2017年 · 第40卷第09期

This issue presents readers with a feast on the application of non-invasive ventilation in the fields of critical illness and sleep respiratory diseases. Experts in the fields of respiratory critical illness, sleep respiratory diseases, pediatrics and neurology are invited to give in-depth guidance on various sub-fields of clinical application of non-invasive ventilation, which deserves readers' attention.

sleep respiratory disorders have a wide spectrum of diseases and a high prevalence in the population, and are the source of many diseases, which seriously endanger physical health and public safety. Since obstructive sleep apnea syndrome (OSAS) was named as a disease in 1965^[1]After more than 50 years of development, sleep respiratory medicine has become one of the three major branches of the modern respiratory disease system, including traditional respiratory diseases and critical illness^[2]。 1981 Sullivan et al.^[3]First, continuous positive airway pressure (CPAP) ventilator is used to treat OSAS. At present, non-invasive ventilation has become the first choice and main treatment for most patients with sleep respiratory diseases. However, how to standardize the application of non-invasive ventilator and improve the compliance of patients' long-term treatment is still the key to maximize its effectiveness, and it is also the top priority to strengthen the discipline construction of sleep respiratory medicine in China.

non-invasive positive pressure ventilation (NPPV or NIPPV) has become a commonly used respiratory support technology in clinic. In recent years, it has been rapidly popularized and widely used in clinical departments and even families. Since NPPV is a single pipeline, it is necessary to intentionally leak air to discharge exhaled air and dilute exhaled carbon dioxide, and NPPV patients are conscious and have spontaneous breathing. Compared with invasive mechanical ventilation, the problem of human-machine asynchronization in NPPV is more prominent and directly affects the treatment effect. How to do a good job in human-machine synchronization requires full consideration from the selection of indications, exclusion of contraindications, ventilator selection, application site, patient factors and application details in clinic to achieve the ideal therapeutic effect. This article focuses on the application of NPPV in patients with acute respiratory failure or acute exacerbation of chronic respiratory failure^[1,2]。

Acute and chronic respiratory failure in children is a disturbance of pulmonary ventilation and oxygenation caused by many reasons, which is a common clinical condition in children and seriously threatens children's health. In addition to the etiological treatment for the primary disease, respiratory support is a commonly used treatment. As a suitable auxiliary ventilation technique, non-invasive ventilation has been confirmed by clinical practice, and it has become one of the conventional treatment measures for acute and chronic respiratory failure^[1]。

Of all patients after surgery, approximately 5% developed pneumonia and approximately 10% developed respiratory failure requiring noninvasive or invasive ventilation^[1]。 However, the incidence rate of postoperative pneumonia in patients undergoing lobectomy is as high as 25%, and the mortality rate of postoperative pneumonia is as high as 20%, which is significantly higher than that in patients without postoperative pneumonia (2%)^[2]。 Factors such as history of chronic obstructive pulmonary disease, extent of surgical resection, preoperative bacterial colonization, and male sex are independent high risk factors for postoperative pneumonia in patients after lobectomy^[2]。

Neuromuscular diseases (NMD) often have respiratory dysfunction. Early recognition and active programmatic management can improve the survival rate, quality of life and survival time of patients. This article introduces the identification and management of respiratory disorders, especially non-invasive ventilatory support and airway management, taking amyotrophic lateral sclerosis (ALS) as an example.

In recent years, more and more scholars have begun to pay attention to the diagnosis and treatment of obesity hypoventilation syndrome (OHS). Home noninvasive positive pressure ventilation is an important treatment for patients with stable OHS. OHS is one of the serious complications of morbid obesity. About 90% of patients with OHS have obstructive sleep apnea (OSA). The incidence of OHS increased significantly with obesity, ranging from 10% to 20% in OSA and from 10% to 20% in BMI>35 kg/m²The incidence rate was 31% in the hospitalized population^[1,2]。 OHS can lead to a significant decrease in the quality of life of patients, an increase in socioeconomic burden, an increase in medical expenses, and a higher morbidity and mortality rate than OSA patients alone^[3]。 Patients with OHS have a worse prognosis compared to simply obese patients, with a case fatality rate of up to 50% in severe OHS hospitalizations, a high ICU occupancy rate and an increased need for non-invasive ventilation in patients with OHS compared to similarly obese patients without hypoventilation syndrome.

obstructive sleep apnea (OSA) is not only a common and frequently occurring disease, but also a risk factor for a variety of systemic diseases. It is associated with the development of hypertension, coronary heart disease, stroke, cognitive dysfunction and type 2 diabetes^[1]。 For more than 30 years, noninvasive positive pressure ventilation (NPPV) has been widely used in the treatment of OSA with different degrees and/or concomitant diseases, and has achieved positive and good results^[2,3,4]。 The appropriate treatment pressure is the key to ensure the success of treatment, and it is also the basis for patients to adhere to long-term treatment. Pressure titration is the basic procedure to determine the appropriate treatment pressure^[5,6]。 Current NPPV pressure titration techniques to standardize OSA are still facing challenges.

Since the beginning of the 21st century, the field of noninvasive positive pressure ventilation (NPPV) has developed rapidly. With the progress of technology and mode, its application is increasingly widespread, and the therapeutic comfort and effectiveness are constantly improved, especially in the treatment of sleep respiratory diseases at home. In recent years, many countries or regions have issued corresponding guidelines and expert consensus to guide clinical practice, covering aspects such as disease spectrum, indication selection, pressure titration and compliance monitoring.

Neuromuscular diseases are a general term for a series of diseases involving motor nerves, neuromuscular junctions or muscles themselves, including amyotrophic lateral sclerosis, myasthenia gravis, Guillain-Barre syndrome, spinal muscular atrophy, Duchenne muscular dystrophy and glycogen storage disease type II. Although different types of neuromuscular diseases have different sites of involvement, severity of disease and survival time, they can all involve respiratory muscles, causing respiratory muscle insufficiency and even respiratory failure. Some neuromuscular diseases even have dyspnea or respiratory failure as the first clinical manifestation. The results of randomized controlled studies showed that noninvasive ventilation can significantly improve sleep quality and quality of life in patients with amyotrophic lateral sclerosis. Except for patients with extremely severe bulbar palsy, noninvasive ventilation usually can prolong survival^[1]Moreover, the patient was satisfied with the initial effect of the noninvasive ventilation therapy. For patients with myasthenia gravis at risk, timely use of non-invasive ventilation can shorten hospital stay and reduce intubation rate^[2,3]。 Noninvasive ventilatory therapy has been reported in the literature to significantly prolong the survival of patients with Duchenne muscular dystrophy, some of whom have lived to middle age^[4]。

Since the 21st century, with the rapid development of the new technology of non-invasive ventilation (NIV), some novel special NIV modes have been developed and used in clinical practice. Its indications have been tested, challenged, updated and expanded in clinical practice, and its application scope has also been continuously developed. The practical experience of two new special NIV modes: adaptive pressure support servo-ventilation (APSSV) and automatic three-level ventilation mode, which we have applied in clinic, is briefly introduced below.

sleep related breathing (SRB) is a group of disorders with abnormal breathing during sleep, with or without abnormal respiratory function during wakefulness. Including sleep apnea, sleep hypopnea and sleep hypoxic diseases caused by different causes^[1,2,3]。 Sleep respiratory disorders are the second largest category of sleep disorders after insomnia. Its high prevalence, frequent complications and serious health hazards have attracted great attention in the clinical medical field in recent years. noninvasive positive pressure ventilation (NPPV) including continuous positive airway pressure (CPAP) Treatment can effectively improve the clinical symptoms and quality of life of various sleep respiratory diseases, and reduce the occurrence and development of cardiovascular, cerebrovascular and metabolic complications. In the past 20 years, with the continuous expansion of clinical indications of NPPV, the continuous improvement of treatment effectiveness and comfort, and the rapid expansion of the number of people receiving treatment, NPPV has become the first choice and the most important treatment for sleep respiratory diseases. At present, the non-invasive ventilation treatment of sleep respiratory diseases in China is still in a state of irregularity and randomness, which seriously affects the treatment of patients and the healthy development of non-invasive ventilation field. Therefore, the Sleep Breathing Disorders Group of the Respiratory Disease Branch of Chinese Medical Association referred to the existing guidelines at home and abroad^[4,5,6]Based on the results of clinical research and clinical research, this consensus is drawn up in combination with domestic clinical practice and research results, which is intended to guide the standardized application and management of NPPV in the treatment of sleep respiratory diseases in China, and is expected to become the clinical basis to guide the standardized application of non-invasive ventilation for sleep respiratory diseases in China.

Methicillin-resistant Staphylococcus aureusStaphylococcus aureusMRSA) is one of the important pathogens of nosocomial infections worldwide, but in recent years, it has been found that MRSA can also appear in community infections, and patients often have no definite risk factors for infection^[1]。 A case of community-acquired methicillin-resistant Staphylococcus aureus (CA-MRSA) severe pneumonia complicated with influenza B virus infection is reported to improve the understanding of this disease.

Diaphragm is the most important respiratory muscle, and diaphragm dysfunction is common in patients with mechanical ventilation, which is one of the main factors of disconnection difficulty, which seriously affects the prognosis and outcome of patients. Ventilator-induced diaphragmatic dysfunction (VIDD) refers to diaphragmatic atrophy and systolic dysfunction with unclear mechanisms caused by mechanical ventilation and diaphragmatic unloading, and cannot be explained by sepsis, drugs, metabolic disorders, malnutrition, acquired neuromuscular diseases, etc^[1]。 In animal experiments, important progress has been made in the pathogenesis, pathological research, monitoring and prevention and treatment methods of VIDD, but its pathogenesis and prevention and treatment methods still need to be further studied to extend to clinical practice and improve the success rate of offline.

acute respiratory distress syndrome (ARDS) is a common acute and critical disease in clinic, with a sudden and dangerous onset. The pathogenesis has not been fully elucidated, and the treatment is difficult and the mortality rate is high. Experimental results indicate that changes in cytokine levels are one of the pathogenesis mechanisms of ARDS^[1]。 IL-18 is a multifunctional cytokine discovered in recent years. It has various biological functions such as inducing the production of interferon-γ (IFN-γ), promoting the proliferation and activation of T cells, enhancing the activity of NK and CTL cells, and enhancing Fas-mediated cytotoxicity, thus exerting strong biological activities such as defense, anti-tumor and immunomodulation^[2,3]。 The biological characteristics of IL-8 and its research progress in ARDS are reviewed as follows.

lymphangioleiomyomatosis (LAM) is a rare disease with lung involvement as the main manifestation. It is characterized by abnormal proliferation, infiltration and spread of smooth muscle cells (LAM cells) resulting in cystic lesions of the lung parenchyma and angiomyolipoma (often occurring in the kidney, but also in the liver and spleen). Due to lymphatic obstruction, it is often accompanied by clinical manifestations such as chylopleural effusion and/or ascites. There are two patterns of onset, the independent onset is sporadic LAM, and the other is LAM (TSC-LAM) with tuberous sclerosis complex (TSC). The incidence of this disease is low, mainly in women of childbearing age, and rare in children and men^[1,2]。 The incidence of sporadic LAM in foreign adult women is about 3.4~7.8/1 million, and the incidence of LAM in adult women with TSC is about 30% ~40%^[3,4,5]The mean age of onset was (38.9±0.7) years^[6]。 About 130 cases were reported in China in the past 30 years, with an average onset age of (36.2±8.6) years^[7]。 Most of the cases reported in China are after 2000. In order to standardize the diagnosis and treatment, the European Society of Respiratory Diseases developed the guidelines for the diagnosis and treatment of LAM in 2010 (2010 edition of the guidelines)^[3]In 2016, the American Thoracic Society/Japanese Respiratory Society jointly developed a new version of the guidelines for the diagnosis and treatment of pulmonary lymphangiomyomatosis (2016 version of the guidelines)^[8]。 A number of studies have found that LAM cells carrying mutant genes and their secreted product-vascular endothelial growth factor-D (VEGF-D) exist in lung tissues and body fluids of patients, which provides a basis for diagnosis and treatment. The relevant research progress at home and abroad is summarized as follows.

OSAHS is the most common sleep disorder, which refers to the periodic partial or complete collapse of the upper respiratory tract during sleep^[1]Or hypopnea and/or apnea during sleep^[2]Leading to structural disorders of sleep and repeated wakefulness, resulting in intermittent hypoxemia, sleep interruption, and metabolic disorders^[3]Increase the incidence and mortality of cardiovascular diseases^[3,4]。 The main clinical manifestations of OSAHS are daytime drowsiness, morning headache, mood disorders, memory loss, related cognitive decline, and decline in quality of life^[5,6]。

A 35-year-old male was admitted to the hospital on April 25, 2016 due to "repeated cough and shortness of breath for 4 years, aggravated with fever for 1 month". Four years ago, the patient developed cough, expectoration with fever without obvious inducement, and felt chest tightness and shortness of breath after activity. At that time, there was no chest pain, no hemoptysis, no hoarseness, no night sweats and fatigue, so he went to the local hospital for treatment. In August 2012, the chest CT showed a nodule of about 10 mm ×15 mm in the upper lobe of the left lung, and no calcification was seen (Figure 1)。 After anti-infection and other treatments, the symptoms improved and he was discharged from hospital. In the past 4 years, the cough symptoms repeatedly attacked, often aggravated at the change of seasons, and no formal treatment was given. One month ago, I developed fever without obvious inducement, with the highest body temperature of 38 ℃, chest tightness and shortness of breath, with mild degree, accompanied by hoarseness, so I came to our hospital for treatment. Chest CT showed a cavity of about 15 mm ×19 mm in the upper lobe of the left lung, with uneven walls and punctate calcification on the cavity wall (Figure 2，Figure 3), coronal reconstruction showed thickening of the left main bronchial wall (Figure 4)。 Previous physique is average, no history of smoking, no history of dust exposure. Physical examination: There were no obvious positive signs in the heart and lung.