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Objective:To describe the clinical features and prognosis of congenital anomalous origin of coronary artery(AOCA) in children to increase our understanding of the disease.Methods:This retrospective study included children diagnosed with AOCA using computed tomography coronary angiography(CTCA) admitted to the Department of Cardiology, Beijing Children′s Hospital, Capital Medical University, from January 1, 2014 to December 31, 2019.The clinical presentations, laboratory results, imaging analyses, treatments, and prognoses of these patients were analyzed.Results:A total of 208 children, including 105 boys and 103 girls, we evaluated the ages(9.03±4.18)years old with AOCA.Of these, 157 cases(75.5%) presented with cardiac symptoms, such as chest tightness, palpitations, dizziness, syncope, fatigue, and decreased endurance.Three cases(1.4%) had atypical symptoms of paroxysmal crying, dyspnoea and cyanosis, and 48 cases(23.1%) were asymptomatic.Levels of serological markers of myocardial injury were elevated in 59 cases(28.4%), and 140 cases(67.3%) had predominant ST-T abnormalities on electrocardiograms.Transthoracic echocardiography identified 27 cases (13%) with cardiac enlargement and ten cases(4.8%) with left ventricular systolic dysfunction.There were 126 cases(60.6%) with the anomalous origin of the left coronary artery revealed by CTCA, 50 cases(24.0%) with the anomalous origin of the right coronary artery and 32 cases(15.4%) with bilateral coronary arteries of anomalous origin.Five children underwent surgical treatment, and the remaining 203 children were treated conservatively with drugs.The whole group was successfully treated, and no death case was recorded during the follow-up period.Conclusion:AOCA may cause different degrees of myocardial ischemia.Diverse clinical presentations and diagnostic limitations of transthoracic echocardiography often lead to missed diagnosis or misdiagnosis.In contrast, CTCA has high diagnostic accuracy and can be used to identify the location and course of the coronary ostia.Hence, the management of AOCA should be tailored on a case-to-case basis, taking into consideration of the specific type of coronary origin, with surgical intervention being warranted if necessary.
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Objective:To summarize the clinical characteristics of cardiomyopathy with coronary artery disease in children and explore the application value of multi-slice spiral CT coronary angiography(MSCTA) in the diagnosis and treatment of cardiomyopathy in children.Methods:Patients diagnosed with cardiomyopathy who were hospitalized in the Department of Cardiology at Beijing Children′s Hospital from January 2016 to December 2022 and had complete cardiac imaging data were selected as the study subjects.The demographic characteristics, general clinical and cardiac imaging data of the patients were collected for systematic retrospective analysis.Results:Among 93 patients included in this study, dilated cardiomyopathy was the most common(34.4%, 32/93). MSCTA detected 17 cases(18.3%, 17/93) of high coronary opening, ten cases (10.8%, 10/93) of myocardial bridge, and nine cases (9.7%, 9/93) of coronary disease were detected by echocardiography.Compared with echocardiography, MSCTA had a higher detection rate of abnormal coronary artery origin and myocardial bridge, and the difference was statistically significant( P<0.05). Conclusion:Children′s cardiomyopathy can be complicated with coronary artery disease.MSCTA has great application value in the diagnosis of coronary artery disease.
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Objective:To analyze the various clinical presentations of anomalous aortic origin of a coronary artery (AAOCA) and determine factors related to myocardial ischemia.Methods:Children diagnosed with AAOCA on CT coronary angiography at Beijing Children′s Hospital, Capital Medical University from 1 January 2014 to 31 December 2022 were classified based on AAOCA type, age and high-risk anatomy.The clinical characteristics of the different AAOCA types and age groups were compared and the correlation between the severity of manifestations and high-risk anatomy was analyzed.Results:A total of 69 children with AAOCA[34 males and 35 females, aged (8.89±4.40) years] were included.Ten (14.5%) patients had anomalous origin of the left coronary artery (ALCA) from the right coronary artery sinus and 57 (82.6%) patients had anomalous origin of the right coronary artery (ARCA) from the left coronary artery sinus.In two (2.9%) patients, AAOCA did not arise from a coronary sinus.Nineteen (27.5%) patients were asymptomatic, including 35 (50.7%) cases presented with minor symptoms (chest tightness, chest pain, fatigue), 13 (18.8%) cases had severe symptoms (syncope), and two (2.9%) cases had atypical symptoms (paroxysmal crying). All children were successfully treated; no deaths were reported during follow-up.There were no significant differences in gender, clinical manifestations, positive myocardial injury markers, electrocardiogram and transthoracic echocardiography findings, and proportion of children with high-risk anatomy among the different AAOCA groups (ALCA, ARCA, and non-coronary sinus AAOCA). Divided by age, there were nine (13.1%) children in infant group, 11 (15.9%) in preschool group, and 49 (71.0%) in school age group.More infants were asymptomatic than preschoolers ( P<0.001). Forty-three (62.3%) patients had a high-risk anatomy: two patients with acute take-off angles, four patients with stenosis or slit-like orifices, and 37 patients with interarterial courses.The remaining patients (37.7%) had non-high-risk anatomies.Children with a high-risk anatomy had severe symptoms and were prone to cardiac syncope ( P<0.05). Logistic multivariate analysis suggested that high-risk anatomy was an independent risk factor for cardiogenic syncope( OR=9.026, 95% CI 1.071~76.084, P=0.043). Conclusion:There are no significant differences in the proportion of high-risk anatomy and clinical characteristics among children with different AAOCA types.Younger children are often misdiagnosed due to atypical or insignificant clinical symptoms.The severity of clinical symptoms is related to the high-risk anatomy.High-risk anatomy is an independent risk factor for cardiogenic syncope in children with AAOCA.
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With the advent of the era of big data information and the Internet of things, cloud computing and other new type of continuous development and progress of network technology,the use of digital information had already been penetrated into every field of people’s life and work, mobile medical are derived from the medical field and the Internet technology and the combination of the medical resources,cause a series of new medical reform. Based on the brief introduction of mobile health technology, this paper discuss the advantages, disadvantages, threats and opportunities in the operation process of mobile health technology in our country. this paper puts forward some suggestions and countermeasures from a humanistic and scientific perspective, such as strengthening the subject responsibility, constructing a scientific supervision system, perfecting the legal mechanism, perfecting the top-level design, strengthening moral education and enhancing humanistic quality.
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Objective:To analyze the clinical characteristics of Kawasaki disease(KD)with complications of acute abdominal disease and to improve the diagnosis, treatment and prognosis of patients.Methods:A total of 2 931 cases with KD hospitalized from January 2016 to December 2019 in our department were retrospectively analyzed, including 14 children with acute abdominal disease(acute abdomen group). And 62 patients with KD but without acute abdominal disease were randomly selected as the control group.The clinical characteristics, laboratory examination and treatment between two groups were compared and analyzed.Results:There were 8 males and 6 females.The average age of patients was(4.46±0.74) years.Compared with control group, there was no significant difference in age, sex and proportion of coronary artery injury in acute abdomen group( P>0.05). Acute abdominal disease group were more likely to have gastrointestinal symptoms, intravenous immunoglobulin(IVIG) resistance and longer fever duration( P<0.05). C-reactive protein(CRP), alanine aminotransferase(ALT), aspartate aminotransferase(AST), γ-glutamyltranspeptidase(GGT) and total bilirubin(TBIL) in acute abdominal disease group were significantly higher( P<0.05). There was no significant difference in white blood cell, erythroayte sedimentation rate, hemoglobin, platelet, Na + , albumin between two groups( P>0.05). All of the 14 patients used IVIG, 3 of them were treated with hormone(methylprednisolone)and 1 patient accepted infliximab.All patients were discharged from hospital.After follow-up for 6 months to 3 years, all patients had no sequelae of digestive system. Conclusion:KD can be complicated with acute abdominal disease, some of which as the first symptom.Gastrointestinal manifestations such as abdominal pain are common.For children with obvious gastrointestinal symptoms, IVIG resistance, and long duration of fever, attention should be paid to the possibility of acute abdominal disease.For children with significantly elevated CRP, ALT, AST, GGT, and TBIL should to be alert to the possibility of acute abdominal disease.KD complicated with acute abdominal disease generally has good prognosis.It is important to treat KD in the first place.
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The clinical data of a child with giant coronary aneurysm complicated with myocardial infarction admitted to the Department of Cardiology, Beijing Children′s Hospital, Capital Medical University in February 2019 was retrospectively analyzed.The child, male, aged 2 years and 8 months, presented with symptoms related to cardiac insufficiency.Echocardiography showed giant coronary aneurysm with thrombosis, severe enlargement of the heart, reduction of cardiac function and ventricular aneurysm formation.After anticoagulation, antiplatelet, cardio tonic, diuretic and vasodilator therapy, the clinical symptoms of the patient were slightly relieved, but the ventricular aneurysm gradually expan-ded, and the prognosis was poor.Myocardial infarction is very rare in children, the main cause of which is coronary aneurysm caused by Kawasaki disease.Its early recognition and diagnosis are particularly important, and timely and correct treatment can significantly improve the prognosis.
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Objective:To summarize the changes of serum cytokines in 301 children with Kawasaki disease(KD) and analyze their clinical significance.Methods:Three hundred and one pediatic patients with KD admitted to Beijing Children′s Hospital, Capital Medical University between June 2018 and July 2019 were enrolled and divided into the immunoglobulin sensitive group and immunoglobulin non-response group according to the therapeutic effect.The levels of interferon gamma (IFN-γ), tumor necrosis factor-alpha (TNF-α), interleukin-10 (IL-10), interleukin-6 (IL-6), interleukin-4 (IL-4), and interleukin-2 (IL-2) were compared between the 2 groups.Results:(1)Fifty (16.6%) children had no-response to immunoglobulin.Before treatment, the level of serum cytokines in the immunoglobulin non-response group and the immunoglobulin sensitive group were as follows: IFN-γ[11.32(10.88) ng/L vs. 4.52(5.09) ng/L], TNF-α[7.43(11.25) ng/L vs. 4.42(6.04) ng/L], IL-10[14.44(20.3) ng/L vs. 8.32(7.99) ng/L], IL-6[100.28(126.39) ng/L vs. 41.34(49.32) ng/L], IL-4[1.06(1.79) ng/L vs. 0.98(1.42) ng/L], IL-2[0.48(0.90) ng/L vs. 0.40(0.62) ng/L]; there were significant differences in IFN - γ, TNF - α, IL-10 and IL-6 between the 2 groups (all P<0.05); there was no significant difference of IL-4 and IL-2 between the 2 groups (all P>0.05). (2)Before treatment and after the temperature was normal, the levels of cyto-kines in the immunoglobulin non-response group were as follows: IFN-γ[11.32(10.88) ng/L vs. 1.26(2.10) ng/L], TNF-α[7.43(11.25) ng/L vs. 1.52(3.46) ng/L], IL-10[14.44(20.3) ng/L vs. 4.63(7.00) ng/L], IL-6[100.28(126.39) ng/L vs. 22.85(39.95) ng/L], IL-4[1.06(1.79) ng/L vs. 0(0.25) ng/L], IL-2[0.48(0.90) ng/L vs. 0(0.02) ng/L]. Before treatment and after the temperature was normal, the levels of cytokines in the immunoglobulin sensitive group were as follows: IFN-γ[4.52(5.09) ng/L vs. 0.79(1.53) ng/L], TNF-α[4.42(6.04) ng/L vs. 1.60(3.4) ng/L], IL-10[8.32(7.99) ng/L vs. 3.59(3.74) ng/L], IL-6[41.34(49.32) ng/L vs. 11.90(18.84) ng/L], IL-4[0.98(1.42) ng/L vs. 0(0.88) ng/L], IL-2[0.40(0.62) ng/L vs. 0(0.14) ng/L]. The levels of cytokines decreased significantly after treatment in between 2 groups (all P<0.05). (3) Logistic regression analysis showed that IFN-γ, IL-6, fever duration and albumin were independent risk factors for no-response to immunoglobulin. Conclusions:Children with abnormally high serum IFN-γ and IL-6 levels before treatment are more likely to resist immunoglobulin.Therefore, the change of the condition in such children should be closely monitored.If there is no response to immunoglobulin, immunoglobulin should be used again as soon as possible or second-line drugs should be applied to improve the prognosis.
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Objective@#To investigate the clinical features and improve the diagnosis and treatment of anomalous origin of the left coronary artery from the right coronary sinus with an interarterial course (ALCA-R-IAC) between the ascending aorta and main pulmonary artery in children.@*Methods@#A retrospective analysis of the clinical manifestation, laboratory test, radiological feature, treatment and prognosis were conducted in four female children presented with ALCA-R-IAC in Beijing Children′s Hospital from November 2015 to June 2018.@*Results@#The four girls with onset age of 7.5-14.7 years were diagnosed with ALCA-R-IAC by CT coronary angiography (CTCA). Four children presented with exercise-induced syncope and clinical manifestations of acute myocardial infarction including 3 patients with acute left heart failure, 1 cardiogenic shock and 1 cardiac arrest. Nervous system involvement was found in one patient. Troponin I increased significantly to 20.65-50.00 μg/L in the four patients. Electrocardiogram (ECG) developed signs of left main coronary artery involvement. Echocardiography revealed reduced left ventricular ejection fraction (LVEF) of 25%-45% in three children and suspected anomalous origin of the left coronary artery in one child. CTCA showed an anomalous left coronary artery originating from the right coronary sinus, which had an interarterial course between the aorta and pulmonary artery leading to a slim left main coronary trunk. Two children underwent unroofing procedure and the other two children in whom physical activities were restricted received conservative managements. During a regular follow-up period of 12-43 months, all the children survived without recurrent symptoms and had good prognosis.@*Conclusions@#ALCA-R-IAC can present as exercise-related syncope and acute myocardial infarction, even sudden death in children and adolescents. CTCA is helpful to clarify the early diagnosis of ALCA-R-IAC. Surgical intervention is the main treatment for ALCA-R-IAC and strenuous physical activities should be avoided.
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Objective: To analysis the clinical characteristics and to summarize therapy experience of pediatric patients with cardiac syncope caused by anomalous origin of the left coronary artery from the right sinus (ALCA-R). Methods: We retrospectively analyzed the clinical data including clinical manifestations, myocardial injury biomarkers, radiological features, treatments and prognoses of pediatric patients with ALCA-R who were admitted to Beijing Children's Hospital from November 2015 to June 2018. Results: Four female patients were included in this analysis, age of onset was 7 to 14 years. All the patients presented with exercise-induced syncope and acute myocardial infarction. During the course, three patients presented with acute left heart failure, and one patient had history of sudden cardiac arrest. Laboratory data showed significant elevation of both the creatine kinase and troponin levels in four patients. All electrocardiogram (ECG) showed left main coronary artery occlusion, echocardiography suggested the possible anomalous origin of the left coronary artery in one child. Coronary CT angiography (CTA) revealed there was no coronary ostium in the left coronary sinus, and the left coronary artery had an anomalous origin from the right sinus. The left main coronary artery passed between the ascending artery and the root of the main pulmonary artery, which was compressed by these two large vessels. Two patients underwent cardiac magnetic resonance examination, which detected late gadolinium enhancement in ALCA-R with an interarterial course. Unroofing of the left coronary ostium (cut-back procedure) was performed in two patients, and the other two patients who were not operated were recommended to restrict their physical activities. During a regular follow-up period of 12-43 months, all the children survived without recurrent cardiovascular event. Conclusion: If an adolescent presents with exercise-induced syncope, acute myocardial infarction and even sudden death, and ECG shows left main coronary artery occlusion characteristics, we should consider the possibility of developmental abnormality of coronary artery, particularly the ALCA-R. Once diagnosed as ALCA-R, patients should be recommended to avoid strenuous activities,early recognition and surgical treatment are imperative for these patients.
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Adolescent , Child , Female , Humans , Contrast Media , Coronary Angiography , Coronary Vessel Anomalies , Gadolinium , Retrospective Studies , SyncopeABSTRACT
OBJECTIVE: To investigate the clinical characteristics and treatment strategies of anomalous origin of the cor⁃onary artery accompanied with myocardial damage,so as to improve the level of clinicians' diagnosis and treatment of this disease. METHODS: The clinical data of 72 cases of anomalous origin of the coronary artery accompanied with myo⁃cardial damage admitted in Department of Cardiology,Beijing Children's Hospital,Capital Medical University from2015 to 2017 were analyzed retrospectively. The clinical characteristics,diagnosis and treatment methods were analyzed.RESULTS: In 42 cases(58.3%),chest pain,chest tightness and palpitation were the main clinical symptoms. ECG abnor⁃malities were mainly ST-T changes(62.5%). Echocardiography showed 13 cases(18.1%)of cardiac enlargement,4 cas⁃es(5.6%)of left ventricular systolic dysfunction. Coronary artery CT angiography(CTA)showed that the anomalous ori⁃gin of the left coronary artery was the most frequent(73.6%),followed by the anomalous origin of bilateral coronary arter⁃ies(13.9%),and the anomalous origin of the right coronary artery was the least frequent(12.5%). The whole group was successfully treated with no death. CONCLUSION: There might be a correlation between the anomalous origin of coronary artery and ischemic myocardial damage. In the examination of patients,pediatricians should pay more attention to the judgments of the position and movement of the coronary artery by CTA,and attach importance to the differential diagno⁃sis between ischemic myocardial damage and myocarditis.
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Hypertrophic cardiomyopathy(HCM)is mainly hereditary heart disease caused by gene mutation encoding cardiac sarcomere protein. HCM is the main cause of sudden cardiac death in athletes and adolescents. The clinical manifestations of HCM in children are complex and varied,including being asymptomatic,exercise intolerance,syncope,and sudden death,etc. Labor dyspnea and chest pain are the most common symptoms in older children. The diagnosis of HCM in children is mainly based on imaging examination,which can be divided clinically into three types:obstructive,non-obstructive and occult obstructive. Risk factors were assessed according to the patient's age,clinical symptoms,imaging findings and family history to guide further treatment,management and prognosis.Drug therapy mainly includes beta blockers,non-dihydropyridine calcium antagonists and other anti-arrhythmic drugs,as well as anticoagulants,diuretics and other applications. For those who are not satisfied with the effect of drug treatment,implantable cardioverter defibrillator(ICD)or surgical treatment can be used. In recent years,with the development of gene detection,the diagnosis of HCM in children is more refined and the treatment is accurate.
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A novel Schiff base probe 4'-hydroxy-3'-((2-pyridin-2-ylmethylimino) methyl)-4-biphenyl carbonitrile (HPBC) for dual sensing of Zn2+and CN-was synthesized and characterized by various techniques such as UV-Vis, fluorescence, HRMS and NMR spectroscopy. HPBC was highly selective toward these two ions with different fluorescence signals in two different media. In EtOH-H2O(2:3,V/V;HEPES,pH 7.4),HPBC selectively bound Zn2+to form a 1:1 ligand/metal complex. Addition of Zn2+to the solution of HPBC resulted in a blue shift (△λ=15 nm) with a pronounced fluorescence enhancement at 468 nm, while there was no enhancement in the presence of other metal ions,especially Cd2+. HPBC displayed an"ON-OFF-ON"mode fluorescence change with alternative addition of Zn2+and EDTA. Hence, HPBC is a reversible and reusable sensor for Zn2+. The dynamic range of the assay is linear up to 4.0 μmol/L Zn2+ion and the limit of detection was 36.5 nmol/L,which was thousand fold lower than the WHO guideline (about 76 μmol/L) for drinking water. The fluorescence response of HPBC toward Zn2+was pH-dependent, and the maximal signal was observed at near neutral pH values, which makes it suitable for application in physiological conditions. Cell imaging studies demonstrate that this sensor is capable of sensing Zn2+in living cells. In DMSO-H2O (3:7,V/V) medium,addition of cyanide ion to HPBC led to deprotonation of the phenol hydrogen,resulting in a color change from colorless to pale yellow and a significant fluorescence enhancement at 510 nm. The probe exhibited high selectivity and sensitivity for CN- ion and the detection limit was 5.75×10-7mol/L. Finally,the use of a test strip of probe HPBC to detect cyanide was reported.
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[Objective] To observe the clinical effect ofWen Yang Zhu Shui Formulaecombined with moxa salt packets in the treatment of malignant ascites. [Methods] 61 patients were randomly divided into an observation group(n=31) and a control group(n=30). The patients in the control group were treated with western medicine, while patients in the observation group, based on the treatment of control group, were treated with Wen Yang Zhu Shui Formulaecombined with moxa salt packets for one time a day for two weeks. The effective rates for malignant ascites and Karnofsky performance score(KPS) were compared in the two groups. [Results] The effective rate for malignant ascites in the observation group(80.64%) was higher than that in the control group (53.33%), P<0.05. The observation group showed significantly higher score of KPS than patients of the control group( P<0.05). [Conclusion]Wen Yang Zhu Shui Formulaecombined with moxa salt packets can effectively reduce the malignant ascites.
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The dried whole plant of Pteris dispar were milled and extracted with 95% EtOH. The resulting dried extract was isolated by kinds of chromatographic column, including polyamide, Sephadex LH-20, preparative HPLC. As a result, ten diterpenes were isolated from the plant. By analyzing of ESI-MS and NMR spectroscopic data, the structures were established as geopyxin B(1), geopyxin E(2), ent-11α-hydroxy-18-acetoxykaur-16-ene(3), ent-14β-hydroxy-18-acetoxykaur-16-ene(4), neolaxiflorin L(5), ent-3β,19-dihydroxy-kaur-16-ene(6), ent-3β-hydroxy-kaur-16-ene(7), 7β,17-dihydroxy-16α-ent-kauran-19-oic acid 19-O-β-D-glucopyranoside ester(8), crotonkinin C(9)and crotonkinin C(10). Compounds 1-10 were obtained from P. dispar for the first time. Compounds 1 and 2 showed moderate activities against Bel-7402 with IC₅₀ values of 7.50 and 10.60 μmol•L⁻¹, and against HepG2 with IC₅₀ values of 6.68,11.80 μmol•L⁻¹, respectively.
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This study was aimed to investigate the protective effect and mechanism of β-asarone on PC12 cells injury induced byAβ₁₋₄₂ activated astrocytes, and provide experimental basis for β-asarone application in the prevention and control of Alzheimer's disease (AD). Firstly, RA-h and PC12 cells were co-cultured in the special transwell chamber, and the Real time cell analysis (RTCA) system was used to real-time observe its effect on PC12 cells survival rate in the co-culture system after astrocytes injury induced by Aβ₁₋₄₂. The best intervention time of β-asarone was selected according to the survival curve and parameters generated automatically. β-asarone with different concentrations was used for intervention on astrocytes, then the changes of PC12 cells survival rate in the co-culture system were observed. Secondly, MTT assay was used to detect the effect of Aβ₁₋₄₂ on PC12 cells survival rate as well as the intervention effect of β-asarone, and verify the testing results of RTCA. The levels of IL-1β, TNF-α and BDNF in culture media of the lower chamber were detected by ELISA. The NF-κB activity and phosphorylation levels of ERK, p38 and JNK were detected by Western blot. Results showed that β-asarone (55.5 mg•L⁻¹) could significantly slowdown the decline of PC12 cells survival rate caused by Aβ₁₋₄₂-induced RA-h activation (P<0.01), significantly reduce the levels of IL-1β, TNF-α and the phosphorylation levels of ERK, p38 and JNK in culture media of the lower chamber (P<0.01). β-asarone(166.7 mg•L⁻¹) could promote the release of BDNF in culture media of the lower chamber(P<0.05). These results indicated that Aβ₁₋₄₂ could induce RA-h activation and its release of IL-1β, TNF-α and other inflammatory factors to aggravate the PC12 cells injury; β-asarone could reduce the levels of IL-1β, TNF-α, promote the release of BDNF, and inhibit the NF-κB activity as well as phosphorylation levels of ERK, p38 and JNK protein in PC12 cells.
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AIM: To evaluate the use of 23G minimally invasive vitreous cutting system in the first stage of anterior segment reconstruction on severe ocular injury. METHODS: Fifteen patients with ocular injuries including the corneal and scleral rupture associated hyphema, vitreous hernia in anterior chamber, traumatic lens rupture, lens subluxation, applied 23G minimally invasive vitreous cutting system in the first stage of anterior segment reconstruction. RESULTS: The anterior ocular media of early postoperative became transparent quickly. No exudative lemma and no complications were found relate to puncture incisions after operations. CONCLUSION:23G minimally invasive vitreous cutting system can be used in the first stage of anterior segment reconstruction on sever ocular injury. It can reduce iatrogenic injury, operative complications and inflammatory response, also can shorten therapeutic time and create favorable conditions.
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<p><b>OBJECTIVE</b>To study the diagnostic value and pitfalls of ultrasound-guided core needle biopsy (CNB) of soft tissue tumors.</p><p><b>METHODS</b>One hundred and six cases of CNB specimens encountered during the period from 2007 to 2012 were enrolled into the study. The pathologic diagnosis using CNB was compared with that using surgical specimens. Diagnostic accuracy was analyzed using Chi-square test, with respect to the histologic pattern (such as spindle cell and myxoid), biologic behavior (benign versus malignant) and immunohistochemical results. The 59 cases of sarcoma were subdivided into three grades according to FNCLCC grading system.</p><p><b>RESULTS</b>Histologic diagnosis could be made in 84.0% (89/106) cases. Thirteen cases were non-diagnostic on CNB. There were 4 cases on CNB showing diagnostic discrepancy with surgical specimens. Four cases of "benign lesions" on CNB found to be myxoid liposarcoma and lipoma-like liposarcoma upon resection. In general, myxoid pattern (9/17) seen on CNB showed less diagnostic correlation with surgical specimens, as compared to spindle cell and other histologic patterns (P < 0.01). The rate of diagnostic correlation was 79.7% (49/59) for the 59 cases of sarcoma studied, with grade 2 and grade 3 sarcoma showing better correlation (in contrast to 7/17 for grade 1 sarcoma) (P < 0.01). Comparative analysis showed no significant difference between benign/borderline tumors and sarcomas. The application of immunohistochemical study did not result in significant improvement in diagnostic accuracy on CNB.</p><p><b>CONCLUSIONS</b>Ultrasound-guided CNB is a reliable tool in pathologic diagnosis of soft tissue tumors and shows a high accuracy rate especially for high-grade sarcoma. Tumors with myxoid pattern, lipomatous tumors and grade 1 sarcomas are associated with lower diagnostic accuracy on CNB. Correlation with clinicoradiologic findings would also be helpful in diagnostic evaluation and surgical planning.</p>
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Adolescent , Adult , Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Young Adult , Biopsy, Large-Core Needle , Methods , Diagnostic Errors , Extremities , Liposarcoma, Myxoid , Diagnosis , Diagnostic Imaging , Pathology , Neoplasm Grading , Sarcoma , Diagnosis , Diagnostic Imaging , Pathology , Soft Tissue Neoplasms , Diagnosis , Diagnostic Imaging , Pathology , Ultrasonography, Interventional , MethodsABSTRACT
Objective To assess the CT characteristics of thoracoabdominal localized Castleman disease (LCD) in 55 cases and correlate with histopathologic features. Methods Fifty-five patients with LCD proved histopathologically in thorax ( n = 25 ) and abdomen ( n = 30 ) were collected during past 20 years. The unenhanced and contrast-enhanced CT were performed in all patients. Two radiologists reviewed CT images and the CT findings were analyzed simultaneously. Results In 54 patients with hyaline-vascular type ( n = 50 ) and mixed type ( n = 4 ) localized CD, the lesion typically presented as solitary mass (90. 7% , 49 cases), with irregular or lobular or infiltrative margin ( 83.3% , 45 cases),central calcification (38. 9%, 21 cases), marked enhancement (100% , 54 cases), focal non-necrosis low attenuation areas (72. 2%, 39 cases), lymphadenopathy (70.4%, 38 cases) and dilated feeding vessels adjacent to the mass (96.3%, 52 cases). One lesion with plasma cell type localized CD presented as a mass with irregular margin, mild enhancement and central necrosis. Four morphologic patterns wereclassified on CT, including solitary mass with well-circumscribed margin (n =4), irregular or lobular margin ( n = 30), infiltrative or halo-like margin ( n = 16 ), and multiple coalescent maasses ( n = 5 ). Conclusion CT features of thoracoabdominal localized CD are closely related to the location and pathological type. LCD with hyaline-vascular and mixed type has typical CT characteristics, while LCD with plasma cell type has no typical CT findings.
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<p><b>OBJECTIVE</b>To study the histologic features and immunohistochemical findings of interfollicular stromal cells in hyaline-vascular Castleman's disease (HVCD), and to explore the role of these stromal cells in the pathogenesis of this disease.</p><p><b>METHODS</b>The clinical findings and microscopic features of 23 cases of HVCD cases were reviewed. Immunohistochemical study for CCL21, MSA, CD21, CD35, S-100 and CD34 was carried out.</p><p><b>RESULTS</b>According to the criteria proposed by Danon et al., stroma-rich variant of HVCD contained prominent interfollicular zone which occupied at least 50% of the lymph node area. In the current study, there were 14 cases of stroma-rich HVCD and 9 cases of conventional HVCD. Eleven of the stroma-rich HVCD had paraneoplastic pemphigus and contrastly, no pemphigus lesion obtained in all the 9 cases of conventional HVCD. The association between stromal cell hyperplasia and paraneoplastic pemphigus was statistically significant (P < 0.01).In all the conventional HVCD cases studied, CCL21 and MSA were positive in the stromal cells.The stromal cells in 13 of the 14 cases of the stroma-rich HVCD were also positive for CCL21 and MSA, however, staining for CD21, CD35, S-100 and CD34 was negative in both groups. There was no statistical significance obtained (P > 0.05) between the differences of the staining results.</p><p><b>CONCLUSIONS</b>Stroma-rich HVCD and conventional HVCD represent two distinctive histologic variants and have a different association with paraneoplastic pemphigus. Most of the stromal cells locating in the interfollicular areas are fibroblastic reticular cells in origin, with the immunophenotype as CCL21(+)/MSA(+)/CD34⁻/CD21⁻/S-100⁻. The stromal cells proliferation correlate with the occurrence of paraneoplastic pemphigus, nevertheless, more cases are expected for a further study of the underlying pathogenesis.</p>
Subject(s)
Adolescent , Adult , Female , Humans , Male , Middle Aged , Young Adult , Actins , Metabolism , Blood Vessels , Pathology , Castleman Disease , Metabolism , Pathology , Chemokine CCL21 , Metabolism , Follow-Up Studies , Hyalin , Cell Biology , Lymph Nodes , Pathology , Pemphigus , Metabolism , Pathology , Stromal Cells , PathologyABSTRACT
<p><b>OBJECTIVES</b>To investigate the clinical and pathological subtypes of Castleman's disease (CD) and their relationship with complications.</p><p><b>METHODS</b>The clinical complications of 53 patients with CD and the relationship of these complications with clinical and pathological subtypes were analyzed retrospectively.</p><p><b>RESULTS</b>Among 53 CD patients, 32 (60.4%) were classified as uni-centric type and 21 (39.6%) multicentric type. Histopathological examination showed that 37 cases (69.8%) were hyaline vascular variants (HV), 9 (17.0%) plasmacytic variants (PC), and 7 (13.2%) mixed cellular variants (Mix). Complications were identified in 32 (60.4%) patients, including the involvements of skin, internal organs and hematopoietic system. Some complications were closely associated with the clinical subtype of CD: the majority of complications in the 32 uni-centric CDs were paraneoplastic pemphigus (PNP) and bronchiolitis obliterans (BO), and those in 21 multi-centric CDs were the involvements of kidney and hematopoietic system. The complications were different among the three kinds of histopathological subtypes: PNP and BO were the predominant complications of HV variants, while the internal organ and hematopoietic system involvements were those of PC and Mix variants. The clinical and histopathological classification of CD patients with PNP were different obviously from other subtypes of CDs. In Kaplan-Meier survival analysis, the survival rate of those with complications was significantly lower than those without complication (P = 0.028).</p><p><b>CONCLUSION</b>The clinical complications of CDs are related to their clinical and histopathological subtypes. CD patients with PNP should be considered as a unique entity to tailor the therapy. The presence of clinical complications is an independent prognostic factor in CD patients.</p>