng/L vs. (53.45±38.67) ng/L, P=0.001], gonadortopin initial used dosage [(230.80±54.07) U vs. (192.11±53.79) U, P<0.001] were statistically significant. The endometrium preparation plan in the FET cycle, more PCOS group patients received hormone replacement treatment [64.1% (431/672) vs. 26.6% (149/561)], while more patients in control group received natural cycle transplantation [73.4% (412/561) vs. 35.9% (241/672)], and the differences were statistically significant (all P<0.001). 2) In terms of the laboratory results, the number of oocytes retrieved in PCOS group (23.36±9.53) was higher than that in control group (20.32±8.81, P=0.002). The number of high-quality embryos and the rate of high-quality embryos in PCOS group [2.94±3.13; 33.3% (2016/6048)] were lower than those in control group [4.17±3.65, P=0.034; 46.3% (2339/ 5049), P<0.001], and the differences were statistically significant. 3) In the pregnancy outcomes, the high-quality embryo transfer rate and the biochemical pregnancy rate in control group were higher than those in PCOS group [71.0% (743/1046) vs. 59.3% (761/1284), P<0.001; 7.3% (41/561) vs. 4.5% (30/672), P=0.033], and the late miscarriage rate in PCOS group [10.3% (43/418)] was higher than that in control group [4.3% (16/326), P=0.002]. 4) Logistic regression analysis was performed on the influencing factors of late miscarriage. After correcting the confounding factors, PCOS (OR=2.573, 95% CI=1.270-5.212, P=0.009) and maternal high BMI (OR=1.080, 95% CI=0.991-1.176, P=0.031) were the risk factors for late miscarriage. Conclusion The number of high-quality embryos and the rate of high-quality embryos in PCOS patients were lower than those in non-PCOS patients. PCOS and high BMI were risk factors for late miscarriage in patients. Improving endocrine disorders and weight control in PCOS patients before fertility treatment is of positive significance for improving the pregnancy outcome of patients. © 2022 Chinese Medical Journals Publishing House Co.Ltd. All rights reserved.     

Impact of Percutaneous Endoscopic Gastrostomy (PEG) on the Evolution of Disease in Patients with Amyotrophic Lateral Sclerosis (ALS)

Dysphagia is a highly prevalent symptom in Amyotrophic Lateral Sclerosis (ALS), and the implantation of a percutaneous endoscopic gastrostomy (PEG) is a very frequent event. The aim of this study was to evaluate the influence of PEG implantation on survival and complications in ALS. An interhospital registry of patients with ALS of six hospitals in the Castilla-León region (Spain) was created between January 2015 and December 2017. The data were compared for those in whom a PEG was implanted and those who it was not. A total of 93 patients were analyzed. The mean age of the patients was 64.63 (17.67) years. A total of 38 patients (38.8%) had a PEG implantation. An improvement in the anthropometric parameters was observed among patients who had a PEG from the beginning of nutritional follow-up compared to those who did not, both in BMI (kg/m²) (PEG: 0 months, 22.06; 6 months, 23.04; p < 0.01; NoPEG: 0 months, 24.59–23.87; p > 0.05). Among the deceased patients, 38 (40.4%) those who had an implanted PEG (20 patients (52.6%) had a longer survival time (PEG: 23 (15–35.5) months; NoPEG 11 (4.75–18.5) months; p = 0.01). A PEG showed a survival benefit among ALS patients. Early implantation of a PEG produced a reduction in admissions associated with complications derived from it.     

Percutaneous endoscopic gastrostomy (PEG) placement in the overweight and obese patient

BACKGROUND: Traditionally, percutaneous endoscopic gastrostomy (PEG) placement in the obese patient has been considered a relative contraindication due to the impedance of gastric transillumination and inability to approximate the abdominal and gastric wall. The aim of this study was to determine the overall success rate, morbidity, and PEG-related mortality in overweight and obese patients. METHODS: Three hundred fifty-five consecutive patients were retrospectively evaluated over a 1-year period at the Virginia Commonwealth University Medical Center for postprocedure-related PEG and PEG/jejunostomy (J) complications. One hundred thirty-four patients were considered overweight (body mass index [BMI] > or = 27 kg/m(2)). Of those, 80 patients were found to be obese (BMI > or = 30 kg/m(2)) with a BMI ranging 30-63 kg/m(2). RESULTS: Gastrostomy placement was successful in 130 of 134 (97%) overweight patients (p < .05). The overall procedure-related mortality was 0%. The rate of significant complications in overweight and obese patients remained 0% when compared with those patients with a normal BMI. Out of 355 patients, 14 failed to receive a PEG; 3 of these were obese and 1 was overweight. These 4 procedures were aborted due to a paucity of anatomical landmarks and failure to transilluminate the abdominal wall. CONCLUSIONS: We believe that PEG placement in the overweight and obese patient can be a technically safe procedure according to our success rate of 100%, with a procedure-related mortality rate of 0%. In those overweight and obese patients who require specialized long-term enteral nutrition support, PEG placement should be considered earlier and more frequently.     

Gastrostomies et nutrition entérale lors des maladies neuromusculaires

Neuromuscular diseases (NMD) are disorders of often severe prognosis, and can easily lead to malnutrition. Swallowing disorders are frequent, and the patients can profit from enteral nutrition (EN), for which the way of election is gastrostomy. The aim of this review is to give a progress report on the evolution of NMD care since the 2000s for amyotrophic lateral sclerosis (ALS), the best explored disease, and for the other NMD. For ALS, percutaneous endoscopic gastrostomy (PEG) is the main method used, competed with because of its relative simplicity by radiological inserted gastrostomy (RIG). The indications are swallowing disorders, loss of weight, insufficient feeding, and difficulties of catching the meals. Current studies, not enough methodologically valuable, does not allow to affirm that EN improves survival of the patients nor their quality of life, but it could improve nutritional status. The complications after gastrostomy are aspecific, similar between PEG and RIG but the methods of evaluation are imprecise. The PEG is desirable if the patients forced vital capacity (FVC) is >50%. RIG is needed when FVC is <50%, after PEG failure or if the patients are in bad general condition. Documentation is limited for other NMD, including particularly child diseases and Duchenne muscular dystrophy (DMD). The indications and complications of gastrostomy are close to those of the ALS. EN improves patients nutritional status and, in DMD, quality of life. The families, the patients and the medical teams, often because of an insufficient communication, can be reserved considering nutrition support.     

Efficacy and tolerance of gastrostomy feeding in pediatric forms of neuromuscular diseases

BACKGROUND: Percutaneous endoscopic gastrostomy (PEG) is a simple and reliable method, but there are few data about its use in pediatric forms of neuromuscular diseases (NMD). The aim of this study was to evaluate the nutritional effects and tolerance of gastrostomy feeding in NMD patients. METHODS: Twelve patients with NMD, ranging in age from 1 month to 25.5 years, underwent a gastrostomy placement (PEG, n = 11; surgical gastrostomy, n = 1) between January 1990 and December 2000. Diseases were muscular dystrophies (n = 5), infantile spinal muscular atrophies (n = 3), congenital myopathies (n = 3), and polyradiculoneuritis (n = 1). Height-for-age (Z(H/A)), weight-for-age (Z(W/A)), and weight-for-height (Z(W/H)) z scores were assessed at birth, at the time of gastrostomy placement, after a 6-month and 1-year follow-up, and at the end of follow-up. Complications that occurred during the gastrostomy feeding period were recorded. Comparisons of z scores at the different times were performed with repeated-measures analyses of variance. RESULTS: Z(W/A) (p < .05) and Z(W/H) (p < .001) improved in the whole group after gastrostomy. For the 10 patients for whom follow-up was more than 1 year, Z(W/A) (p < .03) and Z(W/H) (p < .001) increased from the time of gastrostomy to the end of follow-up. Among 5 patients who had a gastroesophageal reflux diagnosed before gastrostomy placement, 3 had at least 1 episode of pneumonia and 2 died of respiratory distress caused by the worsening of NMD. No other major complication was encountered. CONCLUSIONS: Gastrostomy feeding is well tolerated and results in an improvement of nutritional indices in NMD patients. Special care should be taken in patients with preexisting gastroesophageal reflux.     

Long-term management of percutaneous endoscopic gastrostomy by a nutritional support team

BACKGROUND/AIMS: Percutaneous Endoscopic Gastrostomy (PEG) has become a commonly-performed procedure, to provide enteral nutrition for patients who are unable to eat. The aims of this study were to evaluate the long term efficacy, morbidity and mortality of percutaneous endoscopic gastrostomy (PEG). MATERIAL AND METHODS: We analysed 144 patients who underwent a PEG procedure. Survival curves were done with the Kaplan-Meier method. The indication was long-term enteral nutrition in patients unable to maintain adequate nutrition by mouth. RESULTS: The procedure was successful in all but one case. Mean age was 62 (18-85) years, 89 (62%) males. Seven patients recovered from their primary disease and gastrostomy tube was removed. Mean follow-up was 7.3+/-10.8 (1--66) months. Survival rates at 30 days, 1 year and 3 years following gastrostomy were 82%, 36% and 14%, respectively. Survival curves were better in females (P<0.0001). In almost all cases, patients were fed with current home-prepared food, and were ambulatory. There were no differences in survival curves according to the nutritional status. CONCLUSIONS: There were few procedure-related complications, but a high short-term mortality, probably related with the underlying disease. The use of home-prepared food through the gastrostomy was very well tolerated, and should be encouraged.     

Laparoscopic‐Assisted Percutaneous Endoscopic Gastrostomy

Background/Purpose: Percutaneous endoscopic gastrostomy (PEG) is a simplified catheter placement procedure for alimentation. Although the endoscopic approach to gastrostomy tube placement is a safe and well‐tolerated procedure in most patients, the PEG procedure is difficult in elderly patients disabled since childhood who have severe scoliosis and malpositioning of the stomach. We describe a simple and effective laparoscopic‐assisted PEG (LAPEG) technique that can be used for catheter placement in severely disabled patients. Methods: Thirteen severely disabled patients aged 14–57 years underwent gastrostomy tube placement with the LAPEG technique. After general anesthesia was achieved, an endoscope was placed into the stomach. Then, a 5‐mm camera port was inserted at the umbilicus, and a 3‐mm working port was inserted to identify and lift the optimal site for gastrostomy tube placement. After the 4‐point fixation of the stomach, the 20‐Fr gastrostomy tube was placed under endoscopic and laparoscopic observation. Results: All patients tolerated the procedure well, and there were no major complications. The procedure was successful, and all patients could feed via the tube. Conclusions: Elderly disabled patients who have been bedridden since childhood often have severe scoliosis and malpositioning of the stomach. Our LAPEG procedure is effective, well tolerated, and safe for gastrostomy tube placement in such elderly patients.     

经皮内镜下胃/空肠造口术并发症的预防与治疗

目的:重点探讨经皮内镜下胃肠造口术并发症的预防及治疗. 方法:对2002年10月至2003年12月间85例恶性肿瘤病人共行88例次经皮内镜下胃造口(PEG)和经皮内镜下空肠造口(PEJ),回顾性统计并发症的发生情况.所有PEG/J均采用拉出法. 结果:85例病人PEG/J术后无操作相关死亡,无严重并发症,但微小并发症发生率为8.2%(7/85).7例病人发生8次微小并发症,分别为切口感染2例、导管断裂2例、导管尖端移位2例、导管缠绕1例、导管渗漏1例,均通过非手术治疗后治愈. 结论:经皮内镜下胃/空肠造口术操作简便、安全,加强围手术期的处理是控制并发症的关键.     

Malnutrition and Clinical Outcome of 234 Head and Neck Cancer Patients who Underwent Percutaneous Endoscopic Gastrostomy

Head and neck cancer (HNC) patients who underwent percutaneous endoscopic gastrostomy (PEG) present malnutrition risk and speech impairments. Their assessment relies on objective anthropometric/laboratory data. Focusing on HNC PEG patients, our aims were to evaluate: 1) outcome; 2) nutritional status when the patients underwent PEG; and 3) association of nutritional status/outcome, creating a survival predictive model. We evaluated the outcome based on NRS 2002, dietary assessment, body mass index (BMI), mid-upper arm circumference (MUAC), triceps skinfold thickness (TSF), mid-arm muscle circumference (MAMC), albumin, transferrin, and cholesterol on the day of gastrostomy. Using BMI, TSF, MAMC, and laboratory data, a survival predictive model was created. Of the 234 patients (cancer stages III–IV), 149 died, 33 were still PEG-fed, and 36 resumed oral intake (NRS-2002≥3, caloric needs <50% in all). BMI was 12.7–43. 189, 197, and 168 patients displayed, respectively, low MUAC, TSF, and MAMC. 91, 155, and 119 patients displayed low albumin, transferrin, and cholesterol. Albumin, cholesterol, and transferrin were strongly associated with the outcome. A predictive model was created, discriminating between short-term survivors (<4 months) and long-term survivors. HNC patients were malnourished. Using anthropometric/laboratory parameters, a predictive model provides discrimination between patients surviving PEG for <4 months and long-term survivors. Teams taking care of PEG patients may provide special support to potential short-term survivors.     

Safety of unsedated PEG placement using transoral ultrathin endoscopy in patients with amyotrophic lateral sclerosis

Background and aims: In patients with amyotrophic lateral sclerosis (ALS), percutaneous endoscopic gastrostomy (PEG) placement under sedation often causes apnea or hypoventilation. The aim of the present study was to assess whether unsedated PEG placement in ALS patients using ultrathin endoscopy (UTE) via the transoral route can improve safety.

Methods: Between 2003 and 2013, PEG placement was identified and reviewed in 45 patients with ALS. PEG was performed in 14 patients using transoral UTE without sedation (UTE group), 17 patients using conventional normal-diameter esophagogastroduodenoscopy (C-EGD) without sedation (unsedated C-EGD group) and 14 patients using C-EGD with sedation (sedated C-EGD group). We compared the clinical features, cardiopulmonary data before and during PEG placement, and complications related to PEG placement among the three groups.

Results: There were no significant differences in age, male/female ratio, forced vital capacity, blood pressure, oxygen saturation before and during PEG, or major complications among the three groups. No minor complications were observed in the UTE group, whereas apnea and/or hypoventilation were observed in the sedated C-EGD group and aspiration pneumonia was observed in the unsedated C-EGD group.

Conclusions: Unsedated PEG placement using transoral UTE in ALS patients is a safe method.     

Amyotrophic lateral sclerosis: mechanical ventilation--or not right?

In two patients (men aged 64 and 62 years, respectively) with amyotrophic lateral sclerosis (ALS) tracheostomy ventilation was initiated following (imminent) acute respiratory failure; in one patient this was done because advance directives were lacking, while in the other non-invasive ventilation (NIPPV) was no longer an option. A third ALS patient, a woman aged 36 years, already had chronic respiratory failure when she presented at the local centre for home mechanical ventilation. As a result, the placement of a percutaneous endoscopic gastrostomy (PEG) was impossible. Instead, she had to use a nasopharyngeal feeding tube, which diminished the effectiveness of her NIPPV. The fourth patient, a man aged 60 years, was referred in good time and hence had sufficient time to consider domiciliary ventilation. Following the placement of a PEG he started NIPPV electively. In patients with ALS, domiciliary ventilation should be discussed early in the course of the disease. Advance directives with regard to domiciliary ventilation are important, not only to avoid undesired (invasive) ventilation, but also with respect to the placement of a PEG and the appropriate use of oxygen and morphine.