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1.
Zhonghua Jie He He Hu Xi Za Zhi ; 47(3): 269-274, 2024 Mar 12.
Artigo em Chinês | MEDLINE | ID: mdl-38448181

RESUMO

Pharmacologic prophylaxis is the most commonly used prophylaxis for venous thromboembolism (VTE), but the pharmacologic prophylaxis is limited in patients at high risk of bleeding. Mechanical prophylaxis alone or in combination is an important method of VTE prophylaxis in patients at high risk of bleeding, but the current mainstream mechanical prophylaxis, which includes graded compression stockings, intermittent inflatable compression pumps and plantar venous compression pumps, has some limitations, leading to discomfort for patients wearing them due to the large contact area, and even affecting ability to perform daily activities. Many clinical studies have found that NMES combined with pharmacological prophylaxis has better efficacy and safety than pharmacological prophylaxis alone in preventing VTE in medical and surgical patients, and the preventive effect of NMES alone is not inferior to other mechanical prophylaxis. Besides, it also has the advantages of ease of wear and patient compliance. Currently, clinicians have limited experience and knowledge of NMES. We aimed to present the rationale, progress in clinical research and future perspective of NMES in VTE prophylaxis.


Assuntos
Tromboembolia Venosa , Humanos , Tromboembolia Venosa/prevenção & controle , Veias , Estimulação Elétrica
3.
Zhonghua Yi Xue Za Zhi ; 103(42): 3321-3327, 2023 Nov 14.
Artigo em Chinês | MEDLINE | ID: mdl-37963732

RESUMO

Venous thromboembolism (VTE) is a formidable disease that poses a serious threat to the health and well-being of hospitalized patients. Owing to its high incidence, debilitating morbidity, and alarming mortality rates, VTE has gained increasing attention from the clinical medicine community worldwide. Unfortunately, the current state of clinical prevention and treatment of VTE is not very optimistic, necessitating the establishment of large disease-specific databases and real-world studies, which can accumulate effective evidence-based medical evidence to gradually standardize the clinical prevention and treatment and quality control of VTE. The construction and development of large medical databases depend greatly on standardized datasets, which establish the conceptual data models of VTE through data standardization routes, set the object classes according to the model, define the attributes of the classes, standardize the data types and property values, and organize the standardized data elements. This article focuses on providing an in-depth overview of the unique characteristics of various domestic and foreign VTE datasets, describing their application and research progress in VTE, as well as the role of datasets in standardizing clinical and research practices to strengthen quality control and artificial intelligence. Through this comprehensive discussion, we hope to promote the establishment of VTE datasets and enable their use in high-quality large real-world studies.


Assuntos
Tromboembolia Venosa , Humanos , Tromboembolia Venosa/epidemiologia , Anticoagulantes/uso terapêutico , Inteligência Artificial , Fatores de Risco
4.
Zhonghua Jie He He Hu Xi Za Zhi ; 46(7): 720-725, 2023 Jul 12.
Artigo em Chinês | MEDLINE | ID: mdl-37402665

RESUMO

Acute pulmonary thromboembolism (PTE) is a highly fatal disease. Fibrinolytic therapy can rapidly improve pulmonary hemodynamics and is an important life-saving treatment. How to screen patients who may benefit from thrombolytic therapy and how to reduce the complications of major bleeding are still the focus of PTE treatment. In addition, as our understanding of post-PE syndrome (PPES) has improved, much attention has been paid to whether thrombolytic therapy has any benefit in preventing PPES. This article reviewed the research progress of early risk stratification and prognosis assessment, early major bleeding risk assessment, thrombolytic drug dose reduction, interventional thrombolysis and the long-term prognosis of PTE thrombolysis in recent years.


Assuntos
Embolia Pulmonar , Terapia Trombolítica , Humanos , Embolia Pulmonar/tratamento farmacológico , Embolia Pulmonar/etiologia , Fibrinolíticos/uso terapêutico , Hemorragia/induzido quimicamente , Hemorragia/tratamento farmacológico , Prognóstico
5.
Zhonghua Jie He He Hu Xi Za Zhi ; 46(6): 553-557, 2023 Jun 12.
Artigo em Chinês | MEDLINE | ID: mdl-37278168

RESUMO

In recent years, there has been rapid progress in the field of pulmonary hypertension (PH). With the deeper understanding of the pathogenesis of PH, the increase of evidence-based medical evidence, the continuous updating of PH clinical classification, the hemodynamic diagnostic boundaries, and the emergence of new targeted drugs and interventions, the guidelines are constantly being updated. It brings new challenges to the standard diagnosis, treatment and comprehensive management of PH in China. Compared with the world, there are still many problems in the field of PH in China. The heterogeneity of PH causes the complexity of the disease and the difficulty of clinical management, and the early identification and diagnosis of pH face great challenges. Individualized and precise treatment needs to be further optimized, and standardized diagnosis and treatment strategies need to be popularized and promoted. In recent years, rapid progress has been made in the field of PH, including its pathogenesis, diagnostic thresholds, classification and comprehensive treatment methods, prompting an update of the guidelines, which brings a new level of standardized diagnosis and comprehensive management of PH in China. This guideline brings new challenges to the standardized diagnosis and treatment and comprehensive management of PH in China. Here, we discussed in depth the current situation of diagnosis and treatment in the field of PH, as well as the development of a standardized system for PH in China.


Assuntos
Hipertensão Pulmonar , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/terapia , Hipertensão Pulmonar/etiologia , China , Hemodinâmica
6.
Zhonghua Jie He He Hu Xi Za Zhi ; 46(6): 603-609, 2023 Jun 12.
Artigo em Chinês | MEDLINE | ID: mdl-37278177

RESUMO

The early symptoms of chronic thromboembolic pulmonary hypertension (CTEPH) are not specific, and there is a high rate of misdiagnosis, missed diagnosis, and lack of awareness among clinicians. Understanding the current epidemiological characteristics of CTEPH is helpful to raise the understanding level of Chinese clinicians on CTEPH and improve the current status of prevention and treatment. However, epidemiological information and relevant reviews on CTEPH are currently lacking in China. In this review, we combined the published epidemiological literature on CTEPH in the real world, summarized the research overview, prevalence, incidence, survival rate and risk factors of CTEPH, and provided an outlook for the development of multicenter and high-quality CTEPH epidemiological research in China.


Assuntos
Hipertensão Pulmonar , Embolia Pulmonar , Humanos , Embolia Pulmonar/diagnóstico , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/diagnóstico , Doença Crônica , Fatores de Risco , Incidência , Estudos Multicêntricos como Assunto
7.
Zhonghua Jie He He Hu Xi Za Zhi ; 46(5): 460-465, 2023 May 12.
Artigo em Chinês | MEDLINE | ID: mdl-37147807

RESUMO

Objective: To investigate the clinical features and CT findings of pulmonary hypertension (PH) in patients with fibrosing mediastinitis (FM). Methods: Thirteen patients with FM diagnosed between September 2015 and June 2022 were studied retrospectively, including patients with PH (FM-PH group) and patients without PH (FM group) confirmed on right heart catheterization. The t test of two independent samples, Mann-Whitney U rank sum and Fisher's test were used to compare the general information, symptoms, laboratory examination, right ventricular and pulmonary artery measurement data and pulmonary artery CT findings between the two groups, respectively. Results: Compared with the 7 FM patients aged 28-79 (60.00±17.69) years, the 6 patients in the FM-PH group, aged from 60 to 82 (68.83±8.35) years, had more peripheral edema, lower percentage of PaO2, wider inner diameters of pulmonary artery and right ventricle, a higher ratio of right ventricle and left ventricular transverse diameter, faster tricuspid regurgitation velocity and higher estimated systolic pulmonary artery pressure (P<0.05). There were no differences in BNP levels and tricuspid annular plane systolic excursion between groups (P>0.05). Of the 6 patients with PH, 5 had precapillary PH and 1 had mixed PH. Except that the pulmonary vascular resistance in patients of the FM-PH group was significantly higher than that in the FM group (P<0.05), there were no significant differences in cardiac output, mixed venous oxygen saturation and pulmonary capillary wedge pressure between the two groups. CT pulmonary angiography (CTPA) showed pulmonary artery and vein stenosis. Patients in the FM-PH group had more severe stenosis and occlusion of pulmonary artery and pulmonary vein (P<0.05), and more involvement of multiple pulmonary veins (P<0.05). Conclusions: The clinical manifestation of FM complicated with PH is related to the degree of involvement of pulmonary artery, vein and airway. It is recommended that the disease be evaluated in combination with multiple parameters such as clinical manifestations, cardiac ultrasound, right cardiac catheter and CTPA.


Assuntos
Hipertensão Pulmonar , Humanos , Adulto , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Hipertensão Pulmonar/diagnóstico por imagem , Hipertensão Pulmonar/etiologia , Constrição Patológica/complicações , Estudos Retrospectivos , Tomografia Computadorizada por Raios X
8.
Zhonghua Jie He He Hu Xi Za Zhi ; 46(4): 408-412, 2023 Apr 12.
Artigo em Chinês | MEDLINE | ID: mdl-36990706

RESUMO

A 33-year-old male patient was admitted to hospital because of "dyspnea after activity for 3 years and aggravation for 15 days". With a history of membranous nephropathy, irregular anticoagulation led to acute exacerbation of Chronic thromboembolic pulmonary hypertension(CTEPH) and acute respiratory failure, and endotracheal intubation and mechanical ventilation was given. Although treated with thrombolysis and adequate anticoagulation, the condition worsened and hemodynamics deteriorated, and then VA-ECMO was performed. Due to severe pulmonary hypertension and right heart failure,ECMO could not be weaned off, and the patient subsequently developed pulmonary infection, right lung hemorrhage, hyperbilirubinemia, coagulation dysfunction and other complications. Then the patient was transferred to our hospital by airplane, and multidisciplinary discussions were quickly arranged after admission. Considering that the patient was critically ill and complicated with multiple organ failure, pulmonary endarterectomy (PEA) could not be tolerated, rescue balloon pulmonary angioplasty (BPA) was recommended and performed on the second day after admission. The mean pulmonary artery pressure was 59 mmHg(1 mmHg=0.133 kPa) measured by right heart catheterization, and pulmonary angiography showed that the main pulmonary artery was dilated, while the right lower pulmonary artery was completely occluded, and there were multiple stenoses in the branches of the right upper lobe, middle lobe pulmonary artery and the left pulmonary artery. BPA was performed on a total of 9 pulmonary arteries. VA-ECMO was weaned off on day 6 after admission, and the mechanical ventilation was weaned off on day 41 after admission. The patient was successfully discharged on day 72 after admission. Rescue BPA was an effective treatment for severe CTEPH patients who could not be treated with PEA.


Assuntos
Angioplastia com Balão , Oxigenação por Membrana Extracorpórea , Hipertensão Pulmonar , Embolia Pulmonar , Masculino , Humanos , Adulto , Hipertensão Pulmonar/etiologia , Embolia Pulmonar/complicações , Embolia Pulmonar/terapia , Doença Crônica , Artéria Pulmonar , Pulmão , Anticoagulantes , Resultado do Tratamento
9.
Zhonghua Jie He He Hu Xi Za Zhi ; 46(2): 187-191, 2023 Feb 12.
Artigo em Chinês | MEDLINE | ID: mdl-36740382

RESUMO

Venous thromboembolism (VTE) includes pulmonary thromboembolism (PTE) and deep venous thrombosis (DVT). The mortality rate of PTE in China is comparable to the international level, accounting for a significant portion of the global disease burden and a major aspect of respiratory diseases. The research on VTE has made rapid progress in recent years, especially in the VTE prevention, diagnosis strategy, risk stratification, treatment guideline, poor prognosis and complications. Researchers have gradually realized that VTE is a chronic disease involved multi-system. It still needs to be further standardized about the complete flow scheme of the VTE. The article reviewed the latest progress in the field of VTE in the previous year, aiming to provide more medical evidence for the future.


Assuntos
Embolia Pulmonar , Tromboembolia Venosa , Humanos , Tromboembolia Venosa/diagnóstico , Embolia Pulmonar/diagnóstico , Embolia Pulmonar/terapia , Embolia Pulmonar/etiologia , China , Fatores de Risco
10.
Zhonghua Yi Xue Za Zhi ; 103(2): 73-77, 2023 Jan 10.
Artigo em Chinês | MEDLINE | ID: mdl-36597733

RESUMO

In August 2022, the European Society of Cardiology (ESC) and the European Respiratory Society (ERS) jointly issued the 2022 ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension, which updated the hemodynamic diagnostic criteria for pulmonary hypertension, causing widespread debate among experts. How should we respond to the updated diagnostic criteria for pulmonary hypertension? This paper analyzes the origin of the diagnostic criteria for pulmonary hypertension, how to understand the newly revised diagnostic criteria for hemodynamics, the definition of the new criteria for exercise pulmonary hypertension, and the important impact of the new diagnostic criteria on clinical practice, and puts forward countermeasures.


Assuntos
Cardiologia , Hipertensão Pulmonar , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/etiologia , Hemodinâmica
11.
Zhonghua Yi Xue Za Zhi ; 103(0): 707-713, 2023 Jan 10.
Artigo em Chinês | MEDLINE | ID: mdl-36634914

RESUMO

Heparin resistance is becoming a hot issue of clinical concern. In critically ill patients, heparin resistance can lead to failure of anticoagulation therapy or increase the risk of major bleeding. Prompt recognition of heparin resistance can help to precisely adjust heparin dosage and avoid deterioration and adverse events. Heparin resistance can be mechanistically classified into the antithrombin-mediated and the non-antithrombin-mediated. Common etiologies include heparin-induced thrombocytopenia, severe infections such as severe COVID-19, treatment with extracorporeal circulation or extracorporeal membrane oxygenation (ECMO), and use of factor Xa reversal agents; heparin resistance is now often identified by the concordance of activated partial thromboplastin time (APTT) ratio with anti-FXa. Common clinical management strategies include antithrombin supplementation and replacement of anticoagulant drugs (e.g., direct thrombin inhibitors), but their safety and efficacy still need to be further validated.


Assuntos
COVID-19 , Heparina , Humanos , Heparina/uso terapêutico , Heparina/efeitos adversos , Anticoagulantes/uso terapêutico , Antitrombinas , Tempo de Tromboplastina Parcial , Estudos Retrospectivos
12.
Zhonghua Yi Xue Za Zhi ; 102(42): 3333-3337, 2022 Nov 15.
Artigo em Chinês | MEDLINE | ID: mdl-36372766

RESUMO

Hospital-associated venous thromboembolism (HA-VTE) has become a public health problem that seriously affects the quality of medical care and patient safety. Many countries regarded VTE prevention as a key element of medical quality management, carried out quality improvement work in VTE prevention and achieved remarkable results. In China, there is also unremitting efforts and explorations in the field of VTE prevention and management. The national program for prevention and management of pulmonary embolism and deep vein thrombosis was officially launched. Through a series of measures such as excellent center construction, regional alliance, and strengthening of quality control and informatization, the national program has greatly promoted the construction of VTE prevention and management system in hospitals across the country. Strengthening the prevention and treatment of VTE has become the common will of the government, hospital administrators, clinical medical staff and the public. In the future, we need to continue to steadily promote the prevention and management of VTE, and comprehensively improve the quality control management level of VTE prevention and management in hospitals.


Assuntos
Embolia Pulmonar , Tromboembolia Venosa , Humanos , Tromboembolia Venosa/prevenção & controle , Tromboembolia Venosa/tratamento farmacológico , Embolia Pulmonar/tratamento farmacológico , Hospitais , Controle de Qualidade , China , Anticoagulantes/uso terapêutico
13.
Zhonghua Jie He He Hu Xi Za Zhi ; 45(9): 925-930, 2022 Sep 12.
Artigo em Chinês | MEDLINE | ID: mdl-36097930

RESUMO

Thrombosis is the main cause of the development and progression of venous thromboembolism(VTE). Anticoagulant therapy is the cornerstone for the prophylaxis and treatment of VTE, and it has evolved mainly through indirect thrombin inhibitors, direct thrombin inhibitors, vitamin K antagonists and new oral anticoagulants. Although safety is improving, the risk of bleeding remains a non-negligible side effect of current anticoagulation therapy, especially in patients with higher bleeding risk. Studies of the coagulation pathway have found that FXI is not involved in the initiation of hemostasis, but promotes thrombus growth and stabilization primarily through feedback activation of FⅪ by thrombin. Further studies have found that inhibition of FⅪ significantly inhibits thrombus formation and only affects hemostasis slightly. Recent studies have confirmed the efficacy and safety of FⅪ inhibitors in the prevention of VTE in patients after knee replacement. In addition, the safety of FⅪ inhibitors has been further confirmed by the studies of FⅪ inhibitors in people with higher bleeding risk. FⅪ inhibitors may be the most promising anticoagulant drugs in the next decade.


Assuntos
Trombose , Tromboembolia Venosa , Anticoagulantes/uso terapêutico , Fator XI/metabolismo , Fator XI/uso terapêutico , Fibrinolíticos/uso terapêutico , Humanos , Prognóstico , Tromboembolia Venosa/tratamento farmacológico
14.
Zhonghua Jie He He Hu Xi Za Zhi ; 45(6): 588-592, 2022 Jun 12.
Artigo em Chinês | MEDLINE | ID: mdl-35658382

RESUMO

In the past year, significant progress has been made in the field of venous thromboembolism (VTE) including risk assessment and anticoagulation prevention, diagnostic strategies and model exploration, new drug development and disease management. Particularly, major breakthroughs have been made in the prevention of VTE with FXI inhibitors and the prevention of novel coronavirus pneumonia with coagulation alterations and anticoagulation interventions. Here, we reviewed the progress and achievements in the field of VTE in the past year, aiming to provide evidence and ideas for the diagnosis, treatment and future studies of VTE.


Assuntos
COVID-19 , Tromboembolia Venosa , Anticoagulantes/uso terapêutico , Coagulação Sanguínea , Humanos , Fatores de Risco , SARS-CoV-2 , Tromboembolia Venosa/diagnóstico
15.
Zhonghua Jie He He Hu Xi Za Zhi ; 45(2): 204-208, 2022 Feb 12.
Artigo em Chinês | MEDLINE | ID: mdl-35135090

RESUMO

Venous thromboembolism (VTE) is of high incidence and prevalence worldwide. Renal insufficiency has high disease burden with insidious development and is accompanied with disorder of coagulation system. A higher prevalence of VTE has been observed among patients with renal insufficiency whereas VTE patient with renal insufficiency had higher rates of adverse outcomes. Recent evidence indicated that renal insufficiency was an important risk factor for both short and long-term prognosis for VTE. Renal function also affects the choice of anticoagulation therapy and dosage adjustment of drugs. We conducted a comprehensive review of the pathogenesis, mechanism, prognosis and treatment strategy for VTE patients who comorbid renal insufficiency by searching the latest and most advanced national and international articles, to provide integrated information for the prevention and treatment for VTE patients.


Assuntos
Insuficiência Renal , Tromboembolia Venosa , Anticoagulantes/uso terapêutico , Humanos , Incidência , Insuficiência Renal/epidemiologia , Fatores de Risco , Tromboembolia Venosa/tratamento farmacológico , Tromboembolia Venosa/epidemiologia
16.
Zhonghua Zhong Liu Za Zhi ; 43(10): 1100-1104, 2021 Oct 23.
Artigo em Chinês | MEDLINE | ID: mdl-34695902

RESUMO

Objective: To determine the risk profile of venous thromboembolism (VTE) and evaluate VTE prophylaxis implementation of the hospitalized cancer patients in the DissolVE 2 study. Methods: The data of hospitalized cancer patients in the DissolVE 2 study were analyzed. The risk distribution of VTE, preventive measures and in-hospital VTE events of hospitalized patients with tumors were described by percentage and 95% confident interval (CI). Results: A total of 1 535 cancer patients were included. According to the Padua score, 826 (53.8%) patients were at low risk of VTE, while 709 (46.2%) patients were at high VTE risk. VTE events occurred in 4 low-risk patients (0.5%; 95%CI: 0.1%, 1.2%) and 5 high-risk patients (0.7%; 95%CI: 0.2%, 1.6%). The overall incidence was 0.6% (9/1 535, 95%CI: 0.3%, 1.1%). Among patients with high VTE risk, 666 (93.9%) did not receive any VTE prophylaxis, and only 11 (1.6%) patients received appropriate VTE prophylaxis. Among patients who received VTE prevention, no VTE event was observed. Conclusions: Nearly half of the hospitalized cancer patients are at high risk of VTE, but most of them don't receive VTE prophylaxis. The results reflect the insufficient management of VTE risk for hospitalized cancer patients in China, and improvement of awareness and practice of VTE prophylaxis is urgently needed.


Assuntos
Neoplasias , Tromboembolia Venosa , Anticoagulantes/uso terapêutico , Hospitalização , Humanos , Pacientes Internados , Fatores de Risco , Tromboembolia Venosa/epidemiologia , Tromboembolia Venosa/prevenção & controle
17.
Eur Rev Med Pharmacol Sci ; 25(3): 1358-1365, 2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-33629306

RESUMO

OBJECTIVE: This study aimed to investigate the expression of MMP-9 (matrix metalloproteinases 9) and P53 (tumor suppressor proteins, tumor protein P53) in serum of patients with lung cancer. The relation between the two factors and their clinical pathological features were also explored. PATIENTS AND METHODS: 150 patients were selected as the experimental group. They were diagnosed with lung cancer in Shandong Province Chest Hospital from January 2014 to January 2017. Meanwhile, 90 healthy subjects were selected as the control group. The expression levels of MMP-9 and P53 in serum were detected by fluorescence quantitative Real-Time Polymerase Chain Reaction (RT-PCR). The diagnostic value of MMP-9 and P53 in patients with lung cancer was analyzed by receiver operating characteristic curve (ROC). Pearson test was used to analyze the correlation between MMP-9 and P53 in the experimental group. The patients were divided into high expression and low expression groups according to the median of MMP-9 and P53 expressions. RESULTS: The expressions of MMP-9 and P53 in patients with lung cancer were higher than the normal level (p<0.05). MMP-9 and P53 in serum of the experimental group were closely related to patients' TNM-stage, degree of differentiation, lymph node metastases, smoking history and pattern of organization (p<0.05). The expressions of MMP-9 and P53 were positively correlated (p<0.001). CONCLUSIONS: The expressions of MMP-9 and P53 played important roles in lung cancer and were closely related to clinicopathology and three-year survival rate. They could provide help for the diagnosis and treatment of clinical lung cancer.


Assuntos
Neoplasias Pulmonares/genética , Metaloproteinase 9 da Matriz/genética , Proteína Supressora de Tumor p53/genética , Adulto , Idoso , Feminino , Humanos , Neoplasias Pulmonares/metabolismo , Masculino , Metaloproteinase 9 da Matriz/metabolismo , Pessoa de Meia-Idade , Proteína Supressora de Tumor p53/metabolismo
18.
Zhonghua Yi Xue Za Zhi ; 101(1): 7-10, 2021 Jan 05.
Artigo em Chinês | MEDLINE | ID: mdl-33423444

RESUMO

In recent years, pulmonary hypertension (PH) has attracted increasing attention from scholars worldwide, which involves diverse etiology and complicated pathogenesis. Due to changes in the structure and function of pulmonary vasculatures, it can lead to an increase in pulmonary vascular resistance and pulmonary artery pressure, and then progress to right ventricular heart failure or even death. The diagnosis of PH involves multiple disciplines, which could easily give rise to missed diagnosis and misdiagnosis and non-standardized treatment. Recently, we have made great progress in the field of clinical diagnosis, treatment and research of PH. However, many issues remain to be solved. Accordingly, this article aims to call for further strengthening of multidisciplinary collaboration in PH field and promote the construction of a standardized system for PH in China.


Assuntos
Insuficiência Cardíaca , Hipertensão Pulmonar , Disfunção Ventricular Direita , China , Ventrículos do Coração , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/terapia
19.
Zhonghua Bing Li Xue Za Zhi ; 50(1): 38-43, 2021 Jan 08.
Artigo em Chinês | MEDLINE | ID: mdl-33396985

RESUMO

Objective: To describe the clinicopathological features of pulmonary artery intimal sarcoma (PAIS), and to understand its molecular alterations. Methods: Sixty cases of pulmonary artery endarterectomy performed at the China-Japan Friendship Hospital, Beijing, China from January 2017 to January 2020 were reviewed. Clinical data of 5 patients with pulmonary artery intimal sarcoma were collected. Hematoxylin-eosin staining, immunohistochemistry staining and fluorescence in situ hybridization (FISH) were performed to evaluate the pathological features. RNA sequencing was conducted to assess the fusion gene changes in PAIS. Results: The detection rate of PAIS was 8.3% (5/60), with the median age of 49 years and a female predominance. Their clinical manifestations were non-specific. Histopathological examination showed that the tumors were composed of malignant spindle or epithelioid cells, with various degrees of atypia. Focal heterologous osteosarcomatous or leiomyosarcomatous differentiation was noted. The tumor cells could express PDGFRA, CDK4 and MDM2 with co-amplification of MDM2, CDK4 and EGFR genes. RNA sequencing detected multiple in-frame fusions in the tumors. Conclusions: PAIS is a rare, highly heterogeneous, and poorly-or un-differentiated sarcoma accompanied by complex changes of multiple genes.It has no known effective treatments, and thus has a poor prognosis.


Assuntos
Sarcoma , Neoplasias Vasculares , Biomarcadores Tumorais , China , Feminino , Humanos , Hibridização in Situ Fluorescente , Pessoa de Meia-Idade , Artéria Pulmonar/cirurgia , Sarcoma/genética , Sarcoma/cirurgia , Neoplasias Vasculares/genética , Neoplasias Vasculares/cirurgia
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