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Oocytes and embryos are highly sensitive to environmental stress in vivo and in vitro. During in vitro culture, many stressful conditions can affect embryo quality and viability, leading to adverse clinical outcomes such as abortion and congenital abnormalities. In this study, we found that valeric acid (VA) increased the mitochondrial membrane potential and ATP content, decreased the level of reactive oxygen species that the mitochondria generate, and thus improved mitochondrial function during early embryonic development in pigs. VA decreased expression of the autophagy-related factors LC3B and BECLIN1. Interestingly, VA inhibited expression of autophagy-associated phosphorylation-adenosine monophosphate-activated protein kinase (p-AMPK), phosphorylation-UNC-51-like autophagy-activated kinase 1 (p-ULK1, Ser555), and ATG13, which reduced apoptosis. Short-chain fatty acids (SCFAs) can signal through G-protein-coupled receptors on the cell membrane or enter the cell directly through transporters. We further show that the monocarboxylate transporter 1 (MCT1) was necessary for the effects of VA on embryo quality, which provides a new molecular perspective of the pathway by which SCFAs affect embryos. Importantly, VA significantly inhibited the AMPK-ULK1 autophagic signaling pathway through MCT1, decreased apoptosis, increased expression of embryonic pluripotency genes, and improved embryo quality.

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OBJECTIVE: The aim of this study was to investigate the combined effect of handgrip strength (HGS) and obesity phenotype on the risk of stroke in Chinese middle-aged and elderly people. METHODS: The data was used from the China Health and Retirement Longitudinal Study (CHARLS). Middle-aged and older adults who participated in surveys between 2011 and 2018 were included in the study. They were divided into 4 different types of obesity phenotypes based on obesity and metabolic status: metabolically healthy non-overweight/obesity (MHNO), metabolically healthy overweight/obesity (MHO), metabolically abnormal non-overweight/obesity (MANO), and metabolically abnormal overweight/obesity (MAO). The HGS level was divided into low and high groups according to the median values. Cox proportional risk regression model was used to analyze the joint effect of HGS and obesity phenotype on the risk of stroke among participants. RESULTS: A total of 7904 participants aged 58.89±9.08 years were included in this study. After adjusting for potential confounders, high HGS&MHO (HR=1.86, 95 % CI=1.12-3.09), high HGS&MANO (HR=2.01, 95 %CI=1.42-2.86), high HGS&MAO (HR=2.01, 95 % CI=1.37-2.93), low HGS&MHNO (HR=1.57, 95 % CI=1.00-2.46), low HGS&MHO (HR=2.09, 95 % CI=1.29-3.38), low HGS&MANO (HR=2.02, 95 % CI=1.35-3.03), and low HGS&MAO (HR=2.48, 95 % CI=1.72-3.58) group had significantly higher risks of stroke than the high HGS&MHNO group. CONCLUSION: The coexistence of metabolically unhealthy and low HGS can synergistically increase the risk of stroke in Chinese middle-aged and elderly people.

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BACKGROUND: Podoplanin (PDPN) expressed on tumour cells interacts with platelet C-type lectin-like receptor 2 (CLEC-2). This study aimed to investigate the role of the PDPN-platelet CLEC-2 interaction in melanoma pulmonary metastasis. METHODS: Murine melanoma B16-F0 cells, which have two populations that express podoplanin, were sorted by FACS with anti-podoplanin staining to obtain purified PDPN + and PDPN- B16-F0 cells. C57BL/6J mice transplanted with CLEC-2-deficient bone marrow cells were used for in vivo experiments. RESULTS: The in vivo data showed that the number of metastatic lung nodules in WT mice injected with PDPN + cells was significantly higher than that in WT mice injected with PDPN- cells and in WT or CLEC-2 KO mice injected with PDPN- cells. In addition, our results revealed that the platelet Syk-dependent signalling pathway contributed to platelet aggregation and melanoma metastasis. CONCLUSIONS: Our study indicates that the PDPN-CLEC-2 interaction promotes experimental pulmonary metastasis in a mouse melanoma model. Tumour cell-induced platelet aggregation mediated by the interaction between PDPN and CLEC-2 is a key factor in melanoma pulmonary metastasis.

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The practical applications of solar-driven water splitting pivot on significant advances that enable scalable production of robust photoactive films. Here, we propose a proof-of-concept for fabricating robust photoactive films by a particle-implanting technique (PiP) which embeds semiconductor photoabsorbers in the liquid metal. The strong semiconductor/metal interaction enables resulting films efficient collection of photogenerated charges and superior photoactivity. A photoanode of liquid-metal embraced BiVO4 can stably operate over 120 h and retain ~ 70% of activity when scaled from 1 to 64 cm2. Furthermore, a Z-scheme photocatalyst film of liquid-metal embraced BiVO4 and Rh-doped SrTiO3 particles can drive overall water splitting under visible light, delivering an activity 2.9 times higher than that of the control film with gold support and a 110 h stability. These results demonstrate the advantages of the PiP technique in constructing robust and efficient photoactive films for artificial photosynthesis.

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BACKGROUND:In recent years,a variety of lasers have been widely used in various diseases related to stomatology,including the prevention and treatment of dental caries. OBJECTIVE:To investigate the effect of neodymium-doped:yttrium aluminum perovskite(Nd:YAP)laser combined with two remineralizers on early enamel caries in vitro. METHODS:Early enamel caries models in vitro were artificially established by 60 enamel blocks and randomly divided into 6 groups(n=10).Group A did not undergo any treatment but underwent extracorporeal pH circulation.Group B underwent remineralization of dentin(the main component of casein phosphopeptide-amorphous calcium phosphate composite)and extracorporeal pH circulation.Group C underwent remineralization treatment of Sensodyne toothpaste(the main component of bioactive glass)and then underwent extracorporeal pH circulation.Group D received Nd:YAP laser irradiation and extracorporeal pH circulation.Group E was treated with Nd:YAP laser irradiation,with remineralization of dentin,and then with extracorporeal pH circulation.In group F,Nd:YAP laser irradiation was performed,and then Sensodyne toothpaste was used for remineralization,and the extracorporeal pH circulation was performed;the remineralization treatment was conducted twice a day,and the experimental period was 20 days.Group G was a normal control group,without caries or remineralization,but only underwent extracorporeal pH circulation.After the experiment,the microhardness,morphology and Ca/P ratio of the dental enamel surface were measured in each group. RESULTS AND CONCLUSION:(1)The surface microhardness value of dental enamel in groups B,C and D was higher than that in group A(P<0.000 1);the surface microhardness value of dental enamel in groups E and F was significantly higher than that in groups B,C and D(P<0.000 1),and the surface microhardness value of dental enamel in group F was significantly higher than that in group E(P<0.000 1).(2)Scanning electron microscopy showed that there were a lot of demineralized pores on the enamel surface of group A.There were mineral deposits on the enamel surface of group B,which were uneven and loose.In group C,there were a lot of mineral deposits on the enamel surface,and demineralized pores were found between the calcified masses.The enamel surface of group D was relatively flat;the demineralized pores were significantly smaller than that of group A,and the enamel column interstitium was damaged.In group E,the mineral deposits on the enamel surface were thicker and the demineralized pores were significantly reduced.The mineralized substances deposited on the enamel surface of group F were most dense and uniform and the demineralized pores were small.(3)The Ca/P ratio on the enamel surface of groups B and C was significantly higher than that of group A(P<0.000 1);the Ca/P ratio on the enamel surface of group E was significantly higher than that of groups B,C and D(P<0.000 1),and the Ca/P ratio on the enamel surface of group F was higher than that of group E(P<0.001).(4)These findings indicate that bioactive glass,casein phosphopeptide-amorphous calcium phosphate composite,and Nd:YAP laser after enamel demineralization can promote the remineralization of early enamel caries.Nd:YAP laser combined with bioactive glass or casein phosphopeptide-amorphous calcium phosphate composite can further strengthen the remineralization of dental enamel caries,and the combination of Nd:YAP laser and bioactive glass has the best effect.

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Evidence on the association between dietary nutrient-wide intake and cardiovascular disease (CVD) is inconclusive. Therefore, we systematically assessed the association between dietary intake of 29 nutrients and CVD risk using a nutrient-wide association study. Data were obtained from 7878 Chinese adults participating in the China Health and Nutrition Survey (CHNS) wave 2004-2015. We estimated the association of 29 nutrients with CVD risk. Significant findings were replicated in the National Health and Nutrition Examination Survey (NHANES). Four nutrients (selenium, vitamin A, carotenoids, and total protein) were significantly associated with CVD risk in the CHNS. The hazard ratio (HR) and 95% confidence interval (CI) for nutrient intake in the third tertile compared to the first tertile were 0.68 (0.51-0.90), 0.70 (0.54-0.91), 0.64 (0.50-0.83), and 0.54 (0.38-0.77), respectively. In the NHANES replication, selenium maintained a similar direction and strength of association, while the other nutrients were not replicated successfully. Our results provide support for a negative association between selenium intake and CVD risk, while the association of vitamin A, carotenoids and protein with CVD warrants further studies to confirm.

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This study explored the molecular mechanism of acteoside against hepatoma 22(H22) tumor in mice through c-Jun N-terminal kinase(JNK) signaling pathway. H22 cells were subcutaneously inoculated in 50 male BALB/c mice, and then the model mice were classified into model group, low-dose, medium-dose, and high-dose acteoside groups, and cisplatin group. The administration lasted 2 weeks for each group(5 consecutive days/week). The general conditions of mice in each group, such as mental status, diet intake, water intake, activity, and fur were observed. The body weight, tumor volume, tumor weight, and tumor-inhibiting rate were compared before and after administration. Morphological changes of liver cancer tissues were observed based on hematoxylin and eosin(HE) staining, and the expression of phosphorylated(p)-JNK, JNK, B-cell lymphoma-2(Bcl-2), Beclin-1, and light chain 3(LC3) in each tissue was detected by immunohistochemistry and Western blot. qRT-PCR was performed to detect the mRNA expression of JNK, Bcl-2, Beclin-1, and LC3. The general conditions of mice in model and low-dose acteoside groups were poor, while the general conditions of mice in the remaining three groups were improved. The body weight of mice in medium-dose acteoside group, high-dose acteoside group, and cisplatin group was smaller than that in model group(P<0.01). The tumor volume in model group was insignificantly different from that in low-dose acteoside group, and the volume in cisplatin group showed no significant difference from that in high-dose acteoside group. Tumor volume and weight in medium-dose and high-dose acteoside groups and cisplatin group were lower than those in the model group(P<0.001). The tumor-inhibiting rates were 10.72%, 40.32%, 53.79%, and 56.44% in the low-dose, medium-dose, and high-dose acteoside groups and cisplatin group, respectively. HE staining showed gradual decrease in the count of hepatoma cells and increasing sign of cell necrosis in the acteoside and cisplatin groups, and the necrosis was particularly obvious in the high-dose acteoside group and cisplatin group. Immunohistochemical results suggested that the expression of Beclin-1, LC3, p-JNK, and JNK was up-regulated in acteoside and cisplatin groups(P<0.05). The results of immunohistochemistry, Western blot, and qRT-PCR indicated that the expression of Bcl-2 was down-regulated in the medium-dose and high-dose acteoside groups and cisplatin group(P<0.01). Western blot showed that the expression of Beclin-1, LC3, and p-JNK was up-regulated in acteoside and cisplatin groups(P<0.01), and there was no difference in the expression of JNK among groups. qRT-PCR results showed that the levels of Beclin-1 and LC3 mRNA were up-regulated in the acteoside and cisplatin groups(P<0.05), and the level of JNK mRNA was up-regulated in medium-dose and high-dose acteoside groups and cisplatin group(P<0.001). Acteoside promotes apoptosis and autophagy of H22 cells in mice hepatoma cells by up-regulating the JNK signaling pathway, thus inhibiting tumor growth.

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Objective: To summarize the clinical characteristics of bronchial-pulmonary artery fistula and evaluate the effect of interventional closure of bronchial-pulmonary artery fistula. Methods: A retrospective case study was conducted. Fifteen children with hemoptysis who were diagnosed with bronchial-pulmonary artery fistula in Beijing Children's Hospital, Capital Medical University from January 2018 to March 2022 were selected. Their clinical symptoms and chest-enhanced CT findings were recorded. The children who failed to improve after anti-infection and hemostasis treatment were treated with transcatheter embolization through microparticles under digital subtraction angiography (DSA). The efficacy and post-operation recurrence were evaluated. Results: There were 15 children, including 9 males and 6 females, aged 9.8 (3.7, 12.1) years, weighing 35 (16, 55) kg. There was hemoptysis of varying degrees before surgery. Only 2 children had decreased hemoglobin. Chest enhanced CT showed that their bronchial arteries were thickened and tortuous, including 11 cases of single vessel disease and 4 cases of multivessel disease; 11 children had varying degrees of pneumonia and 4 children had atelectasis. Except for one case effectively treated with medical therapy, the remaining 14 cases were all treated with transcatheter interventional closure with embolic microparticles, among whom 12 had their fistula completely blocked with a single operation and the other 2 children underwent multiple operations because of too many fistulas. One child had extensive bronchial-pulmonary artery fistula which failed to be blocked completely even after multiple operations. Among the remaining 13 children, only 2 patients whose fistula was considered to be completely closed had recurrence presenting with hemoptysis at 3 months and 2 years after the operation, and no hemoptysis was found after the second closure. All children were discharged without chest pain, spinal cord paraplegia, or other serious complications. Fourteen children were followed up for 1.4 (0.9,2.9) years, among whom one still has intermittent mild hemoptysis due to incomplete closure and the rest had a satisfactory outcome. Conclusions: Hemoptysis is the first symptom of bronchial-pulmonary artery fistula. For children with failed medical treatment, transcatheter closure with an embolic pellet is effective, safe and feasible, with a low recurrence rate.

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OBJECTIVE@#To assess the value of fluorescence in situ hybridization (FISH) technique for the verification of the clonalities of non-clonal cytogenetic abnormalities (n-CCA) identified by conventional chromosome banding analysis (CBA) in patients with Myelodysplastic syndrome (MDS).@*METHODS@#Clinical data and results of karyotyping and FISH assays for 91 patients of MDS with n-CCA identified by CBA were retrospectively analyzed. In total 94 non-clonal +8, 5q-, -7/7q- or 20q- were detected by CBA, among which 43 (45.7%) were verified to be clonal abnormalities by FISH.@*RESULTS@#The detection rates for +8, 5q-, -7/7q- and 20q- by FISH were 47.6% (30/63), 25% (2/8), 41.7% (5/12), 40% (2/5) and 66.7% (4/6), respectively, with the positive cells accounting for 4% to 90% of all counted cells, with a median value of 7%. The 91 patients were divided into three groups including ≥ 20, 10 ~< 20 and < 10 based on the numbers of metaphase cells in CBA, and the detection rates by FISH for the three groups were 43.7% (31/71), 33.3% (3/9) and 63.6% (7/11), respectively, which showed no statistically difference (P > 0.05). Continuous CBA and FISH surveys were conducted for 26 patients who received supportive treatment, and the results revealed that 91.7% (11/12) of FISH-verified positive abnormalities had persisted, whereas 92.9% (13/14) of the n-CCA verified as negative by FISH was transient.@*CONCLUSION@#Nearly half of the CBA identified n-CCA have been verified as clonal aberrations by FISH, and the FISH detection rate showed no correlation with the number of metaphase cells. FISH test is strongly recommended for verifying the clonalities of n-CCA detected by CBA, and continuous cytogenetic survey of the patients with MDS is necessary.

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OBJECTIVE@#To explore the genetic basis for a fetus with limb abnormality and cardiac malformation.@*METHODS@#Clinical data of a fetus diagnosed at the Shandong Provincial Maternal and Child Health Care Hospital on April 30th, 2021 was collected. Whole exome sequencing (WES) was carried out, and candidate variant was verified by Sanger sequencing and bioinformatic analysis. X-inactivation analysis was carried out for the female members of its family.@*RESULTS@#The fetus was found to have meningoencephalocele, absence of bilateral radii, cleft lip, abnormal great arteries, and single umbilical artery at the gestational age of 11+ weeks. Sequencing revealed that the fetus has harbored a hemizygous c.1162del (p.Y388Tfs*7) variant of the FANCB gene, which was maternally inherited. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG) and ClinGen, the variant was classified as pathogenic (PVS1+PM2_Supporting+PP4). X-inactivation analysis has revealed complete skewed X-inactivation in the pregnant woman and her mother.@*CONCLUSION@#The hemizygous c.1162del (p.Y388Tfs*7) variant of the FANCB gene probably underlay the multiple malformations in this fetus.

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OBJECTIVE@#To investigate the clinical and prognostic characteristics of primary acute myeloid leukemia (AML) with 11q23/KMT2A rearrangements.@*METHODS@#Clinical data of 90 patients with primary AML and 11q23/KMT2A rearrangements were analyzed retrospectively.@*RESULTS@#By karyotyping analysis, 80 of the 90 patients had translocations involving 11q23/KMT2A, with t(9;11)(p22;q23), t(6;11)(q27;q23), t(10;11)(p12;q23) and t(11;19)(q23;p13) being the most common ones, while 10 cases were found to have non-translocation abnormalities. The overall complete remission (CR) rate was 75.6%, and patients with t(6;11) had lower CR rate compared with non-t(6;11) patients (47.1% vs. 82.2%, P = 0.005). After a median follow-up of 24.5 months, the patients receiving allo-hematopoietic stem cell transplantation (allo-HSCT) had significantly higher 3-year overall survival (OS) (80.3% vs. 16.6%, P < 0.001) and 3-year event-free survival (EFS) (73.5% vs. 16.3%, P < 0.001) compared with non-transplant patients. Patients with t(6;11) had the lowest 3-year OS (11.8% vs. 56.0%, P < 0.001) and 3-year EFS (5.9% vs. 53.8%, P < 0.001) compared with other type of abnormalities. No significant difference was noted in the survival between patients with t(9;11) and non-t(9;11) regardless whether they had received HSCT.@*CONCLUSION@#The clinical characteristics of primary AML with 11q23/KMT2A rearrangements are heterogeneous. Patients did not receive HSCT had poorer survival, particularly with the presence of t(6;11). Allo-HSCT could significantly improve the survival of such patients.

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At present, countries around the world are paying greater attention to the protection of medicinal plants and traditional medicinal knowledge resources, and are looking for various ways to protect medicinal plants. Many countries have established their own databases to save the medicinal plant information resources. This paper focuses on the introduction of medicinal plant databases in six countries including Malaysia, Philippines, and Singapore, and compares their basic information. It is difficult to achieve integration and sharing among these databases. It brings certain difficulties to the use of researchers in related fields. It is suggested that the construction of a multinational common medicinal plant database should be included in the "Belt and Road Initiative" to systematically organize massive information, enhance exchanges between countries on traditional medicinal plants, and achieve medicinal plant information sharing, and the establishment of a shared database will reduce optimization and maintenance to a certain extent or renewal work, laying the foundation for the protection, development and sustainable use of traditional medicinal plant resources.

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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 evaluate the relationship between the blood uric acid/high-density lipoprotein cholesterol ratio (UHR) and diabetes retinopathy (DR) in diabetic and pre-diabetic population.Methods:A cross-sectional study. The data from a health survey from 2010 to 2011 on chronic diseases and risk factors in Changping District in Beijing was used in this study. Total of 2 507 pre-diabetic and diabetic patients who met the inclusion and exclusion criteria were screened out in this study, included 1 212 men and 1 295 women. The patients were divided into DR group and non-DR (NDR) group according to whether DR was present or not. Independent sample t-test, chi-square test and multivariate logistic regression were used for case-control study to investigate whether there was independent correlation between UHR and DR. The receiver operating characteristic (ROC) curve was drawn to evaluate the diagnostic value of UHR for DR. Results:There were gender differences in the relationship between uric acid related indicators and DR, no significant correlation was found in women. In males, the age, duration of diabetes,fasting blood glucose (FPG), glycosylated hemoglobin (HbA 1c), systolic blood pressure (SBP), diastolic blood pressure (DBP), triglyceride (TG), serum uric acid, UHR levels and the proportion of diabetes and hypertension history in DR group were all significantly higher than those in NDR group (all P<0.05). Logistic regression analysis showed that SUR ( OR=1.054, 95%CI: 1.004-1.106, P=0.033) and UHR ( OR=1.391, 95%CI: 1.061-1.823, P=0.017) were the relative risk factors of DR. After adjusting for age, registered residence, education level, smoking, drinking, physical exercise, waist circumference, hypertension history, SBP, DBP, total cholesterol and other risk factors, UHR was still associated to DR [ OR ( 95%CI): 1.438 (1.084-1.908), P=0.012]. The area under the ROC curve of UHR was 0.610 ( 95%CI: 0.514-0.707, P=0.030). When the cut-off value of UHR for predicting DR was 0.24, the sensitivity and specificity were the highest, which was 78.8% and 58.7%, respectively. Conclusion:UHR is significantly correlated with the risk of DR in men with pre-diabetes and diabetes, but not in women. The risk of DR increases with the elevated level of UHR. UHR is helpful to diagnose DR and screen people with DR risk.

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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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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.

RESUMEN

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.