Volume 104 · Issue 42 · 2024

With the increasing age of patients with pulmonary arterial hypertension (PAH) at diagnosis, multiple comorbidities are more and more common in these patients, especially cardiopulmonary diseases. This article introduces the incidence of PAH combined with cardiovascular disease and/or chronic lung disease, elaborates on the impact of cardiopulmonary comorbidities on the diagnosis, prognosis, and targeted drug treatment strategies of PAH, and proposes management recommendations for PAH patients with comorbidities, aiming to further improve the diagnosis and treatment level of pulmonary hypertension.

Human respiratory syncytial virus (RSV) is a major pathogen causing lower respiratory tract infections in humans. It is particularly a common cause of hospitalization among high-risk groups such as infants, tots, and the old people, imposing a heavy burden on families and society. The standardized treatment and prevention of this disease are increasingly emphasized by healthcare professionals. However, there are currently no standardized guidelines in China for lower respiratory tract infections caused by RSV. Therefore, our group for this guideline has developed the "Guidelines for the treatment and prevention of lower respiratory tract infections caused by human respiratory syncytial virus (2024 edition)" based on the latest evidence-based medical evidence and guideline development methods. The aim is to provide guidance to relevant healthcare professionals and improve prevention and treatment levels.

To explore the relevant factors associated with poor prognosis in patients suffering from chronic obstructive pulmonary disease (COPD) combined with pulmonary embolism (PE), and investigate the predictive value of the simplified pulmonary embolism severity index (sPESI) score on adverse outcomes in these patients.

A total of 168 patients with COPD and PE who were treated at West China Hospital of Sichuan University from January 1, 2018, to December 30, 2020 were retrospectively included. Patients were divided into adverse outcome group and control group based on the occurrence of adverse outcomes [any of the following events: in-hospital death, intensive care unit (ICU) admission, and endotracheal intubation]. Correlation factors for poor prognosis were explored using multivariate logistic regression analysis. Receiver operating characteristic (ROC) curve was employed to assess the predictive value of the sPESI score for adverse outcomes in COPD patients with PE.

A total of 168 patients were studied, with an age of (73.4±10.4) years and 119 male (70.8%). In the adverse outcome group, there were 18 cases (10.7%), including 12 in-hospital deaths, 6 ICU admission, and 1 endotracheal intubation. The control group comprised 150 cases (89.3%). Statistically significant differences were observed between two groups regarding the proportion of patients with diabetes, nephrotic syndrome, severe pneumonia, respiratory failure and lower extremity edema, and the pulse, diastolic blood pressure, pulse oxygen saturation, lactate dehydrogenase and cholesterol levels (all P<0.05). Multivariate logistic regression analysis revealed that severe pneumonia, respiratory failure, lower extremity edema, and diastolic blood pressure<60 mmHg (1 mmHg=0.133 kPa) are correlative factors of adverse outcomes in patients with COPD complicated by PE [OR (95%CI) were 7.363 (1.053-51.772), 4.077 (1.030-16.133), 4.490 (1.131-17.832), and 8.060 (1.209-53.918), respectively, all P<0.05]. The sPESI score in the adverse outcome group was higher than that in the control group [M (Q₁, Q₃), 2 (2, 2) vs 1 (1, 2) score, P=0.006]; the optimal cutoff value for sPESI score was 2 score, the sensitivity was 77.8%, the specificity was 54.0%, and the area under the curve (AUC) and 95%CI were 0.681 (0.554-0.809) based on the ROC curve analysis. Patients with sPESI≥2 score exhibited a 4.109-fold (95%CI: 1.292-13.063, P=0.017) increased risk of adverse prognosis compared to those with sPESI<2 score.

Patients with COPD combined with PE have a higher incidence of adverse prognostic outcomes. Severe pneumonia, respiratory failure, lower limb edema, and diastolic pressure<60 mmHg are associated factors for poor prognosis. The sPESI score has some value in predicting adverse outcomes in COPD patients with PE.

To explore the effectiveness and safety of direct oral anticoagulant rivaroxaban and low molecular weight heparin (LMWH) in the treatment of tumor patients with venous thromboembolism (VTE).

A retrospective analysis was conducted on 296 patients diagnosed with tumor associated VTE in the Shanghai Pulmonary Thromboembolism Database from December 2020 to September 2022. Patients were grouped according to the prescription of anticoagulant drugs. Thirteen baseline variables [age, gender, smoking history, physical state (PS) score, tumor type, tumor stage, tumor treatment method, hemoglobin, platelets, D-dimer, creatinine, alanine aminotransferase, and VTE site] were matched. After matching, 100 cases were assigned to rivaroxaban group, including 64 males and 36 females, aged [M (Q₁, Q₃)] 70 (62,74) years old; There were 100 cases in the LMWH group, including 69 males and 31 females, aged 68 (60,73) years old. Kaplan-Meier method was used to plot survival curves. The differences between the rivaroxaban group and LMWH group in 6-month cumulative VTE recurrence rate, clinically significant bleeding rate, and all-cause mortality rate were analysed using log-rank test.

There were no statistically significant differences between the rivaroxaban group and the LMWH group in the 6-month cumulative VTE recurrence rate [13.5% (95%CI: 6.4%-20.1%) vs 7.5% (95%CI: 2.0%-12.7%), P=0.171], bleeding incidence rate [9.2% (95%CI: 3.3%-14.8%) vs 6.2% (95%CI: 1.3%-10.9%), P=0.438] and all-cause mortality rate [8.0% (95%CI: 2.5%-13.2%) vs 10.0% (95%CI: 3.9%-15.7%), P=0.602].

The anticoagulant efficacy and safety of rivaroxaban and LMWH are comparable in tumor patients with VTE.

In the 2022 European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines, the hemodynamic definition of pulmonary hypertension (PH) was revised to mean pulmonary arterial pressure (mPAP)>20 mmHg (1 mmHg=0.133 kPa) at rest. This study aimed to evaluate the impact of the revised hemodynamic definition on the diagnosis of PH.

Patients with dyspnea or highly suspected PH who underwent right heart catheterization (RHC) in Beijing Anzhen hospital affiliated to Capital Medical University from September 2021 to October 2022 were enrolled retrospectively. According to resting hemodynamics, the patients were divided into 3 groups: no-PH (mPAP≤20 mmHg) group, low-pressure PH (20 mmHg<mPAP<25 mmHg) group and high-pressure PH (mPAP≥25 mmHg) group. The diagnosis and classification of PH and the hemodynamic parameters in different groups were compared before and after the revision of diagnostic criteria. Receiver operating characteristic (ROC) curve was used to evaluate the diagnostic efficacy of maximum of mPAP (mPAP_max) and mPAP/cardiac output (CO) slope of multi-point in PH.

A total of 117 patients were included for analysis, the age was (53.7±17.5) years, 37 (31.6%) patients were male. According to the 2022 ESC/ERS definition, 99 patients were diagnosed as PH, including 8 patients (6.8%) in the low-pressure PH group and 91 patients (77.8%) in the high-pressure PH group. There were 18 patients (15.4%) in the non-PH group. Comparing to the 2015 ESC/ERS definition, 8 patients were newly diagnosed as PH. Echocardiography indexes pulmonary systolic pressure (sPAP) [(57.6±7.3) vs (38.5±13.6) mmHg], max tricuspid regurgitation velocity (TRV_max) [(360.4±28.9) vs (271.4±52.2) cm/s] and RHC index mPAP [(22.2±1.3) vs (16.1±2.6) mmHg] in low-pressure PH group were higher than those in no-PH group (all P<0.05). However, further analysis of hemodynamics during exercise showed mPAP_max [(42.1±5.6) vs (35.6±4.7) mmHg, P=0.006] and mPAP/CO slope of multi-point [(4.9±1.3) vs (3.6±1.1) mmHg·L^-1·min^-1,P=0.024] in low-pressure PH group were higher than those in no-PH group. The ROC curve cut-off value of mPAP_max for the diagnosis of PH was 39 mmHg, with a sensitivity of 75.0%, a specificity of 76.5%, and the area under the curve (AUC) and 95%CI is 0.816 (0.638-0.994). The ROC curve cut-off value of mPAP/CO slope of multi-point for the diagnosis of PH was 4.44 mmHg·L^-1·min^-1,with a sensitivity of 75.0%, a specificity of 76.5%, and an AUC (95%CI) of 0.794 (0.606-0.983).

After the revision of the hemodynamic definition of PH in the 2022 ESC/ERS guidelines, more PH patients with pulmonary vascular disease are detected, thereby facilitating early diagnosis and treatment.

To analyze the survival status and prognostic factors of patients with hereditary hemorrhagic telangiectasia-associated pulmonary arterial hypertension (HHT-PAH).

This prospective study included patients diagnosed with HHT-PAH at the Fuwai Hospital of the Chinese Academy of Medical Sciences and Guangdong General Hospital from August 2009 to December 2019. Patients were followed up every 6 months±2 weeks, with all-cause mortality as the study endpoint. Multivariate Cox proportional hazards regression mode was used to further screen and validate the prognostic factors. Survival curves were plotted using the Kaplan-Meier method, and the log-rank test was used to compare the survival time differences between patients with and without hepatic arteriovenous malformations.

A total of 18 HHT-PAH patients were included, with 14 females (77.8%) and a median age [M (Q₁, Q₃)] of 39 (32, 45) years. The median follow-up time was 129 (51, 156) months. During the follow-up period, 12 patients (66.7%) died, with a median survival time of 70 (32, 123) months. The 1-year, 3-year, 5-year, and 10-year survival rates were 100.0%, 70.8%(95%CI: 52.3%-96.0%), 53.1%(95%CI: 34.0%-83.0%), and 26.6%(95%CI: 10.6%-66.4%), respectively. HHT-PAH patients with hepatic arteriovenous malformations had a shorter median survival time than those without hepatic arteriovenous malformations [31.0 months (95%CI: 16.6-45.4 months) vs 84.0 months (95%CI: 54.4-113.6 months), P=0.024]. Cox proportional hazards regression analysis showed that hepatic arteriovenous malformation (HR=4.493, 95%CI: 1.064-18.978) was a risk factor for all-cause mortality in HHT-PAH patients.

The 10-year survival rate of HHT-PAH patients is poor. The presence of hepatic arteriovenous malformations is a risk factor for mortality in HHT-PAH patients.

To analyze the incidence and risk factors of congenital heart disease (CHD) in Qingdao.

A prospective study was adapted, and study participants were pregnant women who underwent prenatal screening in Qingdao from August 2018 to June 2020 and their offspring (the whole population coverage). CHD in neonates was screened by using the pulse oximetry saturation and heart auscultation, and the final diagnosis was determined by the result of echocardiography. Multivariable logistic regression was performed to analyze the risk factors of CHD.

The study included 115 238 live births, among which 709 were diagnosed with CHD, with an incidence of 6.15/1 000. Ventricular septal defect, atrial septal defect, as well as ventricular septal defect and atrial septal defect were the main CHD subtypes, with incidences of 2.97/1 000 (342/115 238), 1.01/1 000 (116/115 238), and 0.39/1 000 (45/115 238), respectively, comprising 48.2% (342/709), 16.4% (116/709), and 6.3% (45/709) of the CHD cases. The results of multivariable logistic regression showed that the offspring of women with a graduate degree (compared to junior high school or below) (OR=1.66, 95%CI: 1.15-2.40), pregnancy history of CHD (OR=9.50, 95%CI: 5.37-16.81), pregestational diabetes mellitus (OR=3.40, 95%CI: 1.58-7.32) had a higher risk of having CHD, whereas the offspring of multiparous women was associated with a lower risk of CHD (OR=0.84, 95%CI: 0.71-0.99). In addition, compared with male newborns, female newborns have a higher risk of having CHD (OR=1.18, 95%CI: 1.01-1.39).

The incidence of CHD in Qingdao is 6.15/1 000, with ventricular septal defect, atrial septal defect, as well as ventricular septal defect and atrial septal defect being the main subtypes. Maternal education, parity, pregnancy history of CHD, pregestational diabetes mellitus, and offspring gender are associated with CHD occurrence.

To summarize the characteristics of urinary system symptoms in 11 patients with neuronal intranuclear inclusion disease (NIID), in order to improve the understanding of the disease. A retrospective analysis was performed on 15 patients with NIID of the First Affiliated Hospital of Zhengzhou University, which were enrolled between January, 2020 and December, 2022. All of them were confirmed by NOTCH2NLC gene and pathology. GGC repeated expansion mutationof NOTCH2NLC gene, the pathogenic gene of NIID, was detected by gene test, and eosinophilic intranuclear inclusions were detected by skin pathological biopsy. 4/15 patients started with urinary symptoms, 11/15 patients with NIID combined with different degrees of urinary system symptoms, manifested as frequent urination, weak urination, incontinence and other neurogenic bladder symptoms, and the serious patients due to long-term urinary retention, secondary ureteral dilation, hydronephrosis, even renal failure, some patients with urinary system symptoms may be earlier than other systems.

This article reports a 62-year-old female patient with lung cancer who developed bile duct disappearance syndrome after being treated with toripalimab. The 62-year-old female was admitted to China-Japan Friendship Hospital on February 26, 2024 because of "yellow staining of skin and sclera for 1 month". After admission, the liver histology was completed and considered as drug-induced bile duct disappearance syndrome with intrahepatic cholestasis. Symptoms improved after treatment with hormones combined with immunosuppressants. It is suggested that in the course of immune checkpoint inhibitor therapy, we should be alert to the occurrence of severe liver injury such as rare bile duct disappearance syndrome, and identify and deal with suspicious drugs in time.

This article reviews the diagnosis and treatment of a patient on maintenance hemodialysis with rapid progression and multiple recurrences of neoplastic calcinosis of the left forearm. The patient was admitted due to rapid recurrence after resection of the left forearm mass. One month before admission, the left forearm mass was surgically resected. Two weeks after operation, the mass recurred and increased compared with the previous, accompanied by local skin redness, increased tension and tenderness. Laboratory tests revealed abnormal blood phosphate; The X-ray film showed a soft tissue mass shadow on the medial side of the left forearm, showing "settlement sign"; Ultrasound showed a subcutaneous hypoechoic mass in the left forearm, with clear boundaries, uneven internal echoes, multiple punctate strong echoes and "streaming" sound shadows. Re-operation was given, and the postoperative pathology was consistent with "tumor-like calcium salinosis". The patient was followed up for 16 months without recurrence, and the left forearm graft fistula was well used.

Pyroptosis, as a special form of cell death, exists widely in eukaryotic cells, which is closely related to the occurrence and development of autoimmune diseases. In recent years, it has been found that the pyrocortin D (CSDMD) protein, located downstream of the pyroptosis axis, plays a key role in the mechanism of cell pyroptosis. The function of this protein is to activate the inflammatory bodies upstream of the signaling pathway and partly cut them into active fragments, and then form a ring columnar cell perforating pore with various components on the cell membrane, which changes the cell membrane permeability and accelerates a variety of intracellular inflammatory factors. A large number of releases, causing a severe immune inflammatory reaction. CSDMD protein is involved in the pathogenesis of many diseases. In this paper, GSDMD protein in pyroptosis axis nucleotide binding oligomerization domain-like receptor thermoprotein domain-associated protein 3 (NLRP3) /caspase-1 (caspase1) /GSDMD is taken as an entry point to review its relationship with five common autoimmune diseases from a clinical perspective, and provide reference for the follow-up research on the role of pyroptosis in the pathogenesis of autoimmune diseases.

Biosynthetic nanobubbles (GVs) are biosynthetic, gas-filled protein nanostructures, which have been used in ultrasound diagnosis and treatment of tumors. Based on its unique structural and physical characteristics, GVs can be targeted and functionally modified to achieve targeted imaging of tumors. In addition, GVs can be used as a cavitation core, drug carrier, and the like for tumor therapy. In this paper, the origin, structure, genetic engineering technology of GVs and its development and application in ultrasound diagnosis and treatment of tumors are briefly reviewed.

Photodynamic therapy is a minimally invasive treatment in which a photosensitizer is injected intravenously into the body to irradiate and activate the photosensitizer in tumor tissue or cells using light of an appropriate wavelength, destroying the target tissue without damaging the surrounding tissue. In this paper, the research progress of photodynamic therapy in ocular diseases is reviewed from three aspects: novel nano-delivery system, photosensitizer and clinical application.

The patient, a 73-year-old male, went to the outpatient clinic of respiratory and critical care medicine department of Qingdao Municipal Hospital for "right chest and back pain for more than 1 month". He had a history of hypertension and diabetes for more than 10 years. On physical examination, the breathing sounds of both lungs were clear, no obvious dry and wet rales were heard, and the heart rhythm was uniform. On admission, neuron-specific enolase was found to be 24.87 μ g/L, carcinoembryonic antigen was found to be 2.09 μ g/L, and cytokeratin 21 fragment antigen was found to be 1.65 μ g/L. Chest plain scan + enhanced CT showed a mass-like soft tissue density shadow at the apex of the right lung, with a maximum cross section of about 57 mm ×43 mm, a CT value of 43 HU and a CT value of 48 HU after enhancement (shown by arrows in Figure 1, A is the plain scan pulmonary window, and B is the enhanced image); Multiple nodular thickening of the right pleura and bone destruction of the adjacent ribs were seen (Figure 2, black arrows show multiple nodular thickening of the pleura; white arrows show bone destruction of the ribs). CT-guided percutaneous biopsy of lung and pleural lesions was performed, and the pathology showed patches of mild-moderate atypical epithelioid cell proliferation in fibrous tissue (arrows in Figure 3, hematoxylin-eosin staining, ×400); Immunohistochemistry: CD31 (+), EMA fraction (+), CD56 (-), CK (-), TTF-1 (-), P40 (-), CD34 (-), F8 (-), VIM (+), D2-40 (-), S-100 (-), HMB45 (-), Mlan-A (-), CD68/PGM1 (-), Calretinin (-), CAM5.2 (+ / -), CK7 (-), CD10 (-), RCC (-), CK8 (-), Ki-67 was 10%, predisposing to epithelioid hemangioendothelioma (EH). According to the imaging and pathology, the patient's pleura and lung EH were diagnosed clearly. EH is a rare low-grade malignant mesenchymal cell-derived tumor. It is rarely reported at home and abroad that EH is found in both pleura and lung. The typical CT manifestations of pleural EH are pleural thickening and pleural effusion, among which the pleural thickening is mostly heterogeneous and diffuse, and pleural collapse and pleural nodules with or without calcification can also be seen. The typical CT manifestations of pulmonary EH are multiple nodules of different sizes in the lungs, mainly distributed in the middle and lower lung fields, especially in the subpleura and around blood vessels. Pleural traction or pleural depression may occur, and some nodules are coagulative necrosis in the center, which can cause calcium salt deposition and calcification. EH with single nodules in the lungs is rare. EH is mainly treated by surgery, and for cases that cannot be completely resected, radiotherapy, chemotherapy or targeted therapy can be used. The overall prognosis of EH is poor, and it is prone to recurrence and metastasis.

A 29-year-old male patient went to the emergency department of Peking Union Medical College Hospital for "fever for 3 days and shortness of breath for 1 day". 3 days ago, high fever, sore throat and dry cough occurred, and the nucleic acid of influenza A virus was tested positive in another hospital, and oseltamivir and moxifloxacin were given orally; 1 d Shortness of breath before; Past physical fitness. Tracheal intubation, mechanical ventilation and vasoactive drug support were performed due to respiratory failure and septic shock. procalcitonin>100 μ g/L; Chest CT (Figure 1) shows multiple patches and consolidation shadows in both lungs, and bronchiectasis and cavity formation can be seen in them; Sputum culture and blood culture were oxacillin-resistant Staphylococcus aureus. Diagnosis of influenza A virus combined with Staphylococcus aureus (Staphylococcus aureus for short) pneumonia. After anti-infective and supportive treatment with vancomycin, linezolid and oseltamivir, the condition improved. After 6 days, the tracheal intubation was removed, and the reexamination of chest CT (Figure 2) showed that the exudation shadow in both lungs was partially absorbed and the cavity lesions increased compared with the previous. The patient was instructed to actively rehabilitate and exercise, excrete sputum, continue anti-Staphylococcus aureus treatment, and change to oral linezolid after discharge. After one month, reexamination of chest CT (Figure 3) showed that the intrapulmonary lesions were significantly improved compared with before, leaving multiple cord shadows and focal bronchiectasis. Staphylococcus aureus is a common pathogen of influenza virus co-infection/secondary bacterial infection, and it is also an important cause of severe illness and death in influenza patients. It can rapidly progress to necrotizing pneumonia with shock. Early identification and timely targeted anti-infective treatment and active supportive treatment can help to improve the prognosis of patients.

The patient, a 40-year-old female, went to the Department of Respiratory and Critical Care Medicine of Peking Union Medical College Hospital in December 2021 due to "intermittent shortness of breath for 27 years". When the patient was 13 years old, bilateral chylopleural effusion was found due to shortness of breath, and "thoracic duct ligation and pleural release" was performed, and the postoperative symptoms were partially relieved. No previous history of tuberculosis infection, no history of asbestos exposure. One month ago (November 2021), I visited the outpatient clinic of our hospital due to aggravation of shortness of breath and light bloody thin phlegm. Chest CT showed right thoracic collapse, bilateral pleural thickening, mediastinal widening with uneven soft tissue density shadow filling in the fat space, and thickening of bronchial vascular bundles and interlobular septa in both lungs (Figures 1 and 2). In November 2021, a CT-guided pleural puncture was performed in a foreign hospital. Pathology showed more irregular proliferation of lymphoid tissue, except lymphatic malformation or lymphangioma. Bronchoscopy in this visit showed vessel filling and inspiratory phase contraction under the tracheal mucosa. The patient did not have obvious manifestations of involvement of extrathoracic organs, and it was highly likely to consider diffuse pulmonary lymphangiomatosis (DPL). Ask the nutrition department to guide the diet, and add sirolimus 1 mg once/d and propranolol 30 mg 3 times/d, orally. The symptoms of the patients were stable after follow-up, and the reexamination of chest CT after 1 year was roughly the same as before. DPL is a rare lymphatic abnormal disease confined to the chest. It is mainly seen in congenital developmental abnormalities, and may also be secondary to trauma, surgery and other factors. Its histopathological features are mainly diffuse dilatation of chest lymphatic vessels with complex lymphatic hyperplasia.