2018年 · 第41卷第02期

The golden rooster bids farewell to the old year, and the barking dog makes a new year. On the occasion of the New Year's Festival, the editorial department of this magazine greets the readers, authors and editors who have worked together with us and made progress together.

Pulmonary hypertension is a group of pathophysiological syndromes characterized by increased pulmonary artery pressure and pulmonary vascular resistance caused by different etiologies. Its main pathological mechanisms are vasoconstriction, remodeling and in situ thrombosis, which ultimately leads to increased right ventricular afterload and right heart failure. Pulmonary hypertension combined with hemoptysis is rare in clinic. Its etiology is different from hemoptysis caused by common respiratory diseases such as bronchiectasis and lung cancer, and its clinical treatment is not exactly the same. When improper treatment leads to aggravation of the disease, it often endangers the lives of patients. In recent 20 years, there are few reports about pulmonary hypertension combined with hemoptysis, and the prognosis of patients is poor. In this forum column, Li Jieying and others introduced the clinical manifestations and treatment progress of different types of pulmonary hypertension combined with hemoptysis. The application choices of bronchial arterial embolization (BAE) in patients with pulmonary hypertension combined with hemoptysis are briefly discussed as follows.

At present, the incidence of non-tuberculous mycobacterial disease (NTM) in European, American and Asian countries is increasing year by year. The annual growth rate of NTM lung disease in the United States was 8.2% from 1997 to 2007^[1]； In Asia, including China, the infection rate of NTM is also increasing^[2]。 There are three main groups of people affected by NTM: chronic lung disease, immunocompromised, trauma and nosocomial infection. NTM has been believed to be non-infectious, but recent literature suggests that NTM may be infectious, and its difficulty in diagnosis, high drug resistance and recurrence are all important reasons leading to its harm.

A 26-year-old male was admitted to the hospital on April 18, 2017 due to "shortness of breath after activity with palpitations for more than 1 year, aggravated for 15 days". The patient developed shortness of breath after activity more than 1 year ago, and developed shortness of breath after walking about 50 m, accompanied by palpitations, fatigue, and occasionally cough. Chest CT examination in an external hospital showed pulmonary infection, a small amount of pleural effusion and pericardial effusion, and multiple swollen lymph nodes in the mediastinum (Figure 1，Figure 2), the symptoms did not improve after anti-infection and phlegm-resolving treatment. In the past year, the symptoms gradually worsened. 15 days ago, he was hospitalized in another hospital. He was diagnosed with "severe pneumonia and cardiomyopathy". He was treated with "caspofungin", "moxifloxacin" and "ganciclovir". The symptoms worsened, and he was transferred to our hospital for further treatment. In 2016, a foreign hospital diagnosed "chronic hepatitis C virus", and gave peginterferon α-2a injection 180 μ g subcutaneously, once a week, regular treatment for 1 year, no tobacco and alcohol habit, no special family history and personal history, unmarried and childless.

It is reported in the literature that the incidence of pulmonary embolism is second only to myocardial infarction and stroke in cardiovascular diseases. fatal pulmonary embolism (FPE) refers to acute attacks with cardiac arrest, shock, syncope, severe dyspnea and difficult to correct hypoxemia^[1]。 The prognosis of patients with sudden cardiac arrest is extremely poor. The survival rate of patients with sudden cardiac arrest in hospital is 15% ~37%, and that of patients with sudden cardiac arrest out of hospital is only 5% ~14%. The annual incidence of pulmonary embolism is 30~80/100,000, of which the incidence of FPE out of hospital is about 1.6/100,000^[2,3], the literature reports that 70% of out-of-hospital cardiac arrest patients die from myocardial infarction or FPE^[4,5]。 Whether to perform thrombolytic therapy after cardiac arrest is currently controversial, but the benefit of thrombolysis for FPE has been widely recognized. The clinical characteristics of three FPE patients with cardiac arrest in Sir Run Run Shaw Hospital Affiliated to Zhejiang University School of Medicine from January 2014 to October 2015 are reported as follows, and their early diagnosis and timely thrombolytic treatment methods are discussed.

The patient, a 53-year-old male, was admitted to our hospital in August 2016 due to "intermittent cough and expectoration for 2 years and another 3 months". Two years ago, the patient developed nasal congestion, runny nose, paroxysmal cough and a small amount of white foamy sticky sputum without obvious triggers. Pulmonary function test revealed mild obstructive ventilatory dysfunction, FEV₁/FVC was 69%, FEV₁Represents 80.3% of the estimated value. Chest CT showed increased lobular density in the right lung, and the symptoms were relieved after oral administration of "amoxicillin and cough medicine". Eighteen months ago, the symptoms reappeared, and chest CT revealed right middle lobe atelectasis with bronchial stenosis (Figure 1)； Bronchoscopy showed that the bronchial orifice in the middle lobe of the right lung was narrow, the surface was not flat, the biopsy tissue was slightly brittle and bleeding, and the biopsy pathology showed chronic mucosal inflammation with more eosinophil infiltration; Total blood IgE was 2 646 IU/ml. Seventeen months ago, positron emission tomography (PET-CT) showed atelectasis in the right middle lobe, slightly increased glucose metabolism in the right middle lobe, and slightly increased glucose metabolism in the mediastinum and right hilar lymph nodes, which were considered as inflammatory lesions; Bronchoscopy showed obstruction of new organisms, the surface was covered with white moss-like necrosis, and the new organisms were removed by cryotherapy; Biopsy pathology showed necrotic tissue and inflammatory exudate, and a small amount of pseudo-stratified ciliated columnar epithelial tissue was seen in between; Total blood IgE>1 000 IU/ml; Ultrasound bronchoscopy (EBUS) showed mediastinal lymph node enlargement; Cefixime was administered orally for 1 week. Fifteen months ago, the patient coughed and coughed up sputum again, the amount of sputum increased, and coughed brown-yellow sticky sputum. Yellow purulent sputum was seen in the right bronchial branch (B4) by bronchoscopy, and a large number of sputum plugs were frozen and aspirated; Total blood IgE was 880 IU/ml; Oral expectorants (ambroxol, acetylcysteine) and traditional Chinese medicine. Four months before admission, the patient had a cough again, coughed a small amount of brown-gray sticky sputum, and occasional dull pain and discomfort in the right front chest. Three months before admission, chest CT showed bronchiectasis in the middle lobe of the right lung with cord-like shadows (Figure 2)； Total blood IgE was 2 081 IU/ml; There was no significant relief of symptoms after oral administration of ambroxol and montelukast sodium. Oral prednisone 10 mg 2 times/d was started 2 months before admission and was admitted to our hospital for further treatment.

In 2013, my country approved bronchial thermoplasty (BT) for the treatment of severe asthma patients over 18 years old^[1]。 A patient with bronchial asthma (asthma) complicated with right middle lobe atelectasis was reported. After receiving BT treatment, the asthma symptoms were alleviated, the disease was controlled and the right middle lobe atelectasis was eliminated. The cases are now reported as follows.

pulmonary arterial hypertension (PAH) is characterized by pulmonary arteriole contraction, vascular wall remodeling and in situ thrombosis, with high disability and mortality rates, which seriously endanger patients' lives. In the early stage of PAH, there may be no symptoms or only shortness of breath, fatigue and fatigue after activity. Some patients may have chest tightness, precordial discomfort, angina pectoris or syncope, and may also be manifested as dry cough, nausea, vomiting and hemoptysis after activity.

pulmonary arterial hypertension (PAH) is defined as a disease state with a mean pulmonary artery pressure ≥25 mmHg (1 mmHg =0.133 kPa) at sea level at rest, accompanied by a pulmonary capillary wedge pressure ≤15 mmHg^[1]。 Researchers have done a lot of work on the pathogenesis and treatment of PAH, especially in the fields of immune and inflammatory response mechanisms, but there is still no specific drug to treat this disease^[2]So we expect a better entry point to solve this problem. Although the etiology of PAH is different, the most important pathological basis is the abnormal proliferation and vascular remodeling of pulmonary artery vascular endothelial cells, which is also the intersection of pathological changes of various types of PAH, in which the role of growth factors has been widely confirmed^[3]。 With the increasing maturity of precision medicine technology, it is worth looking forward to whether new therapeutic targets can be found. This article will make a comprehensive summary of the progress of growth factors in PAH research.

thrombotic microangiopathy (TMA) secondary to cancer was first reported by Brain in 1962. In 1987, Murgo proposed that TMA is a tumor thrombotic microangiopathy secondary to extensive tumor metastasis. The main pathological changes of TMA associated with malignant tumors are extensive vascular and lymphatic involvement and hyperplasia and hypertrophy of the intima of small vessels, mainly pulmonary vessels, with microvascular abnormalities in about 90% of cases, and tumor thrombus often distributed in pulmonary arterioles, arterioles and alveolar capillaries. TMA is a group of acute clinicopathological syndromes, mainly including hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP). Its characteristics include microvascular hemolysis, thrombocytopenia, and abnormal broken red blood cells such as helmet-shaped, spinous and triangular shapes can be seen in peripheral blood smears, as well as smaller red blood cells and spherical cells. TMA can occur in various types of tumors, adenocarcinoma is more common, and symptoms can occur in the early or spread stage of cancer. The activity of von Willebrand factor cleaving protease (ADAMTS13) in patients usually does not decrease significantly. Godbole et al.^[1]This paper reports a case of a 42-year-old young man with previous physical health who developed exertion dyspnea in the past 6 weeks. Cardiac color Doppler ultrasound showed pulmonary hypertension and right heart enlargement. pulmonary enhanced CT showed ground glass shadows and small nodules. pulmonary tumor thrombotic microangiopathy (PTTM) caused by metastatic gastric adenocarcinoma was confirmed by pulmonary puncture biopsy. The patient died of cardiac arrest due to obstructive shock 2 weeks after diagnosis. PTTM is a special type of TMA, which is rare in clinic, progresses rapidly, and is difficult to diagnose in early stage. It is mostly confirmed by autopsy.

Bronchiectasis (bronchiectasis) is a chronic respiratory disease characterized by permanent bronchial dilatation. Its main clinical features are cough, dyspnea, hemoptysis and recurrent respiratory infections^[1]。 As a simple, safe and non-invasive auxiliary examination, pulmonary function examination is widely used in the evaluation of respiratory diseases, guiding medication and prognosis judgment. Compared with other respiratory diseases, insufficient attention has been paid to pulmonary function testing in patients with bronchiectasis. The characteristics of pulmonary function and its influencing factors in patients with bronchiectasis are summarized as follows.

2007 Soda et al.^[1]echinoderm mierolubule-associated protein-like 4 (EML4) and anaplastic lymphoma kinase (ALK) can be fused into EML4-ALK gene in lung cancer tissues after fragmentation. The fusion gene is located on chromosome 2. The tumors of nude mice transfected with this fusion gene grow rapidly, while ALK inhibitor can significantly reduce tumor burden and prolong survival time, which proves that this gene mutation is a driving mutation^[2]。 At present, ALK inhibitors mainly include: the first-generation inhibitor crizotinib, whose target is ALK/MET/ROS-1. Second-generation inhibitors: ceritinib, which targets ALK/insulin-like growth factor 1 receptor (IGF-1R) /ROS-1; alectinib, whose target is ALK; brigatinib, whose target is ALK/epidermal growth factor receptor (EGFR) /ROS-1. Third generation ALK inhibitor: lorlatinib, whose target is ALK/ROS-1. In the PROFILE1014 study, the disease control rate of crizotinib treatment was 91%, but the median response time was only 11.3 months, and most initially effective patients eventually developed resistance^[3]。 The resistance mechanisms of different types of ALK inhibitors discovered in recent years are reviewed as follows.

Assisted respiratory therapy is the key in the treatment of respiratory diseases. At present, the commonly used assisted respiratory therapy methods include nasal tube or mask oxygen inhalation, non-invasive and invasive ventilation, etc. High-flow nasal cannula therapy (HFNC) is a new auxiliary respiratory therapy method. Its equipment is mainly composed of venturi air-oxygen mixing valve, heating humidifier and connecting pipeline. It can provide heating and humidifying gas, set accurate oxygen concentration, have a large regulation range of gas flow rate (1~60 L/min), and can reduce the contact interface with patients through nasal tube to improve comfort. The current clinical application of HFNC in sleep respiratory diseases is reviewed.

amyloidosis is a group of diseases in which amyloid is deposited in the extracellular matrix, causing tissue and organ damage at the deposition site^[1]。 systematic amyloidosis (SA) can involve heart, liver, kidney, skin tissue, peripheral nerves, lungs and glands at the same time. pleural amyloidosis (PA) involving pleura is relatively rare^[1]。 This paper reports a case of PA with pleural effusion as the first symptom. At the same time, the PA cases with pleural effusion confirmed by pathology at home and abroad are searched. The characteristics of pleural effusion and thoracoscopic findings of these cases are retrospectively analyzed, so as to help clinicians better understand SA, make a good differential diagnosis of pleural effusion, and avoid misdiagnosis and misdiagnosis.

The patient was a 57-year-old female. He was admitted to hospital on June 11, 2013 due to "repeated cough and expectoration for 4 months and another week". Four months ago, the patient developed cough and white foamy sputum without obvious trigger, with a small amount and easy cough, accompanied by swelling and pain of the knee joint, no fever, chills, chills, fatigue, night sweats, hot flashes in the afternoon, and no hemoptysis. In a hospital in Shaanxi Province, chest CT showed multiple lymph nodes in the mediastinum of both hilar, multiple small nodules in both lung fields, enlarged right hilar with obstructive changes in the lower lobe of the right lung (Figure 1)。 Bronchial mucosal pathology showed chronic inflammation of bronchial mucosa with granulation tissue formation (Figure 2)。 After treatment with anti-infection and phlegm, the effect was not good, and erythema began to appear in both knee joints. It was diagnosed as erythema nodosum in the Department of Dermatology, Xijing Hospital, Fourth Military Medical University. After treatment with "cefixime, indomethacin, prednisone" and topical drugs, erythema nodosum in both knee joints disappeared, leaving some pigmentation. There was still cough and expectoration. In the Department of Respiratory Medicine, Xijing Hospital, Fourth Military Medical University, ultrasound bronchoscopy showed multiple lymph nodes in the mediastinum. The pathology of mediastinal lymph node biopsy by ultrasonic bronchoscopy showed chronic granulomatous inflammation. The immunocytochemical staining results showed that the extruded cells were positive for LCA, the rest (AE1/AE3, CD68, CD163, CD56, CgA, CK7, P63, Syn, TTF1, Vim) were negative, the epithelioid cells were positive for CD68, and the sections of Ki-67 were negative. T-spot test (T-spot) for tuberculosis infection was performed, and TB-DNA was negative. He was diagnosed as "pulmonary sarcoidosis?", and was discharged from hospital after the treatment of resolving phlegm and relieving asthma. Subsequently, the patient coughed and expectorated repeatedly, and each attack was similar and could be relieved by itself. One week ago, the patient had cough and expectoration again, and the outpatient clinic of our hospital was admitted to the hospital with "pulmonary sarcoidosis?" Physical examination: body temperature 36.5 ℃, pulse 88 beats/min, respiratory rate 20 beats/min, blood pressure 120/70 mmHg (1 mmHg =0.133 kPa). Clearness, poor spirit, clear breathing sounds in both lungs, and no dry and wet rales in both lungs.

The patient, a 53-year-old female, was admitted to the hospital on April 20, 2016 due to "fever, cough and multiple lymph node enlargement for more than 1 month". The patient stayed in the eastern United States for one month in February 2016. After returning to China, he developed fever without obvious trigger in mid-March 2016, accompanied by chills without chills, cough without expectoration, and the highest body temperature was 39.4℃. The fever peak mostly appeared in the afternoon. At the same time, he could palpate the swollen lymph nodes in the right supraclavicular fossa and left inguinal area by himself, without redness, swelling, tenderness and ulcer, and denied other accompanying symptoms. After cooling treatment, his body temperature could temporarily drop to normal, but rose again after a few hours. Repeated visits to other hospitals, considering "lung abscess", successively given imipenem cilastatin sodium, vancomycin, linezolid, moxifloxacin for injection to fight infection, rifamycin, isoniazid, pyrazinamide tablets, ethambutol to fight tuberculosis, methylprednisolone 40 mg/d for anti-inflammatory treatment for 3 days, compound methonamine capsules for antitussive treatment, and ambroxol for phlegm reduction, but still chest tightness, shortness of breath and abdominal distension, continue to use loxolol sodium tablets, indomethacin suppository and other antipyretic treatments. The patient has lost about 3 kg of weight since his illness, has a history of "allergic rhinitis" for more than 10 years, has undergone subtotal hysterectomy for "uterine fibroids" and vocal cord polypectomy.

OSAHS is a condition of obstructive apnea or hypopnea caused by repeated partial or complete collapse of the upper airway during sleep^[1]It is closely related to a variety of cardiovascular and cerebrovascular diseases and endocrine system diseases, and can damage a variety of systems of the body^[2]。 Stenosis of upper airway anatomy, abnormal neuromuscular function and ventilatory regulation are important factors in the development of OSAHS^[3]At present, the main treatment methods are continuous positive airway pressure (CPAP), upper airway surgery and oral appliances, but the compliance of CPAP treatment is not good^[4]And side effects of appliances^[5]Can affect the therapeutic effect, while the long-term effective rate of surgical treatment is low^[6,7]。 Rogers^[8]Proposed in 1918 that orofacial muscular functional therapy (OMT) could improve mandibular development, nasal breathing and facial profile, Guimaraes^[9]Based on the changes of oropharyngeal soft tissue in patients with OSAHS in 1990s, it was proposed that orofacial muscle function therapy can be used as a treatment method for OSAHS. In recent years, more and more studies have been made on this treatment method. The relevant studies on the application of OMT in patients with OSAHS are summarized and analyzed as follows to evaluate its effectiveness in the treatment of OSAHS.

Bronchopleural fistula (BPF) is a serious complication after lobectomy and total pneumonectomy. It most often occurs after total pneumonectomy. Although the incidence rate is small, once it occurs, it is life-threatening^[1,2]。 Patients are prone to repeated fever, cough and other lung infection symptoms, and are prone to repeated pleural effusion, pneumothorax and empyema. Surgical operation is often impossible due to poor general condition and severe infection. However, bronchoscopic treatment can choose bio-adhesive adhesion, stent or coil for occlusion^[3,4]。 Two cases of BPF closure using an atrial septal occluder are reported as follows.

A 70-year-old male was admitted to the hospital on 3 June 2017 due to "intermittent fever with cough for 1 month". One month ago, the patient developed cough, white sticky sputum and yellow purulent sputum without obvious trigger, accompanied by fever, with a maximum body temperature of about 38℃, chest tightness, shortness of breath, dull pain in the back on both sides, and no hemoptysis. Cefotiam was given to the local hospital to fight infection for 1 week without improvement, and then transferred to the respiratory department of the top three hospital in the provincial capital for 13 days. CT of the chest in the outer hospital showed large consolidation of the right upper lung (Figure 1), was diagnosed with pneumonia, and meropenem (1.0 g intravenous drip, 3 times/d, 9 d) and etilmicin sulfate (0.3 g intravenous drip, 1 time/d, 6 d) were given successively. After treatment, the body temperature dropped, but re-examination of chest CT showed a new large patch of high-density shadow in the left lung (Figure 2，Figure 3), suspected tuberculosis referred to our hospital. The patient was previously in good health. Physical examination: body temperature 36.3 ℃, pulse 84 beats/min, respiratory rate 18 beats/min, blood pressure 126/73 mmHg (1 mmHg =0.133 kPa), clear respiratory sounds in both lungs, no dry or wet rales, heart rate 84 beats/min, no murmur in each valve area. The abdomen was flat and soft, without tenderness, the liver and spleen were not touched under the costs, and there was no edema in both lower limbs. Blood routine white blood cells were 10.8×10 during hospitalization in the top three hospitals in the provincial capital⁹/L, 0.82 for neutrophils, 112 g/L for hemoglobin and 225×10 for platelets¹²/L, and no abnormalities were found in urine routine, liver and kidney function, electrolyte and blood glucose tests. D-dimer was 0.22 mg/L, ESR was 84 mm/1 h, CRP was 49.6 mg/L, and procalcitonin was 0.10 μ g/L. Blood gas analysis (without oxygen inhalation) pH 7.40, PaO₂72 mmHg, PaCO₂35 mmHg, HCO₃^－25.2 mmol/L. Tumor marker: Ferritin was 297.9 μ g/L, and the rest were negative. HIV antibody negative. Anti-nuclear antibody 1:1 000 (spot type), anti-extractable nuclear antigen antibody (ENA profile) negative, rheumatoid factor, immunoglobulin and complement were normal. Sputum culture was negative, sputum smear was negative for acid-fast staining, serum mycoplasma and chlamydia antibodies were negative, and serum cytomegalovirus and Epstein-Barr virus nucleic acid tests were negative. Bronchoscopy: Inflammatory changes in the dorsal segment of the upper and lower lobes of the right lung with carbon deposition, and cytology and bacteriology of lavage fluid were negative.