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Non-invasive ventilatory support (NVS) is a technique used to reduce respiratory work in neuromuscular diseases, preventing the progression of respiratory failure. NVS is usually administered via a nasal or an oronasal mask, causing discomfort, especially in patients ventilated for more than 16 h/day. Intermittent abdominal pressure ventilation (IAPV) differs completely from conventional NVS and consists of a portable ventilator and a corset with Velcro closures as the interface. In our study, the practicability and efficacy of IAPV were studied in three Italian centers monitoring 28 neuromuscular patients using IAPV who were then retrospectively analyzed. The primary outcomes were an improvement in hypoxemia and the normalization of hypercapnia, and the secondary outcome was an improvement in quality of life. Data were collected at baseline (T0) and after two hours of ventilation (T1), with follow-ups at three months (T2) and six months (T3). Statistical significance was found for PaCO2 over time (F (2.42) = 7.63, p = 0.001) and PaO2 (W = 0.539, p = 0.033). The time of NVS usage also significantly affected the quality of life (F (2.14) = 6.90, p = 0.010), as seen when comparing T0 and T3. As an alternative ventilation method, IAPV is still relevant today and could become a key part of daytime support, especially for patients who do not tolerate standard daytime NVS with an oral interface.

Sleep-disordered breathing (SDB) is a group of sleep-related breathing disorders which includes obstructive sleep apnoea (OSA), central sleep apnoea (CSA), and obesity hypoventilation syndrome (OHS). OHS is characterized by a combination of obesity, daytime hypercapnia and hypoxemia, and sleep-disordered breathing without other known hypoventilation causes, such as severe obstructive or restrictive parenchymal lung disease, kyphoscoliosis, severe hypothyroidism, neuromuscular disease, or congenital central hypoventilation syndrome.490 potentially eligible references were identified ; of these, 462 abstracts or full texts were excluded because they did not fulfil inclusion criteria. We reviewed the full text of the remaining 38 papers which fulfilled the inclusion criteria.The role of gender in SDB and particularly in OHS is not well known; in general, the diseases are under-recognized in women and only a few studies have reported the impact of gender on clinical presentation and treatment outcome. On the other hand, there is often a delay in diagnosing these diseases in women as compared to men; therefore, they are often more advanced when diagnosed in women.Better understanding and clinical awareness of the higher OHS prevalence in postmenopausal women may lead to earlier diagnosis and a more timely and appropriate treatment. Further studies are needed to assess the prevalence of OHS in women, the effect of menopause on OHS, and the increased risk of OHS, which will hopefully lead to optimizing OHS patient care.

High-flow oxygen therapy (HFOT) improves gas exchange and dead space washout and reduces the level of work required for breathing. This study aimed to evaluate pulmonary rehabilitation (PR) combined with HFOT in COPD patients treated with nocturnal non-invasive ventilation (NIV) and long-term oxygen therapy (LTOT). In particular, we sought to discover whether the addition of HFOT during exercise training could improve patients’ performance, mainly with regard to their Six-Minute Walking Test (6MWT) outcomes, and reduce the exacerbation rates, periods of rehospitalization or need to resort to unscheduled visits. Thirty-one COPD subjects (13 female) who used nocturnal NIV were included in a randomized controlled trial and allocated to one of two groups: the experimental group (EG), with 15 subjects, subjected to PR with HFOT; and the control group (CG), with 16 subjects, subjected to PR without HFOT. The primary outcome of the study was the observation of changes in the 6MWT. The secondary outcome of the study was related to the rate of exacerbation and hospitalization. Data were collected at baseline and after one, two and three cycles of cycle-ergometer exercise training performed in 20 supervised sessions of 40 min thrice per week, with a washout period of 3 months between each rehabilitation cycle. Statistical significance was not found for the 6MWT distance (W = 0.974; p = 0.672) at the last follow-up, but statistical significance was found for the Borg scale in regard to dyspnea (W = 2.50; p < 0.001) and fatigue (W = 2.00; p < 0.001). HFOT may offer a positive option for dyspnea-affected COPD patients in the context of LTOT and nocturnal NIV.

BACKGROUND: Early chest physiotherapy is recommended for patients with post-COVID syndrome to improve dyspnea, relieve anxiety, minimize disability, preserve lung function and improve quality of life. However, there is still no consensus on the best treatments to manage respiratory symptoms. We aimed to test a method based on a guided in/expiratory (I/E) modulation to treat the lung inhomogeneity. METHODS: Twenty patients with post-COVID syndrome and mild-to-moderate obstructive syndrome performed 3 15-min sessions/day using the I/E mode of the T-PEP®4 device, for 15 consecutive days. Lung function parameters, dyspnea and quality of life scores, as well as exercise capacity were assessed before and after treatment. RESULTS: All patients concluded the treatment and showed significant improvements in symptoms (chest pain during deep inspiration, chest tightness, inability to yawn, fatigue during activities of daily living [ADL], desaturation ≥4% during ADL) and in health status (BCSS -1.75, p=0.0003; CAT -5.2, p=0.0001). Lung function (FVC +10.9%, p=0.0002; FEV1 +8%, p=0.0001) and respiratory muscle strength (MIP +13.8%, p

Patients with obstructive sleep apnoea (OSA) have a higher risk of developing vascular diseases. In this study, we evaluated the clinical profile of patients with OSA who develop Retinal Vein Occlusion (RVO) compared with a population of OSA patients without RVO.We analysed patients with OSA diagnosed with RVO (21 cases; mean of age 61 (12) yrs. range 44-87 yrs. 67% men), belonging to a large cohort of people with long-term follow-up for RVO (up to 12 years). We compared them with 21 patients with OSA, without RVO, matched by age and gender, selected from the Sleep Unit Registry (control group).There were no differences in the prevalence of arterial hypertension (AHT) or Diabetes mellitus (DM), but the RVO patients presented a higher diastolic blood pressure compared to controls (87.6±12.6 mmHg vs. 77.9±10.1 mm Hg respectively). The polygraphic parameters were similar in both groups. The Apnoea-Hypopnoea Index (IHA) similar in both groups (30.4 ±20.9 RVO vs. 33.7 ± 22.1 controls). In addition, RVO patients had a less favourable lipid profile, with higher total cholesterol (218±52 mg/dL vs. 179±41 mg/dL), higher LDL cholesterol (139±47 mg/dL vs. 107±32 mg/dL) and higher atherogenic indices: LDL/HDL (2.78 ± 0.95 RVO vs. 2.03±0.67 controls) and total cholesterol / HDL (4.37± 1.08 vs. 3.45 ± 0.84). Among the cases, 81% had peripheral RVO (superior temporal branch in 20 out of 21 cases) and 19% had central RVO. 62% of the cases received intravitreal antiangiogenic therapy and dexamethasone implants and 33% received argon laser photocoagulation.Poor control of cardiovascular risk factors, particularly dyslipidaemias, in patients with OSA may lead to the development of this ocular complication.

Background Pneumomediastinum (PNM) can develop as a severe complication of severe COVID 19 and may be correlated with greater morbidity and mortality. PNM is a rarely reported complication in COVID-19 patients and usually associated with endotracheal intubation. Methods Our aim is to describe the characteristics of patients with PNM in twenty-one patients with COVID-19 related pneumonia and acute respiratory failure in a retrospective case series. Results Twenty-one patients were diagnosed, two were treated with high flow nasal cannula, five with non invasive ventilation and four with invasive mechanical ventilation. In four cases PNM was massive PNM was often associated to subcuatneous emphysema; more rarely associated also with pneumothorax. Conservative management was the most used therapeutic strategy. Conclusions PNM should is s serious but not extremely rare complication of severe forms of pulmonary involvement of COVID 19. The clinician should consider this rare complication; moreover, we advise careful attention when clinicians start mechanical ventilation.

Background: In obstructive sleep apnoea (OSA), treatment with mandibular advancement devices (MADs) reduces patients’ Apnoea–Hypopnoea index (AHI) scores and improves their sleepiness and quality of life. MADs are non-invasive alternatives for patients who cannot tolerate traditional continuous positive airway pressure (CPAP) therapy. The variability of responses to these devices makes it necessary to search for predictors of success. The aim of our study was to evaluate the presence of mandibular torus as a predictor of MAD efficacy in OSA and to identify other potential cephalometric factors that could influence the response to treatment. Methods: This was a retrospective cohort study. The study included 103 patients diagnosed of OSA who met the criteria for initiation of treatment with MAD. Structural variables were collected (cephalometric and the presence or absence of mandibular torus). Statistical analysis was performed to evaluate the existence of predictive factors for the efficacy of MADs. Results: A total of 103 patients who were consecutively referred for treatment with MAD were included (89.3% men); the mean age of the participants was 46.3 years, and the mean AHI before MAD was 31.4 (SD 16.2) and post- MAD 11.3 (SD 9.2). Thirty-three percent of patients had mandibular torus. Torus was associated with a better response (odds ratio (OR) = 2.854 (p = 0.035)) after adjustment for sex, age, body mass index (BMI; kg/m2), the angle formed by the occlusal plane to the sella–nasion plane (OCC plane to SN), overinjection, and smoking. No cephalometric predictors of efficacy were found that were predictive of MAD treatment success. Conclusions: The presence of a mandibular torus practically triples the probability of MAD success. This is the simplest examination with the greatest benefits in terms of the efficacy of MAD treatment for OSA.

he standard treatment for patients with neuromuscular respiratory failure is non-invasive ventilation (NIV) as non-invasive ventilation support-setting (NVS). NVS is administered through a nasal or face mask and/or mouthpiece with the potential to cause nasal ulcers, discomfort, and/or aesthetic issues, resulting in poor compliance. We reported the observation of a 45-year-old woman with limb-girdle muscular dystrophy (LGMD), secondary to Dysferlin deficiency, who was on NVS since 2017 for nocturnal hypoventilation. In 2018, despite nocturnal ventilation, due to weight gain and daytime hypoventilation, a nasal mask was introduced. We initiated daytime intermittent abdominal pressure ventilation (IAPV) to mitigate cosmetic problems, improving in pO2 and decreasing in pCO2 versus baseline (52>84 mmHg, 46>33 mmHg respectively) at 6 (85 mmHg, 42 mmHg) and 18 months (93 mmHg, 38 mmHg), respectively. IAPV was effective, safe, and well-tolerated in our patients who did not tolerate standard daytime NVS with the known interface.

COVID-19 has high mortality rate mainly stemming from acute respiratory distress leading to respiratory failure (ARF). Aim of the study was to evaluate the management of severe ARF due to COVID-19 pneumonia using noninvasive ventilatory support (NIVS), studying safety and effectiveness of NIVS.This is a retrospective, multicenter study. Primary outcomes were NIVS failure with intubation rate and hospital mortality. Secondary outcomes were hospital stay and factors related to NIVS failure and mortality. These outcomes were compared with patients intubated and admitted to ICU.One hundred sixty-two patients were hospitalized because of severe respiratory failure (PaOinf2/inf/FiOinf2/infratio250). One hundred thirty-eight patients were admitted to Respiratory Intermediate Care Unit (RICU) for a NIVS trial. One hundred patients were treated successfully with NIVS (74.5%); 38 failed NIVS trial (27.5%). In-hospital mortality was 23.18% in RICU group and 30.55% in ICU group. Patients with NIVS failure were older, had a lower number of lymphocytes, a higher IL-6, lower PaOinf2/inf, PaC Oinf2/inf, PaOinf2/inf/FiOinf2/infratio, higher respiratory rate (RR) and heart rate at admission and lower PaO2, and PaOinf2/inf/FiOinf2/infratio and higher RR after 1-6 hours. Multivariate analysis identified higher age, C-reactive protein as well as RR after 1-6 hours and PaOinf2/inf/FiOinf2/infratio after 1-6 hours as an independent predictor mortality.NIVS is a safe and effective strategy in the treatment of severe ARF due to COVID-19 related pneumonia, that reduces mortality and length of hospital stay in the carefully selected patients.

Background Amyotrophic lateral sclerosis (ALS) is an incurable neurodegenerative disease affecting upper and lower motor neurons and resulting in progressive skeletal muscle weakness. Weak cough and difficulty in clearing secretions are often the cause of pulmonary infections and acute respiratory failure. Cough assistance is commonly used to provide support in coughing for patients with ALS. Methods This was a preliminary parallel randomized study comparing two cough-assist devices: one utilizing mechanical insufflation/exsufflation (MI/E) and expiratory flow accelerator (EFA) technology, the other utilizing only MI/E technology. The aim was to compare the effectiveness, safety and acceptability of the two devices. Thirty patients with ALS and similar severity and functional scale were enrolled. The primary outcome was the change in respiratory function, respiratory muscle function, gas exchange, and peak cough expiratory flow as an indicator of cough efficacy. Secondary outcomes were the number of exacerbations at 1, 6 and 12 months of treatment, and the patient-perceived comfort/distress related to the interventions together with the perceived efficacy of cough. Results 30 subjects were recruited and randomized into the two groups (1:1 ratio). Primary outcomes: Respiratory function parameters decreased over time in both groups, but significantly less in the Kalos group, as did the respiratory muscle strength parameters and peak cough flows. Gas exchanges decreased over time in both groups with no clinically relevant differences between groups. Secondary outcomes there were no significant differences between groups regarding the number of exacerbations over time. No adverse events were reported. All participants, in both groups, reported a similar increase in perceived cough efficacy and there was no significant difference in comfort and distress between the two treatments. Conclusions The cough-assist device with EFA technology performed better than a traditional MI/E device in ALS patients regarding respiratory function and cough efficacy, although number of exacerbations and acceptability of the two devices was similar. Following these promising preliminary results, further investigation is required in a larger cohort to confirm the superiority of EFA technology associated with a MI/E device.

Introduction Pneumonia is both the most common type of lower respiratory tract infection and a major cause of morbidity and mortality worldwide. The COVID-19 pandemic caused by the SARS-CoV-2 raised an extremely serious concern, because its most frequent clinical presentation was pneumonia. Features such as sex play an active role in the incidence and outcomes of pneumonia. This study aimed to evaluate differences between sexes concerning COVID-19-related pneumonia. Methods This was a retrospective, multicentre study that enrolled 340 consecutive adult patients admitted to hospital for COVID-19-related pneumonia. Of these patients, 219 were males (64.4%) and 121, females (35.6%). Primary endpoints were differences between both sexes as per clinical features, laboratory and radiologic results, and inhospital and 30-day mortality. Secondary outcomes included differences between both sexes and factors associated with mortality. Results Males admitted to the COVID-19 Unit were older than females (74.5 ± 15.7 vs. 64.5 ± 11.9). Cardiovascular disorders were more frequent in males (19.17% vs 13.25%), whereas obesity was more common in females (54.5% vs 37.45%). In-hospital and 30-day mortality were higher in males than in females (23.3% vs 15.7%; 24.6% vs 19.8%, respectively). No differences were observed in hospital stay; however, males had a longer ICU stay when compared with females (11.04±5.4 vs 7.05±3.4). Variables associated with a higher mortality rate included older age, a lower number of lymphocytes upon admission and higher levels of ferritin and troponin upon admission. Conclusions Males had significantly higher mortality and longer ICU stay than females. More comorbidities in males than in females could explain the difference in mortality rates. The protective role of genetic factors can partially explain the better outcomes observed in female patients with COVID-19.

Thunderstorm asthma is a rare event: in this letter we describe two cases observed during the same month of 2018 at an Italian Emergency department, assessed by the same medical team and according to the same methodology and approach. Given the infrequency of such a phenomenon and the debate around its nature, frequency, and - at times - existence, we strongly believe it is important for all specialists who observe such cases to report them, building an evidence base to expand its knowledge and understanding.

Background Chest physiotherapy is an important tool in the treatment of bronchiectasis. High frequency chest wall oscillation (HFCWO) is a technique designed to create a global percussion of the lung which moves secretions and probably clears the peripheral bronchial tree. We propose the comparison between an existing device (SmartVest) and a new device (RespIn 11). Methods Sixty patients were randomized into three groups: a group was treated with SmartVest, a group with RespIn 11, and a group with pharmacological therapy alone. Primary outcome measures included exacerbations at 3, 6 and 12 months after the end of treatment. Secondary outcome measures were pulmonary function testing, arterial blood gas analysis (ABG), and hematological examinations, cough, dyspnea, health and quality of life scores (Bronchiectasis Health Questionnaire [BHQ], Breathlessness, Cough, and Sputum Scale [BCSS], COPD Assessment Test [CAT], Leicester Cough Questionnaire [LCT]). A 5-point Likert scale was used to evaluate patient's preference. Results Both patients in the HFCWO groups showed a significant improvement in the tests of dyspnea, cough and health and quality of life score evaluations (BHQ, BCSS, CAT, LCT) compared to the control group, but not in pulmonary function tests and ABG. Only RespIn 11 significantly reduced exacerbations in comparison with the control group. RespIn 11 also had a higher score regarding patients' preference. Conclusions The two machines have improved health and quality of life scores in patients with bronchiectasis. RespIn 11 also demonstrated a significant lowering of exacerbations and a better patient acceptance.

Objectives Parkinson's disease (PD) causes dysfunction both to swallowing and to the cough mechanism. Oropharyngeal dysphagia is the main cause of pneumonia, due to silent aspiration of food and saliva. Pneumonia is the leading cause of death in PD. Different strategies exist to reduce the risk of inhalation and associated lung infections, but evidence of their efficacy is still unclear. The aim of this preliminary study was to investigate if adding an expiratory flow acceleration (EFA®) technique to standard therapy (ST) for dysphagia can reduce the incidence of bronchopulmonary infections and improve quality of life, respiratory function parameters, cough, and airways encumbrance perception. Materials and Methods Twenty-five patients with PD were randomized to two groups: ST vs. ST + EFA. Patients were re-assessed at 30, 180 and 360 days from start of treatment. The primary outcome was the incidence of respiratory exacerbations together with quality of life score (PDQ-39). Secondary outcomes were changes in respiratory function tests, cough capacity (CPEF), perceived health status (Euro-QOL-VAS), cough, and upper airways encumbrance perception evaluated by visual numeric scale (VNS). Results Twenty patients concluded the study (10 each group). Albeit the difference was not significant, less respiratory infections, symptoms, hospital admissions and medical visits were found in the study group. Furthermore, there was a significant difference in cough effectiveness measured with the peak cough expiratory flow (PCEF) and other spirometry parameters (FEV1, FVC), and also in specific and generic health-related quality of life measures (PDQ-39, Euro-QoL-VAS). Conclusion The results of this preliminary study support the use of EFA® technology in Parkinson's patients with dysphagia to reduce the risk of respiratory complications. Nevertheless, further studies are needed in a larger, more representative sample to definitively confirm the usefulness of this technique in PD patients.

Background Respiratory disorders are a major cause of morbidity and mortality in amyotrophic lateral sclerosis (ALS). Current guidelines suggest the provision of noninvasive ventilation (NIV) for symptomatic hypoventilation in patients with ALS. Inspite of these results the proportion of ALS patients on tracheostomy invasive ventilation (TIV) is relatively high. Methods Thirty-two patients were included in the study: 16 patients were treated with nocturnal NIV associated with diurnal mouthpiece ventilation (MPV) and 16 with TIV .The primary endpoint of the study was to evaluate survival in the two groups. Secondary endpoints were to evaluate differences in the two populations in terms of clinical outcomes and quality of life (HRQoL). Results Cox analysis survival data shows no statically difference in the hazard function of the two groups. The comparison between the two groups showed a significant improvement in the average value of gas indices (paO2, paCO2) in the group treated with TIV in comparison to the group treated with MPV/NIV. Conversely, the evaluation of the questionnaires on HRQoL showed a higher score in patients treated with MPV/NIV compared to those treated with TIV. Conclusions Ventilatory treatment with MPV and TIV did not demonstrate significant differences in survival. Patients treated with MPV reported a better HRQoL, although TIV group showed higher ventilatory parameters improvement than MPV group.

Non-invasive ventilation (NIV) has been used successfully for the management of acute respiratory failure (ARF) more often in the last two decades compared to prior decades; nevertheless, NIV can have failure rates ranging from 5% to 50%. However, there are particular groups of patients that are more likely to benefit from NIV. One of these groups is patients with hypoventilation syndrome (OHS). The aim of this review is to seek evaluation of the effectiveness of NIV in acute setting. Only a few studies have investigated NIV success or failure in OHS patients. More than 30% of them were diagnosed when hospitalized for ARF. NIV rarely failed in reversing ARF. OHS patients who exhibited early NIV failure had a high severity score and a low HCO3 level at admission; more than half of hypercapnic patients with decompensated OHS exhibited a delayed but successful response to NIV. Patients with decompensation of OHS have a better prognosis and response to NIV than other hypercapnic patients. They require more aggressive NIV settings, a longer time to reduce PaCO2 levels, and more frequently a delayed but successful response to NIV.

Chest physiotherapy is a set of techniques used to help the draining of the mucus from the lung in pathological situations. The choice of the techniques, and their adjustment to the patients or to the pathologies, remains as of today largely empirical. High Frequency Chest Wall Oscillation (HFCWO) is one of these techniques, performed with a device that applies oscillating pressures on the chest. However, there is no clear understanding of how HFCWO devices interact with the lung biomechanics. Hence, we study idealised HFCWO manipulations applied to a mathematical and numerical model of the biomechanics of the lung. The lung is represented by a fluid--structure interaction model based on an airway tree that is coupled to an homogeneous elastic medium. We show that our model is driven by two dimensionless numbers that drive the effect of the idealised HFCWO manipulation on the model of the lung. Our model allow to analyze the stress applied to an idealised mucus by the air--mucus interaction and by the airway walls deformation. This stress behaves as a buffer and has the effect of reducing the stress needed to overcome the idealised mucus yield stress. Moreover, our model predicts the existence of an optimal range of the working frequencies of HFCWO. This range is in agreement with the frequencies actually used by practitioners during HFCWO maneuvers. Finally, our model suggests that analyzing the mouth airflow during HFCWO maneuvers could allow to estimate the compliance and the hydrodynamic resistance of the lung of a patient.

W ciągu ostatnich dwóch dekad obserwuje się coraz częstsze stosowanie wentylacji nieinwazyjnej (NIV) w leczeniu ostrej niewydolności oddechowej (ARF). Wskaźnik niepowodzeń NIV waha się jednak od 5% do 50%, a warunkiem powodzenia jest dobór pacjentów. Istnieją jednak grupy pacjentów, w których jest większe prawdopodobieństwo skuteczności NIV. Jedna z nich to chorzy na zespół hipowentylacji osób otyłych (OHS). W niniejszym przeglądzie podjęto próbę oceny skuteczności stosowania NIV u chorych z ostrą niewydolnością oddechową i określenia predyktorów niepowodzenia NIV u pacjentów z otyłością patologiczną. Zaledwie w kilku badaniach podjęto problem oceny skuteczności NIV w tej grupie chorych. Metoda ta okazywała się skuteczniejsza przy bardziej restrykcyjnej selekcji pacjentów. Otyli, u których niepowodzenie NIV było zauważalne wcześnie, charakteryzowali się wysokim stopniem ciężkości choroby przy przyjęciu do szpitala. Natomiast u więcej niż połowy pacjentów z hiperkapnią i zdekompensowanym zespołem OHS występowała opóźniona, aczkolwiek pozytywna odpowiedź na NIV. U pacjentów z dekompensacją OHS zaobserwowano korzystniejsze rokowanie i lepszą reakcję na NIV niż u innych chorych z hiperkapnią. Potrzebowali oni wyższych nastawień aparatu, dłuższego czasu do obniżenia PaCO2, częściej występowała u nich opóźniona, ale pozytywna odpowiedź na zastosowane leczenie, co powinno raczej przekonywać do stosowania NIV zamiast wczesnej intubacji. Ponieważ nie odnotowano wyraźnych predyktorów niepowodzenia stosowania NIV, konieczna jest dalsza uważna i długookresowa obserwacja.

Non-invasive ventilation ( NIV ) has been used successfully for the management of acute respiratory failure (ARF) more often in the last two decades than previously. Unfortunately, NIV can have failure rates ranging from 5% to 50% and patient selection is the key to success. There are particular groups of patients that are more likely to benefit from NIV. For patients with hypoventilation syndrome (OHS) this treatment can be beneficial. This review seeks to evaluate the effectiveness of NIV in acute ARF and determine predictors of NIV failure in morbidly obese patients. Only a few studies have investigated NIV success or failure in these patients. NIV was most often effective when patients were carefully selected. Obese patients who exhibited early NIV failure had a high severity score at admission. In contrast, more than half of hypercapnic patients with decompensated OHS exhibited a delayed but successful response to NIV. Patients with decompensation of OHS had better prognosis and response to NIV than other hypercapnic patients. They required more aggressive NIV settings, a longer time to reduce paCO2 levels , and more frequently a delayed but successful response to NIV which should encourage the use of NIV rather than early intubation. Since clear predictors of NIV failure have not been identified, a strict and prolonged monitoring is mandatory.

Parenteral nutrition is indicated in amyotrophic lateral sclerosis (ALS) when dysphagia, loss of appetite, and difficulty protecting the airways cause malnutrition, severe weight loss, dehydration, and increased risk of aspiration pneumonia. The aim of this review is to compare percutaneous endoscopic gastrostomy (PEG), radiologically inserted G-tube (RIG), and percutaneous radiologic gastrostomy (PRG) in patients with ALS, performed with or without noninvasive ventilation (NIV). We searched PubMed, MEDLINE, EMBASE, the Cochrane Central Register of Controlled Trials (CENTRAL), the EBSCO Online Research Database, and Scopus up to December 2015. A priori selection included all randomized controlled trials (RCTs), quasi-randomized trials, and prospective and retrospective studies. The primary outcome was 30-d survival. We found no RCTs or quasi-RCTs. Seven studies about the implementation of the PEG/RIG procedure during the use of NIV and 5 studies without NIV were included. In another study of 59 subjects undergoing open gastrostomy, all with vital capacity < 30% of normal, 18 of whom were dependent on continuous NIV at full ventilatory support settings, there were no respiratory complications. Thus, the use of NIV during the implementation of these procedures, especially when used at full ventilatory support settings of pressure preset 18-25 cm H2O, can support alveolar ventilation before, during, and after the procedures and prevent respiratory complications. The procedures investigated appear equivalent, but the methodological quality of the studies could be improved. Possible benefits with regard to nutrition parameters, quality of life, and psychological features need to be further investigated.