Effects from Extramedullary Design of Femoral Stem on Hip Contact Force: A Musculoskeletal Multi-Body Dynamic Study / 医用生物力学

Objective To investigate the effects of design parameters such as neck-shaft angle, femoral offset and anteversion angle of total hip arthroplasty ( THA) prosthesis on contact forces of the hip. Methods A musculoskeletal multi-body dynamic model of THA was established based on AnyBody software. The effects of single or multiple factors on hip contact force were studied when the neck-shaft angle, eccentricity and anteversion angle varied within ±10°, ±20 mm and ±10°, respectively. Results The maximum hip contact force increased by 26. 08% when femoral offset was reduced by 20 mm. The maximum hip contact force increased by 5. 99% when the neck-shaft angle increased by 10°. When the anteversion angle increased by 10°, the hip contact force decreased at 0% -24% of gait cycle, with the peak decreasing by 19. 16% . However, the hip contact force was significantly increased at 38% -70% of gait cycle, with the peak increasing by 67. 78% . Conclusions In extramedullary design of the femoral stem, based on reconstruction of the patient’s anatomical parameters, the offset of the femoral stem can be appropriately increased, and the neck-shaft angle and anteversion angle can bereduced to avoid increasing forces on the hip.

Study on material basis of cathepsin K targeted inhibitor in Erzhi Wan / 药学实践杂志

Objective To investigate the active ingredients and components that inhibiting cathepsin K activity in Erzhi Wan, a classic kidney-tonifying formula. Methods Then-butanol, dichloromethane, ethyl acetate and petroleum ether parts and 30 active components in Erzhi Wan were screened by established high throughput fluorescence methods of inhibit the binding activity of CTSK with Z-FR-MCA substrate, the formation of CTSK and chondroitin sulfate A (CSA) complex activity, and the activity of substrate type I collagen degradation by CTSK. Molecular docking and insoluble collagen substrate binding assays were applied to verify the potential CTSK inhibitors. Results The n-butanol and petroleum ether parts of Erzhi Wan inhibited the formation of CTSK and CSA* complex by more than 90%, the petroleum ether part inhibited the binding of CTSK to substrate Z-FR-MCA by more than 90%, the collagen degradation inhibition rate of CTSK in n-butanol part was more than 95% and that in petroleum ether part was 58.6%. Among the 30 active components, 11 showed that the inhibition rate of CTSK and CSA* complex formation was more than 50%, and 5 components with the inhibition rate of Z-FR-MCA binding activity more than 50%. Finally, there were four components including eclalbasaponin Ⅸ, (-)-epicatechin gallate, nuezhenoside and wedelolactone. The inhibition rate of collagen degradation was more than 50%. Eclipta saponin IX inhibited the binding rate between collagen fibers and CTSK, up to 60%, but all of them failed to dock with CTSK active site. Conclusion There are active components that inhibiting cathepsin K in Erzhi Wan, which mainly exists in the n-butanol ingredients, but the active components is not an active-site inhibitor. It might inhibit the binding of CTSK with oligosaccharides by binding to other sites of CTSK, and then reduce the collagen degradation activity of CTSK.

Overexpression of chicken Klf2 promotes klf7 transcription and inhibits adipocyte differentiation / 生物工程学报

Overexpression of Krüppel like factor 2 (Klf2) or Klf7 inhibits adipocyte formation. However, it remains unclear whether Klf2 regulates klf7 expression in adipose tissue. In this study, oil red O staining and Western blotting were employed to study the effect of Klf2 overexpression on the differentiation of chicken preadipocytes. The results showed that Klf2 overexpression inhibited the differentiation of chicken preadipocytes induced by oleate and the expression of pparγ, while promoted klf7 expression in chicken preadipocytes. Spearman correlation analysis was used to study the correlation between the expression data of klf2 and klf7 in the adipose tissue of both human and chicken. The results showed that there was a significantly positive correlation between the expression of klf2 and klf7 in adipose tissues (r > 0.1). Luciferase reporter assay showed that overexpression of Klf2 significantly promoted the activity of chicken klf7 promoter (-241/-91, -521/-91, -1 845/-91, -2 286/-91, -1 215/-91; P < 0.05). In addition, the activity of klf7 promoter (-241/-91) reporter in chicken preadipocytes was significantly positively correlated with the amount of klf2 overexpression plasmid transfected (Tau=0.917 66, P=1.074×10-7). Moreover, Klf2 overexpression significantly promoted the mRNA expression of klf7 in chicken preadipocytes (P < 0.05). In conclusion, upregulation of klf7 expression might be one of the pathways that Klf2 inhibits chicken adipocyte differentiation, and the sequence from -241 bp to -91 bp upstream chicken klf7 translation start site might mediate the regulation of Klf2 on klf7 transcription.

Effectiveness of arthroscopic "hybrid" suture for delaminated rotator cuff tear: A prospective randomized controlled study / 中国修复重建外科杂志

OBJECTIVE@#To investigate the effectiveness of "hybrid" suture with en masse combined with double-layer repair under arthroscopy in repair of delaminated rotator cuff tear by comparison with en masse suture.@*METHODS@#Fifty-six patients with delaminated rotator cuff tears met selection criteria between June 2020 and January 2022 were included in the study. Patients were divided into two groups ( n=28) using a random number method. The patients in trial group underwent arthroscopic "hybrid" suture with the combination en masse and double-layer suture. The patients in control group underwent en masse suture under arthroscopy. There was no significant difference between the two groups ( P>0.05) in terms of gender, age, rotator cuff tear side, tear size, cause of injury, disease duration, and preoperative American Association of Shoulder and Elbow Surgeons (ASES) score, the University of California at Los Angeles (UCLA) shoulder scoring, visual analogue scale (VAS) score, and shoulder range of motion (forward flexion and lateral external rotation). The operation time, the difference of ASES score, UCLA score, VAS score, and shoulder range of motion (forward flexion and lateral external rotation) between pre- and post-operation were recorded and compared between the two groups ( P>0.05). The rotator cuff healing was examined by MRI and evaluated based on the classification criteria of rotator cuff healing proposed by Sugaya et al.@*RESULTS@#Three cases (1 case in the trial group and 2 cases in the control group) were excluded from the study due to loss of follow-up. Twenty-seven cases in the trial group and 26 cases in the control group were included in the final study analysis. All operations of the two groups were completed successfully. There was no significant difference in the operation time between groups ( P>0.05). The follow-up time was 10-12 months (mean, 10.9 months) in the trial group and 10-13 months (mean, 11.4 months) in the control group. All incisions healed by first intention. No surgery-related complications occurred. The UCLA score, ASES score, VAS score, and shoulder range of motion (forward flexion and lateral external rotation) of both groups at 9 months after operation were significantly superior to those before operation ( P<0.05). The difference of UCLA score, ASES score, and VAS score between before and after operation in the trial group were significantly better than those in the control group ( P<0.05). There was no significant differences between the two groups in the difference of shoulder range of motion (forward flexion and lateral lateral rotation) ( P>0.05). At 9 months after operation, according to the classification criteria of rotator cuff healing proposed by Sugaya et al, MRI showed that the rotator cuff healing of the trial group was significantly better than that of the control group ( P<0.05).@*CONCLUSION@#Compared with en masse suture, arthroscopic "hybrid" suture for the repair of delaminated rotator cuff tear has advantages in relieving pain and improving shoulder joint function, and the rotator cuff healing is better.

Clinical Effect of Chaishao Longmu Decoction on Insomnia in Patients with Liver Depression and Spleen Deficiency Based on Infrared Thermal Images / 中国实验方剂学杂志

ObjectiveTo observe the therapeutic effect of Chaishao Longmu decoction on insomnia in the patients with the syndrome of live depression and spleen deficiency and explore the correlation between infrared thermal imaging and insomnia with liver depression and spleen deficiency. MethodA total of 72 insomnia patients treated in the outpatient department of Shenzhen Hospital of Guangzhou University of Chinese Medicine （Futian） from May to December in 2022 were selected and randomized into a treatment group and a control group， with 36 patients in each group. The patients in the treatment group were treated with Chaishao Longmu decoction and those in the control group with eszopiclone for 4 weeks. The clinical efficacy， Pittsburgh sleep quality index （PSQI） score， TCM syndrome score and infrared thermal imaging characteristics ［temperature and temperature changes of frontal sleep line， frontal region， anterior trunk， Zhongwan （CV12）， conception vessel （CV）， left hypochondrium， right hypochondrium， dorsal trunk， and governor vessel （GV）］ of two groups were determined before and after treatment. ResultAfter treatment， the clinical response rate in treatment group was 91.67% （33/36）， which was higher than that （66.67%， 24/36） in the control group （Z=-2.617， P<0.01）. The treatment in both groups decreased the PSQI score and TCM syndrome score （P<0.01）， and the decreases were more significant in treatment group than in the control group （P<0.01）. After treatment， the total response rate of sleep line improvement in the treatment group was 86.11% （31/36）， which was higher than that （66.67%， 24/36） in the control group （Z=-2.591， P<0.05）. The frontal temperature of the two groups decreased （P<0.01） after treatment. Compared with those before treatment， the temperatures of anterior trunk， CV12， CV， left hypochondrium， right hypochondrium， dorsal trunk， and GV rose after treatment （P<0.01）. After treatment， the treatment group had lower frontal temperature and higher temperatures of anterior trunk， CV12， CV， left hypochondrium， right hypochondrium， dorsal trunk， and GV than the control group （P<0.01）. The treatment in the treatment group reduced the ∆T values of GV （P<0.01） and increased that of the CV12， CV， left hypochondrium， and right hypochondrium （P<0.05，P<0.01）. The treatment in the control group increased the ∆T value of CV12 （P<0.01）. After treatment， the treatment group had lower ∆T values of GV （P<0.01） and higher ∆T value of CV12， CV， left hypochondrium， and right hypochondrium （P<0.05， P<0.01） than the control group. Compared with that before treatment， the temperature difference between GV and CV in the two groups increased after treatment （P<0.01）. According to the infrared thermal image characteristics， the normal temperature difference between GV and CV was within the range of 0.5-1. The median value after treatment in the treatment group was 0.69 （0.52， 0.88）， which was within the normal range， indicating that the treatment group outperformed the control group. ConclusionChaishao Longmu decoction can alleviate short-term insomnia by soothing liver， invigorating spleen， harmonizing the middle energizer， and regulating GV and CV. With definite clinical effect， this decoction deserves promotion. Furthermore， the frontal temperature， sleep line， CV12， CV ∆T， and temperature difference between GV and CV revealed by the infrared thermal images could be used for the diagnosis and of insomnia with liver depression and spleen deficiency.

Radiofrequency ablation of ventricular arrhythmias from the pulmonary sinus cusp in pediatric patients and the follow-up / 中华实用儿科临床杂志

Objective:To evaluate the strategy and safety of the radiofrequency ablation (RFA) on ventricular arrhythmias (VAs) originating from the pulmonary sinus cusp (PSC) in pediatric patients.Methods:Retrospective study.Fifteen patients with VAs originating from the PSC who were intervened by RFA in the Department of Pediatric Cardiology, Guangdong Provincial People′s Hospital between March 2014 to July 2020 were enrolled.All the patients met the indication criteria for RFA in pediatric patients.The electrocardiogram, ablation method of ablation were analyzed.Different curved catheters were selected for RFA according to the age and weight of the patients.The catheter was then inserted in a " U" or inverted " P" shape to the PSC.The long-term effect of ablation were reviewed.Results:The mean age and body weight of 15 patients with VAs originating from the PSC were (11.6±2.6) (6-15) years and (39.9±12.2) (19-65) kg, respectively.The electrocardiogram recorded during VAs originating from the PSC showed left bundle branch block and inferior axis with monomorphic R pattern, as well as a QS-wave in aVR and aVL.The electrocardiogram characteristics varied in patients with VAs originating from the PSC.The ideal excitation point was not found in the right ventricular outflow tract or the ablation was unsuccessful in all patients, and the earliest target was mapped and RFA was successful.Among the 15 patients, the successful ablation site was in the lower regions of the PSC, involving the right cusp in 11 patients(73.3%), the anterior cusp in 3 patients(20.0%), and the left cusp in 1 patient(6.7%). The earliest potential recorded at the PSC ablation site preceded the QRS complex onset by (27.3±6.0) ms.During the follow-up period for (2.7±2.0) years, no recurrence of VAs or complications were recorded.Conclusions:Under the premise of gentle catheterization procedure and appropriate radiofrequency energy, ablation was effective, safe and with low recurrence rate to eradicate VAs originating from the PSC in children.

Clinical strategies for COVID-19 in surgeons / 中华消化外科杂志

Surgeons need high sensitivity to the epidemic of Corona Virus Disease 2019 (COVID-19) and take preventive measures in hospital as early as possible. They need to totally play their roles in anti-epidemic to treat patients with acute and critical diseases as well as overall arrangement under the condition of adapting to epidemic situation.

Application and follow-up of implantable cardioverter defibrillator for children and adolescents / 中华实用儿科临床杂志

Objective:To explore the application of implantable cardioverter defibrillator(ICD) in the prevention of sudden cardiac death for children and adolescents, the treatment of adverse events and follow-up studies.Methods:A total of 19 pediatric patients (12 males, 7 females) were admitted in the Department of Pediatric Cardio-logy, Guangdong Provincial People′s Hospital from January 2008 to March 2019, and their previous inpatient and outpatient data including medical history, ICD implantation method, postoperative program control, the use of antiarrhythmic drugs, proper/improper discharge of ICD and management of ventricular electrical storm were collected retrospectively and follow-up study was carried out.Results:A total of 19 pediatric patients were included, all of whom had once syncope or more before ICD implantation.The age at the time of ICD implantation was (12.30±2.08) years, and the weight was (40.00±13.93) kg.Eleven patients were implanted in the subpectoralis major space, 6 cases in the subfascial space, and 2 cases in the left abdomen and left armpit, respectively.All patients received oral antiarrhythmic drugs after surgery.The follow-up period was 3.2 years(0.3-11.1 years). All the children survived without complications such as electrode fracture, dislocation, venous thrombosis, cardiac perforation, capsular hematoma, rupture, and infection.Twelve cases (63.2%) had discharge events, including 10 cases (83.3%) of appropriate recognition/treatment events, and 2 cases (16.7%) of inappropriate recognition/treatment events, while 5 cases were ventricular electrical storms.Totally, 7 ICD cases (36.8%) were equipped with remote monitoring system, and 4 cases (57.1%) were detected whit adverse reactions, which were timely handled online and offline, thus avoiding adverse consequences.Conclusions:Currently, ICD is the most effective treatment for the prevention of sudden cardiac death in children and adolescents.According to age, weight and developmental conditions, different implantation methods should be selected.Appropriate/inappropriate discharge events after implantation should be identified in time, drug treatment should be optimized, and radiofrequency ablation should be performed if necessary.

Transthoracic echocardiographic diagnosis for partial anomalous pulmonary venous connection in children / 中华实用儿科临床杂志

Objective:To investigate the cause of missed diagnosis by transthoracic echocardiography (TTE) based on the characteristics of partial anomalous pulmonary venous connection (PAPVC), and to improve the ultrasonic diagnostic accuracy of PAPVC in children.Methods:The TTE results of 252 children under 12 years old who were confirmed with PAPVC at Guangdong Provincial People′s Hospital from January 2011 to June 2019 were reviewed retrospectively.The types of PAPVC and the associated atrial septal defects (ASD) as well as the confirmed and missed cases by TTE were analyzed.Results:PAPVC was right-sided in 238 patients (94.4%), left-sided in 8 patients (3.2%), and bilateral in 6 patients (2.4%). There were 177 cases (70.2%) whose pulmonary veins were abnorma-lly connected to the right atrium(RA), 37 cases (14.7%) to the junction of the RA and the superior vena cava (SVC), 27 cases (10.7%) to the distal SVC, and 6 cases (2.4%) to the inferior vena cava.Besides, pulmonary veins of 5 cases (2.0%) flew back to the RA through the coronary sinus.One hundred and ninety PAPVC cases were combined with sinus venous defects (SVD) and 53 cases combined with secondum ASD.Two hundred and twenty-one cases were accurately diagnosed while 31 cases were underestimated by TTE.The omission diagnostic rates of right superior pulmonary veins connecting to the distal SVC, 1 or 2 right pulmonary veins connecting to the RA or the junction of the RA and SVC, and left-sided PAPVC were 8 out of 18 (44.4%), 22 out of 215 (10.2%) and 1 out of 8 (12.5%), respectively.Among 54 cases with right superior pulmonary veins anomalously connected to the RA or the junction of the RA and SVC, 88.9% of them (48/54 cases) were combined with superior SVD.Among 161 cases with the right inferior pulmonary veins or 2 right pulmonary veins connected to the RA, 78.2% of them (126/161 cases) were combined with inferior SVD.There were statistically significant differences in ASD types between the 2 right-sided PAPVC groups.Conclusions:SVD is often associated with 1 or 2 right pulmonary veins connected to the RA or the junction of the RA and SVC.The cases with right superior pulmonary veins connected to the distal SVC are prone to be underestimated by TTE.Whether there is abnormal blood flowing into SVC should be noticed during superior sternal fossa examinations.Each pulmonary vein should be examined in detail in the TTE test and accurate diagnosis of PAPVC can be made in most cases.

Progress in interventional treatment of children with congenital heart disease / 中华实用儿科临床杂志

The interventional treatment of congenital heart diseases has been developed for more than 50 years since Rashkind et al.performed balloon atrial septostomy in 1966.From the initial catheter examination and palliative operation to the current radical occlusion,stent implantation,valve replacement,etc,interventional catheterization has become one of main means of treating congenital heart disease.The interventional therapy of congenital heart disease in China has made great progress,but there is still a certain gap with developed countries.This article summarizes the progress of interventional treatments in septal defect diseases,aortic artery to pulmonary artery shunt diseases,valvular diseases,and hybrid therapy,in order to provide reference for the interventional treatment of congenital heart disease in China.

Percutaneous vertebroplasty through unilateral and bilateral pedicle approaches and unilateral pedicle extrapedicle approach for the treatment of thoracolumbar vertebral compression fractures: Bone cement perfusion volume and cement leakage rate / 中国组织工程研究

BACKGROUND: Percutaneous vertebroplasty is clinically performed mainly through unilateral and bilateral pedicle approaches and unilateral pedicle extrapedicle approach. However, there are still disputes on the clinical effect and complications of the three approaches. OBJECTIVE: To compare the clinical effect of percutaneous vertebroplasty in the treatment of thoracolumbar vertebral compression fractures among three approaches. METHODS: Seventy-eight patients with thoracolumbar vertebral compression fractures who received treatment in Hainan Provincial Hospital of Traditional Chinese Medicine, China between January 2016 and January 2018 were included in this study. These patients consisted of 30 males and 48 females and were aged 40-71 years. Twenty patients received bone cement perfusion through the unilateral pedicle approach (unilateral pedicle group), 35 patients received bone cement perfusion through the bilateral pedicle approach (bilateral pedicle group), and 23 patients received bone cement perfusion through the unilateral pedicle extrapedicle approach (unilateral pedicle extrapedicle group). Bone cement perfusion volume and cement leakage rate were recorded. All patients were followed up for 1 year. The Visual Analogue Scale score, Oswestry Disability Index, vertebral height reconstruction rate, Cobb angle improvement, patient satisfaction and complications were compared among the three groups. This study was approved by the Hospital Ethics Committee, Hainan Provincial Hospital of Traditional Chinese Medicine, China (approval No. HKL20151203). RESULTS AND CONCLUSION: (1) Bone cement perfusion volume in the bilateral pedicle group was significantly lower than that in the unilateral pedicle and unilateral pedicle extrapedicle groups (P 0.05). At 1 year after surgery, Visual Analogue Scale score and Oswestry Disability Index were significantly lower than those before surgery in each group (P < 0.05). (3) Cement leakage rate in the bilateral pedicle group was significantly lower than that in the unilateral pedicle and unilateral pedicle extrapedicle groups (P < 0.05). Cement leakage rate in the unilateral pedicle extrapedicle group was significantly lower than that in the unilateral pedicle group (P < 0.05). (4) The fracture rate of adjacent vertebral bodies in the bilateral pedicle group was significantly lower than that in the unilateral pedicle group (P < 0.05). (5) These results suggest that three approaches of vertebroplasty and bone cement injection for treatment of thoracolumbar vertebral compression fractures can achieve better clinical efficacy. Bilateral pedicle approach can significantly reduce the incidence of cement leakage and adjacent vertebral fractures compared with the unilateral pedicle approach and unilateral pedicle extrapedicle approach.

Progress in interventional treatment of children with congenital heart disease / 中华实用儿科临床杂志

The interventional treatment of congenital heart diseases has been developed for more than 50 years since Rashkind et al.performed balloon atrial septostomy in 1966.From the initial catheter examination and palliative operation to the current radical occlusion, stent implantation, valve replacement, etc, interventional catheterization has become one of main means of treating congenital heart disease.The interventional therapy of congenital heart disease in China has made great progress, but there is still a certain gap with developed countries.This article summarizes the progress of interventional treatments in septal defect diseases, aortic artery to pulmonary artery shunt diseases, valvular diseases, and hybrid therapy, in order to provide reference for the interventional treatment of congenital heart disease in China.

Advances in biomechanical assessment of risk characteristics of thoracic aortic aneurysm / 中华胸心血管外科杂志

The clinical assessment of the risk of rupture and dissection of thoracic aortic aneurysm(TAA) is mainly dependent on the measurement of the maximum diameter and growth rate of the aneurysm itself. The use of aortic size alone may ignore the role of vascular heterogeneity in assessing the risk of catastrophic complications. Biomechanics could help predict the risk of TAA in a more sophisticated way. In this paper, we reviewed the latest advances in biomechanical assessment of risk characteristics of TAA.

Outcome and follow-up analysis of transcatheter or surgical therapy for neonates with pulmonary atresia with intact ventricular septum and critical pulmonary stenosis / 中华实用儿科临床杂志

Objective@#To evaluate the safety and effectiveness of transcatheter or surgical procedure for neonates diagnosed with pulmonary atresia with intact ventricular septum (PA/IVS) or critical pulmonary stenosis (CPS) by retrospectively analyzina the clinical data, thus to discuss the clinical strategy decision.@*Methods@#From November 2006 to August 2018, 105 neonates (PA/IVS 51 cases, CPS 54 cases) went through transcatheter or surgical procedure in Guangdong Provincial Cardiovascular Institute (transcatheter procedure 43 cases, surgery 62 cases). All cases manifested mild to moderate right ventricular dysplasia.Transcatheter procedure included pulmonary radio frequency perforation and percutaneous balloon pulmonary valvuloplasty (PBPV). Surgical procedure included reconstruction of right ventricular outflow tract (transannular patch), pulmonary valvotomy and closed transventricular pulmonary valvuloplasty, while Aortic-Pulmonary shunt was performed in certain cases.Average follow-up period was (40.1±36.9) months.Hospitalization and follow-up data were analyzed to evaluate the safety and efficacy of transcatheter and surgical procedure.@*Results@#Operative time, hospital stay, complication rate were lower in transcatheter procedure group than those of surgery group[(107.8±61.6) min vs.(183.3±36.8) min, (18.6±7.9) d vs.(31.1±13.4) d, 30.2% vs.80.6%], and the differences were significant(all P=0.000). In transcatheter procedure group, 2 cases (4.7%) died and biventricular circulation is obtained in all survival cases.In surgery group, 5 cases (8.1%) died.Of the survival cases from surgery group, 39 cases (69.6%) obtained biventricular circulation, 3 cases (5.4%) obtained one and a half ventricular circulation while other 14 cases (25.0%) were candidates for biventricular repair.@*Conclusions@#Biventricular circulation can be achieved in most PA/IVS or CPS neonates with mild-moderate right ventricular dysplasia.PBPV turns out to be a safe and effective therapy for neonates with CPS while perforation of right ventricular outflow tract remains a nonnegligible complication in transcatheter therapy for PA/IVS.

Expression of heterogeneous nuclear ribonucleo-protein A2B1 in mouse cerebellar development and human medulloblastoma / 中华病理学杂志

Objective@#To investigate the expression and potential role of heterogeneous nuclear ribonucleo-protein A2B1 (HNRNPA2B1) in mouse cerebellar development and the significance of HNRNPA2B1 in human medulloblastoma.@*Methods@#The data of HNRNPA2B1 RNA expression in mouse and human cerebella were obtained from databases. Western blot and immunohistochemical staining were performed to detect the protein level of HNRNPA2B1 in mouse cerebella at different ages. The expression level of HNRNPA2B1 in control human cerebellum and medulloblastoma was detected by immunohistochemical staining. m6A-IP-qPCR method was applied to confirm whether HNRNPA2B1 RNA in Daoy cells was modified with m6A.Western blot was used to detect the effect of MG132 treatment on the HNRNPA2B1 protein level in Daoy cells.@*Results@#The level of HNRNPA2B1 protein in postnatal mouse cerebella was higher than that in adult mouse cerebella, with weak HNRNPA2B1 staining in external granular cells while strong staining in mature Purkinje cells and molecular layer. Compared with control normal human cerebella, the RNA expression level of HNRNPA2B1 increased in medulloblastoma, while immunohistochemical staining showed that the mean intensity of HNRNPA2B1 decreased in medulloblastoma. HNRNPA2B1 RNA in medulloblastoma and Daoy cells was modified by m6A. The HNRNPA2B1 protein level in Daoy cells increased upon MG132 treatment.@*Conclusions@#HNRNPA2B1 is dynamically expressed during mouse cerebellar development. Compared with normal human cerebella, HNRNPA2B1 is significantly up-regulated at transcriptional level but obviously down-regulated at translational level in medulloblastoma. These results indicate that HNRNPA2B1 may be involved in cerebellar development process and medulloblastoma tumorigenesis. The m6A methylation in HNRNPA2B1 transcript and protein ubiquitin-proteasome pathway may account for the down-regulation of HNRNPA2B1 at protein level.

Analysis of 30 cases of inherited cardiac arrhythmia syndrome in children / 中华儿科杂志

Objective@#To analyze and summarize the diagnosis and treatment experience of common inherited cardiac arrhythmia syndrome in pediatric patients, and explore the most appropriate therapy.@*Methods@#A retrospective review identified 30 pediatric cases (19 males, 11 females) diagnosed with long QT syndrome (LQTS), Brugada syndrome (BrS), catecholaminergic polymorphic ventricular tachycardia (CPVT), hypertrophic cardiomyopathy (HCM), arrhythmogenc right ventricular cardiomyopathy (ARVC) from January 2008 to December 2018 in the Pediatric Cardiology Department, Guangdong Provincial People′s Hospital. Data obtained included the diagnosis, treatment and follow-up outcome.@*Results@#The most common inherited cardiac arrhythmia syndromes were LQTS (n=14) including 1 case with epilepsy, CPVT (n=5), HCM (n=7), ARVC (n=1), and BrS (n=3). Twenty-seven cases were admitted to hospital due to syncope, whereas the remaining 3 cases of BrS had not presented with syncope before admission. The average onset age of inherited arrhythmia was (10.0±3.3) years. Genetic testing was performed on 20 patients. The median follow-up time was 40 months. Among 15 patients who underwent implantable cardioverter defibrillator (ICD) and survived, 2 patients had frequent ICD discharge. One patient underwent radiofrequency ablation, and the other one received left cardiac sympathetic denervation and an increased ICD defibrillation threshold, and the number of ICD discharge was significantly reduced. Among 10 patients who received drug therapy, 4 patients including two patients who discontinued treatment without advices died. Two patients whose parents refused treatment died, 1 case diagnosed with unexplained sudden cerebral death, and the remaining 2 cases without indication for drug therapy survived without any treatment.@*Conclusions@#Mortality rate is high in pediatric patients with inherited cardiac arrhythmia and syncope. The therapeutic effect of drugs are not satisfactory, ICD implantation is the most effective treatment to prevent sudden cardiac death currently, but the postoperative frequent discharge should be brought to the forefront and handled in time.

Outcome and follow _up analysis of transcatheter or surgical therapy for neonates with pulmonary atresia with intact ventricular septum and critical pulmonary stenosis / 中华实用儿科临床杂志

Objective To evaluate the safety and effectiveness of transcatheter or surgical procedure for neo_nates diagnosed with pulmonary atresia with intact ventricular septum(PA/IVS)or critical pulmonary stenosis(CPS) by retrospectively analyzina the clinical data,thus to discuss the clinical strategy decision. Methods From November 2006 to August 2018,105 neonates(PA/IVS 51 cases,CPS 54 cases)went through transcatheter or surgical procedure in xuangdong Provincial Cardiovascular Institute(transcatheter procedure 43 cases,surgery 62 cases). All cases mani_fested mild to moderate right ventricular dysplasia. Transcatheter procedure included pulmonary radio frequency perfora_tion and percutaneous balloon pulmonary valvuloplasty( PBPV). Surgical procedure included reconstruction of right ventricular outflow tract(transannular patch),pulmonary valvotomy and closed transventricular pulmonary valvuloplas_ty,while Aortic _Pulmonary shunt was performed in certain cases. Average follow _up period was(40. 1 ± 36. 9) months. Hospitalization and follow_up data were analyzed to evaluate the safety and efficacy of transcatheter and surgi_cal procedure. Results Operative time,hospital stay,complication rate were lower in transcatheter procedure group than those of surgery group[(107. 8 ± 61. 6)min υs.(183. 3 ± 36. 8)min,(18. 6 ± 7. 9)d υs.(31. 1 ± 13. 4)d, 30. 2% υs. 80. 6%],and the differences were significant(all P=0. 000). In transcatheter procedure group,2 cases (4. 7%)died and biventricular circulation is obtained in all survival cases. In surgery group,5 cases(8. 1%)died. Of the survival cases from surgery group,39 cases(69. 6%)obtained biventricular circulation,3 cases(5. 4%)obtained one and a half ventricular circulation while other 14 cases( 25. 0%) were candidates for biventricular repair. Conclusions Biventricular circulation can be achieved in most PA/IVS or CPS neonates with mild_moderate right ventricular dysplasia. PBPV turns out to be a safe and effective therapy for neonates with CPS while perforation of right ventricular outflow tract remains a nonnegligible complication in transcatheter therapy for PA/IVS.

Congenital heart disease and neurodevelopment in children from the perspective of genetics / 中华实用儿科临床杂志

Children with congenital heart disease (CHD) are at risk of neurodevelopmental disorder (NDD) which can be manifested as a diversity of clinical type.Up to 20％-30％ of CHD children have genetic abnormalities.As an independent risk factor for NDD,genetic abnormalities may predict a poorer neurodevelopmental outcome in these children.Because neurodevelopmental outcomes are closely related to the presence of underlying syndromes or genetic abnormalities,early diagnosis is the key to establish the prognosis of it,which is conducive to provide guidance for the children and their families in early time.In order to draw the attention of pediatric clinicians and CHD families and provide more comprehensive support for those children,this brief review is written for illustrate the relationship between CHD children and their neurodevelopmental outcome from the perspective of genetics.

Different interventions in treatment of children with congenital aortic stenosis / 中华实用儿科临床杂志

Objective To evaluate the efficacy and safety of percutaneous balloon aortic valvuloplasty (PBAV) in retrograde,antegrade way or right ventricular pacing in children.Methods This was retrospective clinical study included 32 children who were treated with PBAV for congenital aortic stenosis from January 2008 to June 2017 in Guangdong Cardiovascular Institute.The general clinical data including residual stenosis and aortic stenosis again,and degree of artery injury,aortic regurgitation were particularly assessed.Results A total of 32 patients consisting of 27 boys and 5 girls underwent the procedure,with age of (55.8 ± 52.0) months (ranging from 20 days to 15 years) and body weight (18.2 ± 14.0) kg (ranging from 3.5 kg to 59.0 kg).Two infants accepted left cardiac catheterization through femoral vein and one with interventional indication then accepted PBAV through the patent foramen ovale.The others underwent the retrograde way,including 24 cases with rapid right ventricular pacing.The catheter-measured peak systolic the aortic valve gradient decreased from (81.6 ± 28.0) mmHg(1 mmHg =0.133 kPa) to (41.4 ± 19.0) mmHg immediately after percutaneous interventional treatment,and the difference was statistically significant (t =9.543,P =0.000).The peak systolic valve gradient mea-sured pre-PBAV and on the second day after PBAV measured by Doppler echocardiography decreased from (82.7 ±23.0) mmHg to (44.6 ± 18.0) mmHg,and the difference was statistically significant (t =11.732,P =0.000).The diameters of the aortic valve were (13.8 ± 3.0) mm(ranging from 6.5 mm to 21.0 mm) and the balloons were (14.1 ±4.0) mm(ranging from 6 mm to 23 mm).The follow-up period was 1 to 72 months.The peak systolic valve gradient measured by Doppler echocardiography increased from (44.6 ±18.0) mmHg to (58.6 ± 30.0) mmHg,and the difference was statistically significant (t =-2.549,P =0.016).During the procedure,5 children (14.7％) accepted surgery for restenosis or regurgitation.Seven had regurgitation,one had femoral artery embolism and one died on the second day after the procedure.Conclusions With the diversification of procedures and the continuous improvement of interventional devices,PBAV can play a very good role in alleviating congenital aortic stenosis in children,and the safety has been continuously improved.

Prenatal interventional therapy in two cases with critical pulmonary stenosis or pulmonary atresia with intact ventricular septum / 中华儿科杂志

Objectives@#Two cases who underwent fetal pulmonary valvuloplasty (FPV) for pulmonary atresia with intact ventricular septum (PA-IVS) or critical pulmonary stenosis with intact ventricular septum (CPS-IVS) successfully were reported. The aim of the report was to explore the criteria for case selection, the technical essentials of FPV, and the postpartum outcome of the fetus.@*Methods@#One case with PA-IVS and the other with CPS-IVS were enrolled in September 2016 and February 2017 in Guangdong General Hospital, and both cases were diagnosed with severe right ventricular dysplasia and tricuspid regurgitation by fetal echocardiogram. Parameters of right ventricle development and hemodynamics from echocardiography included tricuspid/mitral annulus (TV/MV), right ventricle/left ventricle long-axis (RV/LV), pulmonary/aortic annulus (PV/AV), tricuspid inflow duration/cardiac cycle, degree of tricuspid regurgitation (TR), blood flow direction of arterial duct and ductus venosus. Multidisciplinary team including the maternal-fetal cardiology, pediatric cardiology, cardiac surgery, obstetrics, neonatology and anesthesiology was summoned to discuss the indications and timing of PFV. Two cases underwent ultrasound-guiding trans-abdominal PFV at the 28 weeks of gestational age. Echocardiography was performed to observe the opening and closing of the pulmonary valve, and to evaluate the development of right ventricle and improvement in hemodynamics every 2-4 weeks until delivery.@*Results@#From the technical perspective, pulmonary balloon valvuloplasty was successfully performed in these two cases. The opening of pulmonary valve improved in these two cases at 2-4 weeks after FPV. However, an obvious restenosis was detected in the first case at 5-8 weeks after FPV. In the first case, the echocardiography parameters including TV/MV, RV/LV, PV/AV and tricuspid inflow duration/cardiac cycle increased from 0.56, 0.42, 0.85,0.26 to 0.59, 0.51, 0.87, 0.32 at 5-8 weeks after FPV, respectively. However, the direction of blood flow through the arterial duct was still reverse. In the second case, TV/MV, RV/LV, PV/AV and tricuspid inflow duration/cardiac cycle ratio increased from 0.70, 0.63, 0.91,0.35 to 0.80, 0.80, 0.97, 0.42 at 5-8 weeks after FPV, respectively. The direction of blood flow through the arterial duct changed to bidirectional. Both fetuses were born alive. The first case underwent pulmonary valve commissurotomy and modified Blalock-Taussig shunt on the 8th day after delivery and received follow-up for 6 months. The strategy for the next-step therapy was still pending. The second case underwent transcutaneous pulmonary balloon valvuloplasty on the 19th day after delivery and received follow-up for 3 months. The opening of pulmonary valve improved obviously and the cardiac function was normal in the second case.@*Conclusions@#FPV is safe and effective for fetus during the second and third trimester of pregnancy, and FPV is beneficial for the development of fetal ventricle, valve and large artery. In addition, FPV may help to avoid the postnatal surgery for isolated single ventricle, improve fetal heart failure and prevent fetal death.