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Objective: To summarize the clinical features and prognosis of Budd-Chiari syndrome with hepatopulmonary syndrome (HPS) in children. Methods: The clinical data of a child who had Budd-Chiari syndrome with HPS treated at the Department of Pediatrics of the First Affiliated Hospital of Zhengzhou University in December 2016 was analyzed retrospectively. Taking "Budd-Chiari syndrome" and "hepatopulmonary syndrome" in Chinese or English as the keywords, literature was searched at CNKI, Wanfang, China Biomedical Literature Database and PubMed up to July 2023. Combined with this case, the clinical characteristics, diagnosis, treatment and prognosis of Budd-Chiari syndrome with HPS in children under the age of 18 were summarized. Results: A 13-year-old boy, presented with cyanosis and chest tightness after activities for 6 months, and yellow staining of the skin for 1 week. Physical examination at admission not only found mild yellow staining of the skin and sclera, but also found cyanosis of the lips, periocular skin, and extremities. Laboratory examination showed abnormal liver function with total bilirubin 53 μmol/L, direct bilirubin 14 μmol/L, and indirect bilirubin 39 μmol/L, and abnormal blood gas analysis with the partial pressure of oxygen of 54 mmHg (1 mmHg=0.133 kPa), the partial pressure of carbon dioxide of 31 mmHg, and the alveolar-arterial oxygen gradient of 57 mmHg. Hepatic vein-type Budd-Chiari syndrome, cirrhosis, and portal hypertension were indicated by abdominal CT venography. Contrast-enhanced transthoracic echocardiography (CE-TTE) was positive. After symptomatic and supportive treatment, this patient was discharged and received oxygen therapy outside the hospital. At follow-up until March 2023, there was no significant improvement in hypoxemia, accompanied by limited daily activities. Based on the literature, there were 3 reports in English while none in Chinese, 3 cases were reported. Among a total of 4 children, the chief complaints were dyspnea, cyanosis, or hypoxemia in 3 cases, and unknown in 1 case. There were 2 cases diagnosed with Budd-Chiari syndrome with HPS at the same time due to respiratory symptoms, and 2 cases developed HPS 1.5 years and 8.0 years after the diagnosis of Budd-Chiari syndrome respectively. CE-TTE was positive in 2 cases and pulmonary perfusion imaging was positive in 2 cases. Liver transplantation was performed in 2 cases and their respiratory function recovered well; 1 case received oxygen therapy, with no improvement in hypoxemia; 1 case was waiting for liver transplantation. Conclusions: The onset of Budd-Chiari syndrome with HPS is insidious. The most common clinical manifestations are dyspnea and cyanosis. It can reduce misdiagnosis to confirm intrapulmonary vascular dilatations with CE-TTE at an early stage. Liver transplantation is helpful in improving the prognosis.
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Male , Humans , Child , Adolescent , Budd-Chiari Syndrome/therapy , Hepatopulmonary Syndrome/therapy , Retrospective Studies , Hypoxia/complications , Oxygen , Dyspnea/complications , Cyanosis/complications , BilirubinРеферат
Objective:To assess the effectiveness and safety of beat chemotherapy in treating non-small cell lung cancer, and to investigate its anti-tumor molecular mechanism.Methods:In this study, we developed a subcutaneous tumor model of lung cancer in mice.The mice were subsequently divided into two groups: the beat chemotherapy group and the placebo group(negative control group).Throughout the treatment period, we monitored the changes in body weight and tumor size of the mice.At the conclusion of the treatment, we collected blood samples from the mice to conduct blood routine and biochemical examinations.Furthermore, we obtained tumor tissues from the mice to perform immunohistochemical staining and sequencing of the transcriptome.Results:The study found that beat chemotherapy could effectively delay the growth of lung cancer.The tumor tissues in the beat chemotherapy group were significantly smaller compared to the placebo group.The results of routine blood and blood biochemistry tests showed that the levels of red blood cells(RBCs), white blood cells(WBCs), alanine aminotransferase(ALT), aspartate aminotransferase(AST)and blood creatinine(Scr)were similar between the placebo group and the beat chemotherapy group.The values for RBCs, WBCs, ALT, AST and Scr in the placebo group were(6.97 ± 0.41)× 10 12/L, (13.26 ± 0.29)× 10 9/L, (33.33 ± 2.51)U/L, (235.33 ± 57.62)U/L and(20.67 ± 2.08)μmol/L, respectively.The corresponding values in the beat chemotherapy group were(6.87 ± 0.66)× 10 12/L, (12.59 ± 2.27)× 10 9/L, (38.67 ± 3.79)U/L, (225.33 ± 6.81)U/L and(20.33 ± 3.79)μmol/L.Statistical analysis showed no significant differences between the two groups( t=0.509, 0.209, 2.032, 0.299, 0.134, P=0.638, 0.845, 0.112, 0.780, 0.900).Furthermore, there were no signs of inflammatory infiltration or pathological changes in the liver, kidney, spleen, and lung tissues of the mice.Transcriptome analysis identified 68 differentially expressed genes, which were mainly associated with signal transduction and immunity.Kyoto Encyclopedia of Genes and Genomes(KEGG)pathway enrichment analysis revealed the involvement of several signaling pathways, including the transforming growth factor β(TGF-β)signaling pathway, the interleukin-17(IL-17)signaling pathway, and the tumor necrosis factor(TNF)signaling pathway. Conclusions:The use of chemotherapy has been proven to be safe and effective in treating non-small cell lung cancer.It primarily functions by regulating tumor growth through various signaling pathways, including the TGF-β signaling pathway, IL-17 signaling pathway, and TNF.
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Objective:To evaluate the efficacy and safety of Burosumab in patients with X-linked hypophosphatemic rickets.Methods:Clinical data of 9 children diagnosed with X-linked hypophosphatemic rickets and treated with Burosumab in the Department of Pediatric Nephrology, Anhui Children′s Hospital from November 2021 to September 2023 were retrospectively analyzed, including the general information, clinical manifestations, auxiliary examination, Burosumab treatment and follow-up.Results:Among the 9 cases, there were 5 males and 4 females, with a median age at diagonosis of 2 years. After traditional treatment, the fluctuation of serum phosphorus ranged from 0.7 to 0.9 mmol/L. The median age at the initiation of Burosumab treatment was 2.8 years, and the initial dosage was 0.8 mg/kg, administrated subcutaneously every 2 weeks. The laboratory and imaging indexes were improved after 6 months of Burosumab treatment, and the mean serum phosphorus level increased from(0.81±0.14) mmol/L to(1.02±0.10) mmol/L at 1 month( t=3.85, P=0.001) and(1.14±0.25) mmol/L at 6 months( t=3.58, P=0.002). The average alkaline phosphatase(ALP) level decreased from(509.89±110.10) U/L before treatment to(447.89±106.76) U/L after 1 month( t=1.21, P=0.243). After 6 months, the ALP level significantly decreased to(385.89±60.33) U/L ( t=2.96, P=0.009). The average height percentile increased from 18.42±10.09 before treatment to 26.56±16.59 after 6 months( t=1.26, P=0.227). Rachitis severity scores of both lower limbs ranged from 4.61±1.36 before treatment to 3.06±1.51 after 6 months( t=2.29, P=0.036). No serious adverse events occurred during treatment. Conclusion:Burosumab is safe and effective in treating X-linked hypophosphatemic rickets, exhibiting minimal side effects and significant clinical applicability value.
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Objective:To develop a recombinant protein vaccine based on KPC-2, a drug resistance target in Klebsiella pneumoniae, and evaluate its immunogenicity, protective efficacy and mechanism in a mouse model of pneumonia. Methods:KPC-2 was expressed in Escherichia coli and purified using GST affinity chromatography. A recombinant protein vaccine was prepared with KPC-2 and used to immunize New Zealand rabbits through subcutaneous injection. Serum samples were isolated from cardiac blood and Protein G chromatography was used to purify polyclonal antibodies against KPC-2. Opsonophagocytic killing assay was used to assess the bactericidal activity of the polyclonal antibodies in vitro. Female BALB/c mice were immunized three times with the recombinant protein vaccine, and the titers of specific IgG antibodies in serum were measured by indirect ELISA. One week after the last vaccination, the mice were infected with Klebsiella pneumoniae strain SRT through tracheal intubation, and received a single intravenous dose of meropenem (0.1 mg) 1 h later. The protective efficacy of the KPC-2 recombinant protein vaccine was evaluated by comparing the survival rates, bacterial colonization and histopathological changes between vaccine group and adjuvant group as well as the survival rates between meropenem group and normal saline group. Moreover, the protective efficacy of polyclonal antibodies against KPC-2 was evaluated through passive immunization. Results:The level of specific IgG antibodies in serum was significantly higher in the vaccine group than in the adjuvant group ( t=4.325, P<0.05). The survival rate in the vaccine group was also higher than that of the adjuvant group [70% (7/10) vs 10% (1/10), P<0.05]. Furthermore, lung inflammation was less severe and bacterial burden was reduced in the vaccine group as compared with those of the control group ( t=3.127, P<0.05). Both active and passive vaccination strategies demonstrated strong protective efficacy against Klebsiella pneumoniae infection, and had a synergistic effect when used in combination with antibiotic therapy. The polyclonal antibodies against KPC-2 had bactericidal activity in vitro ( t=5.427, P<0.05). Conclusions:The prepared KPC-2 vaccine has better immunogenicity and protective efficacy. It can induce strong humoral immune responses. This study suggest that drug resistance target may be used as a candidate antigen for future vaccine development.
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Objective:To construct a training course system for online appointment nurses based on post competence,and to provide references for the training and development of online appointment nurses in China.Methods:From January 2021 to May 2022, based on the theory of post competence, literature review, policy research, questionnaire survey and and expert consultation were used to establish the training course system of online appointment nurses.Results:The effective recovery rates of the two rounds of expert consultation questionnaires were 18/18 and 17/18, respectively; the expert authority coefficient was 0.928 and 0.938, respectively; and the Kendall harmony coefficient was 0.185 and 0.284, respectively, the differences were statistically significant (all P<0.001). The final content of the training course system for online appointment nurses included 4 first-level indicators,which were professional knowledge, professional skills, professional abilities and traits, 19 second-level indicators and 58 third-level indicators. Conclusions:The training course system of online appointment nurses based on post competency is scientific, reasonable and prominent,which can provide reference for training of online appointment nurses in China.
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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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Cardiogenic syncope in children is common in clinic and is highly regarded because of its high risk of sudden death.The main causes of cardiogenic syncope in children are organic and arrhythmia.In the diagnosis of syncope in children, the clinical characteristics and the primary diagnosis of the etiology of cardiogenic syncope should be emphasized.Holter electrocardiogram and intracardiac electrophysiology are indispensable in arrhythmia syncope.Cardiac ultrasound and cardiac MRI in syncope with structural heart disease is importance.Genetic testing is highly recognized.Different causes of cardiogenic syncope should be treated in different ways.The aim of this review was to improve the ability of the clinician to identify cardiogenic syncope quickly and accurately, so as to improve the treatment of such children.
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Objective:To investigate the pathogenic genes, clinical features and treatment as well as follow-up of children with congenital long QT syndrome (LQTS).Methods:The clinical data, genetic test results and follow-up data of 16 congenital LQTS children with syncope as the first manifestation admitted to the Department of Cardiology, Beijing Children′s Hospital Affiliated to Capital Medical University from August 2016 to March 2023 were collected and retrospectively analyzed.Results:Among the 16 LQTS patients, the age of first syncope onset was 1.3-13.3 (7.37±3.41) years, and the interval between first syncope onset and clinical diagnosis was 0-48 (14.8±16.2) months.A total of 13 (81.3%) patients had triggers of syncope, of which nine were exercise-induced and four were emotional induced.Genetic testing was performed in 13 patients with LQTS, of which 12 (92.3%) were found to have pathogenic or suspected pathogenic mutations from KCNQ1, KCNH2, and SCN5A gene.The corrected QT interval of 16 patients was (550.0±50.2) ms, all cases≥460 ms.Schwartz scored 6.0 (5.0, 6.0) points, all cases≥4 points.All patients were initially treated with metoprolol or propranolol, of which 14 patients were followed up to date, three patients had recurrent syncope, and five patients stopped taking the medicines by themselves.One patient with high-dose metoprolol (LQT2) was treated with mexiletine after recurrent episodes.One patient who was intolerant to high-dose propranolol underwent left cardiac sympathectomy and was followed up after surgery without syncope episodes.None of the patients underwent implantable cardioverter defibrillator implantation. Conclusion:Children with LQTS and syncope symptoms have high positive rate of genetic tests.The genetic results could assist typing of patients with LQTS and guide treatment.Routine electrocardiogram screening in children with syncope may diagnose LQTS earlier and reduce misdiagnosis and missed diagnosis.β-blockers are the cornerstone of treatment for patients with LQTS.Strengthening follow-up management and improving patients′ treatment compliance is conducive to further improving the treatment response rate of patients.
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Objective:To analyze the clinical features and follow-up of children with cardiogenic syncope (CS), and accurately and efficiently guide clinical diagnosis as well as improve the prognosis of children with CS.Methods:Ninety-eight children with CS who were hospitalized in the Department of Cardiology, Beijing Children′s Hospital Affiliated to Capital Medical University from April 1, 2016 to June 31, 2023 were selected as the study objects.According to the etiology type, the children with CS were divided into arrhythmia group, organic cardiovascular disease group and mixed group.The causes of syncope episodes, type of aura, frequency of syncope at first diagnosis, duration of loss of consciousness, concomitant symptoms, past history, family history, physical examination and follow-up were collected and statistically analyzed in each group.Results:A total of 98 children with CS were included, including 59 males and 39 females.The age of first onset was (8.69±3.90) years old.There were 60 cases in arrhythmia group, 18 cases in organic cardiovascular disease group and 20 cases in mixed group.There were no statistically significant differences among three groups of children in whether had inducement, whether had aura, incidence of aura types, duration of loss of consciousness, incidence of urinary and fecal incontinence and associated symptoms of fall injury, incidence of liver macrosis, and recurrence of syncope during follow-up.The children in arrhythmia group were more likely to induce syncope due to intense exercise than those in mixed group ( χ2=9.785, P<0.05). Compared with the organic cardiovascular disease group and the mixed group, the number of syncope attacks in the arrhythmia group was more than five times at the first diagnosis ( P=0.020). Compared with the organic cardiovascular disease group, the children in mixed group and arrhythmia group were more likely to have accompanying symptoms during syncope( P<0.05), and the incidences of convulsion were the higher in both groups.The positive signs of heart in mixed group were more than those in arrhythmia group and organic cardiovascular disease group( P<0.05). Compared with arrhythmia group, facial cyanosis was more common in mixed group and organic cardiovascular disease group ( P<0.05). Of the 87 children with CS who were followed up regularly, 73 (83.9%) did not have recurrent syncope after timely treatment and regular outpatient medication adjustment. Conclusion:Children with CS have special clinical characteristics, such as syncope is easily induced by strenuous exercise or emotional excitement, syncope is often preceded by no aura of seizure, loss of consciousness lasts for a relatively short period of time, the main accompanying symptom of syncope is convulsions, positive cardiac signs can be seen on physical examination, and there can be cardiac disorders in the past history or sudden death in the family history.It is of great significance to improve the diagnosis and prognosis of children with CS by mastering its characteristics and giving timely and appropriate treatment.
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Objective:To survey the job satisfaction of online contracted nurses who provide " Internet plus nursing services" for reference of hospital managers in improving their management mechanism in this regard.Methods:Based on the two-factor theory, a questionnaire was designed and a purposive sampling method was used to survey the online contracted nurses in Anhui province in April and May 2022. The motivational factors included such five dimensions as workload, work content, colleague relationship, doctor-patient relationship, and their own development, and the healthcare factors included such three dimensions as salary, job recognition and social status. The questionnaire data and job satisfaction scores were analyzed descriptively, and the correlation between the overall job satisfaction of the online contracted nurses, while each dimension was analyzed by Pearson correlation analysis, and the influence of each dimension on job satisfaction was analyzed by stepwise regression analysis.Results:A total of 335 valid questionnaires were recovered. The mean score of job satisfaction of online contracted nurses was (2.26±0.38), with the highest score of (2.56±0.53) for salary satisfaction and the lowest score of (1.78±0.67) for job recognition, and each dimension was positively correlated with job satisfaction ( r=0.34-0.88, P<0.01). Regression analysis showed that workload ( B=0.07), salary ( B=0.11), job content ( B=0.23), social status ( B=0.12), and self-development ( B=0.15) were the main factors affecting their job satisfaction ( P<0.01). Conclusions:The job satisfaction of online contracted nurses was at a medium level, mainly influenced by workload, salary, job content, social status and their own development. It is recommended that hospitals implement multiple targeted measures to improve the job satisfaction of online contracted nurses and promote the healthy development of " Internet plus nursing services" .
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Objective: To evaluate the household secondary attack rates of the SARS-CoV-2 Delta variant and the associated factors. Methods: A COVID-19 outbreak caused by the Delta variant occurred in Nanjing in July 2021. A total of 235 cases with current addresses in Nanjing were reported from 171 households. The subjects in this study were selected from household close contact(s) of infected cases. The information on household index cases and their contacts were collected, and the household secondary attack rate (HSAR) and the risk factors were analyzed by the multi-factor logistic regression model. Results: A total of 234 cases of household close contacts and 64 household secondary cases were reported from 103 households, and the HSAR was 27.4% (64/234, 95%CI:22.0% to 33.4%). The proportions of household size for 2 to 3, 4 to 5, and 6 to 9 were 64.1% (66), 26.2% (27) and 9.7% (10), respectively. A total of 35 cases of household cluster outbreaks were reported (35/103, 34.0%). The number of the first case in the household (FCH) was 103 and males accounted for 27.2% (28 cases), with the median age (Q1, Q3) of 49 (9, 56). The number of household close contacts was 234 and males accounted for 59.0% (138 cases), with the median age (Q1, Q3) of 42 (20, 55) and the median exposure period (Q1, Q3) of 3 (1, 3) days. The multi-factor logistic regression model showed that the higher HSAR was observed in the FCH with the features of airport staff (OR=2.913, 95%CI:1.469-5.774), detection from home quarantine screening (OR=6.795, 95%CI:1.761-26.219) and detection from mass screening (OR=4.239, 95%CI:1.098-16.368). Meanwhile, higher HSAR was observed in cases with longer household exposure (OR=1.221, 95%CI:1.040-1.432), non-vaccination (OR=2.963, 95%CI:1.288-6.813) and incomplete vaccinations (OR=2.842, 95%CI:0.925-8.731). Conclusion: The generation interval of the Delta variant is shortened, and the ability of transmission within the household is enhanced. In the outbreak in Nanjing, the associated factors of HSAR are occupation, detection route, vaccination and exposure period.
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Male , Humans , SARS-CoV-2 , COVID-19/epidemiology , Incidence , Family CharacteristicsРеферат
【Objective】 To investigate the effect of isoliquiritigenin on inflammatory response of vascular endothelial cells and whether the regulatory effect of isoliquiritigenin on inflammation is mediated by histone deacetylase 3 (HDAC3). 【Methods】 Human umbilical vein endothelial cells (HUVECs) were cultured in vitro and treated with LPS, different concentrations of isoliquiritigenin and HDAC3 specific inhibitor, respectively. Real-time PCR and Western blotting were used to detect the mRNA and protein expressions of inflammatory cytokines and HDAC3. Male C57BL/6J mice were randomly divided into vehicle group and isoliquiritigenin treatment group. The vascular inflammation model of C57BL/6J mice was established by ligation of the left carotid arteries. The mRNA expressions of inflammatory cytokines and HDAC3 in the carotid arteries of mice were detected by Real-time PCR. A molecular docking study was performed to investigate the interaction between isoliquiritigenin and HDAC3. 【Results】 Compared with the vehicle group, isoliquiritigenin reduced the mRNA expressions of inflammatory cytokines NLRP3, IL-1β, IL-18, MCP-1 and ICAM-1 and decreased the expression of HDAC3 mRNA and protein in HUVECs stimulated with LPS. In addition, isoliquiritigenin also decreased the mRNA expressions of NLRP3, IL-1β and HDAC3 in carotid arteries of ligated C57BL/6J mice. The docking of isoliquiritigenin in the active site of HDAC3 showed that isoliquiritigenin might act through HDAC3. Furthermore, HDAC3 specific inhibitor RGFP966 further promoted the inhibitory effect of isoliquiritigenin on the expression of inflammatory cytokines in vascular endothelial cells. 【Conclusion】 These results suggest that isoliquiritigenin suppresses the inflammatory response of vascular endothelial cells via HDAC3.
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Heart failure is the end-stage clinical manifestation of various functional or structural heart diseases, which is an acute and critical condition in the field of pediatrics.Arrhythmias are often complicated with heart failure in children and could also trigger heart failure exacerbation, affecting the prognosis of children.The clinical manifestations of arrhythmias in children are atypical except the malignant types, and children with heart failure should always be alerted to the possibility of arrhythmias.Therefore, it is important for improving the prognosis of children with heart failure by raising awareness of these diseases, thus making a rational assessment of disease and actively managing it.
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Objective:To investigate the effect of drug therapy on heart failure caused by supraventricular tachycardia(SVT) in infants.Methods:Fifty-five infants with heart failure caused by SVT, including 24 boys and 31 girls, were treated at Beijing Children′s Hospital of Capital Medical University from January 2014 to December 2021.The drug treatment effects of heart failure caused by SVT were analyzed.Results:The average age of 55 infants at the first diagnosis was 5.8 months(1-11 months). All of them had heart failure, including three cases of atrial flutter, 23 cases of atrial tachycardia(13 cases of disordered atrial tachycardia and ten cases of monomorphic atrial tachycardia), and 29 cases of paroxysmal supraventricular tachycardia.Ultrasonic cardiogram showed that the left ventricular diameter increased and/or left ventricular systolic function decreased.Anti-heart failure therapy was effective in 55 cases(100.0%). Anti-arrhythmic drug therapy: atrial flutter and atrial tachycardia were mainly treated by controlling ventricular rate, digitalis combined with metoprolol was effective in 21 cases(80.8%, 21/26), digitalis alone was effective in four cases(15.4%, 4/26), and sotalol was effective in one case(3.8%, 1/26); paroxysmal supraventricular tachycardia was mainly treated with sinus rhythm conversion.The success rates of conversion were: ATP 20.7%(6/29), ATP combined with digitalis 26.1%(6/23), propafenone combined with digitalis 42.9%(3/7), amiodarone combined with digitalis 60.0%(3/5), and sotalol 92.9%(12/13). During follow-up period for 1 to 12 months, heart failure symptoms of all 55 cases(100.0%) improved, and ultrasonic cardiogram of 53 cases(96.3%)returned to normal.Conclusion:Anti-heart failure and anti-arrhythmic drugs for infants with heart failure caused by SVT need to be selected individually.Atrial tachycardia, especially disordered atrial tachycardia, is sensitive to digitalis.Sotalol can be used to treat refractory SVT.
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Objective:To investigate the effect of radiofrequency ablation on infants with incessant ventricular tachycardia(IIVT) and heart failure.Methods:Twenty-eight infants with IIVT combined with heart failure admitted to Beijing Children′s Hospital of Capital Medical University from January 2006 to December 2021 were selected, including 16 boys and 12 girls; 26 cases were treated with radiofrequency ablation.The characteristics of IIVT and heart failure and the results of radiofrequency ablation were analyzed.Results:The average age of the first diagnosis of 28 infants was 13.9 months old, and all of them had heart failure.Eleven infants had cardiogenic shock, three infants had cardiogenic syncope, two infants had respiratory failure for respiratory support, and one infant died.Color Doppler echocardiography showed that the left ventricular diameter increased and/or left ventricular systolic function decreased.Anti-heart failure treatment was effective in 27 cases(96.4%), electrical cardioversion in five cases, effective in three cases, and anti-arrhythmic drugs were effective in 17 cases(60.7%). Twenty-six cases(92.9%, 26/28) were treated with radiofrequency ablation, with immediate success in 23 cases(88.5%, 23/26) and effective in three cases(11.5%, 3/26). During the follow-up period for 3 to 36 months, cardiac function returned to normal in 25 cases(96.2%, 25/26) and recurred in three cases(11.5%, 3/26 cases), which were cured after radiofrequency ablation again.Temporary complications of atrioventricular block occurred in one case(3.8%, 1/26). In 26 cases of surgical children, 15 cases were measured by X-ray two-dimensional mapping and 11 cases were measured by three-dimensional mapping.The cumulative X-ray exposure was 87.0(51.5, 151.5) mGy and 1.2(0, 15.9) mGy, respectively, and the dose area product was 39.8(19.2, 427.8) μGy/m 2 and 2.8(0, 44.3) μGy/m 2.The cumulative X ray exposure and the dose area product were significantly reduced under the three-dimensional mapping method( P<0.001). Conclusion:The infants with IIVT combined with heart failure are prone to serious complications, and the effects of cardioversion and anti-arrhythmic drugs are limited.Radiofrequency ablation should be performed as soon as possible after heart failure is controlled.The application of three-dimensional mapping technology in surgery can significantly reduce ionizing radiation.
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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 compare the clinical effect of "de-sharpening" intramedullary elastic reduction with Kirschner wire and traditional three-dimensional manipulation for the treatment of Gartland type III posterolaterally-displaced supracondylar humerus fracture in children.Methods:A retrospective cohort analysis was made on 106 children with Gartland type III posterolaterally-displaced supracondylar humerus fracture treated in Anhui Provincial Children′s Hospital from March 2020 to June 2022, including 58 males and 48 females; aged 1-12 years [(8.7±2.3)years]. The patients were assigned to "de-sharpening" intramedullary elastic reduction with Kirschner wire (study group, n=50) and traditional three-dimensional manipulation (control group, n=56). The operation time, frequency of intraoperative fluoroscopy, fracture healing time, difference of Baumann angle between the normal and injured side at postoperative 3 and 6 months, elbow function Flynn score at last follow-up and complications were compared between the two groups. Results:All children were followed up for 6-12 months [(8.5±1.2)months]. The operation time and frequency of intraoperative fluoroscopy were (32.9±3.7)minutes and (20.6±5.4)times in study group, significantly different from (45.6±10.1)minutes and (32.5±8.2)times in control group (all P<0.05). The fracture healing time was (33.0±5.1)days in study group, similar with (33.8±4.7)days in control group ( P>0.05). At 3 and 6 months after operation, the difference of Baumann angle between the normal and injured side was (3.2±0.8)°and (2.3±0.6)°in study group compared to (6.0±2.1)°and (5.8±1.3)°in control group (all P<0.01). According to the elbow function Flynn score at the last follow-up, the results were excellent in 44 children, good in 5 and fair in 1, with the excellent and good rate of 98.0% (49/50) in study group, and were excellent in 47 children, good in 5 and fair in 4, with the excellent and good rate of 92.9% (52/56) in control group ( P>0.05). There were no following complications in both groups, such as osteomyelitis, poor fracture healing, compartment syndrome, iatrogenic neurovascular injury or myositis ossificans. Conclusions:Both "de-sharpening" intramedullary elastic reduction with Kirschner wire and traditional three-dimensional manipulation for the treatment of Gartland type III posterolaterally-displaced supracondylar humerus fracture in children can achieve elbow joint function recovery, with low incidence of complications. However, the former avoids repeated manual reduction, with shorter operation time, less frequency of intraoperative fluoroscopy and better correction of the coronal plane deformity and rotation deformity.
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Objective:To investigate the predictive value of the epithelial cell proliferation(ECP)pathway genes for the prognosis of elderly non-small cell lung cancer patients treated with immunotherapy.Methods:A total of 106 elderly patients aged 70 years and over receiving immunotherapy in the POPLAR and OAK clinical trials were retrospectively analyzed in October 2022.According to the mutation status, patients were divided into an ECP pathway-related gene mutation group(ECP mutation group, n=25)and an ECP pathway-related gene non-mutation group(ECP non-mutation group, n=81). The primary endpoints were overall survival(OS)and progression-free survival(PFS). Differences in survival and efficacy between the two groups were compared, and subgroup analysis was performed on clinical factors and genes involved in the pathway.Pyclone was used to calculate the distribution of major clones and subclones in each patient, and differences in survival were compared.Results:Survival outcomes were worse in the ECP(+ )group than in the ECP(-)group(mOS: 10.9 months vs.17.1 months, HR=1.84, 95% CI: 1.09-3.08, P<0.05; mPFS: 2.8 months vs.4.2 months, HR=1.58, 95% CI: 1.00-2.51, P<0.05). Of all mutations in ECP pathway-related genes, mutations in the RB1 gene had a significant prognostic effect on all patients, with the negative prognostic effect especially prominent in ECP(+ )patients.Compared with ECP(-)patients, ECP(+ )patients had a shorter mOS(6.9 months vs.12.6 months, HR=3.14, 95% CI: 1.10-8.97, P=0.024). Ten patients had clonal mutations and 15 patients had sub-clonal mutations in ECP pathway-related genes and the former had a shorter mPFS than the latter(1.3 months vs.5.3 months, HR=3.23, 95% CI: 1.25-8.37, P=0.011). Conclusions:Gene mutations in the epithelial cell proliferation pathway are a negative prognostic factor in elderly non-small cell lung cancer patients receiving immunotherapy, and mutations located in the clonal cluster have a stronger impact on the prognosis.
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AIM: Lipopolysaccharide (LPS) on the cell membrane of gram negative bacteria is closely related to the occurrence and development of severe acute pancreatitis (SAP). Local and systemic monocyte / macrophages play an important role in the inflammatory process of SAP. Artesunate (AS) was reported to protect rats with severe acute pancreatitis by reducing the release of proinflammatory cytokines. This study further explored the molecular mechanism of anti-inflammatory effect of AS. METHODS: The release of proinflammatory cytokines in the supernatant were studied by enzyme-linked immunosorbent assay. Then, the mRNA expressions of PI3K-III and its key molecules in signaling pathway were detected by real-time quantitative PCR. Finally, the phosphorylation levels of PI3KIII were detected by Western blot. RESULTS: AS could significantly inhibit the release of proinflammatory cytokines from mouse macrophage induced by LPS. Autophagy inhibitor 3-methyladenine (3-MA) could significantly inhibit the release of TNF-α from mouse macrophages induced by LPS; LPS significantly increased the mRNA expression of PI3KIII and its key molecules in mouse peritoneal macrophages (PMs). Finally, AS could significantly inhibit the increase of PI3K-III phosphorylation induced by LPS in PMs. CONCLUSION: The anti-inflammatory mechanism of AS is closely related to the inhibition of PI3K-III phosphorylation.