Surgical treatment and perioperative multidisciplinary management of neuromuscular scoliosis in children / 中国小儿急救医学

Scoliosis is a common deformity in neuromuscular disease, which usually has the characteristics of early onset age, severe degree of deformity, and rapid progression.Neuromuscular scoliosis often leads to serious damages to the quality of life, and results in the loss of walking, standing and sitting, and cardiopulmonary insufficiency.Surgical treatment can improve the quality of life for children with neuromuscular scoliosis, but surgical treatment is still challenging due to the complex surgery and many complicated diseases.The complications are much higher than those of idiopathic scoliosis.A multidisciplinary team is necessary in the surgical treatment of neuromuscular scoliosis to promptly and effectively reduce the complications.

Progress and challenges in diagnosis and treatment of neuromuscular disease in children / 中国小儿急救医学

Neuromuscular disease (NMD) is a group of disorders that affect the nervous system and muscles, leading to weakness, wasting, and the causes are diverse, often causing disability and death.In recent years, there have been significant advances in the diagnosis and treatment of NMDs in children, including new gene therapies, drug therapies, and rehabilitation interventions.However, there are still many challenges and problems that need to be addressed in the management of these conditions.Only through further research and investment, we can provide better treatment and support for patients, thus improve their quality of life.

Disease-modifying therapy for spinal muscular atrophy / 中华实用儿科临床杂志

Spinal muscular atrophy (SMA) was a severe inherited neuromuscular disease caused by the deficiency of the survival motor neuron (SMN) protein encoded by SMN1 gene with pathogenic variants.According to the age of onset and maximal achievement of motor function, SMA was classified into 4 subtypes.The most common and severe subtype was SMA type 1.During the natural course, most of patients with SMA type 1 die of respiratory failure within 2 years of age if not treated.Patients with other subtypes have progressive muscle weakness and atrophy at varying degrees.Before the emergence of disease-modifying therapy, a multidisciplinary management, including physical therapy, respiratory and nutritional support, has been the only approach to delay the disease progression and enhance the survival rate of SMA.However, motor milestone progress is unable to achieve by SMA patients, because the lack of SMN protein has not been solved.In the past 5 years, 3 drugs have been approved for the treatment of SMA.Clinical trials and a growing number of real-world study data have shown that medications of disease-modifying agents contribute to effectively improve motor function in SMA patients with different subtypes, and promote the motor milestone progress in some patients, which significantly reduce the mortality.However, treatment response of disease-modifying agents to SMA varies with the subtypes of SMA, individualized conditions and courses of disease.Therefore, it is particularly important to choose an optimal treatment to ensure the quality of life of patients.This review focuses on recent advances and emerging challenges in the treatment of SMA.

Clinical and genetic characteristics of 9 rare cases with coexistence of dual genetic diagnoses / 中华儿科杂志

Objective: To analyze the clinical and genetic characteristics of pediatric patients with dual genetic diagnoses (DGD). Methods: Clinical and genetic data of pediatric patients with DGD from January 2021 to February 2022 in Peking University First Hospital were collected and analyzed retrospectively. Results: Among the 9 children, 6 were boys and 3 were girls. The age of last visit or follow-up was 5.0 (2.7,6.8) years. The main clinical manifestations included motor retardation, mental retardation, multiple malformations, and skeletal deformity. Cases 1-4 were all all boys, showed myopathic gait, poor running and jumping, and significantly increased level of serum creatine kinase. Disease-causing variations in Duchenne muscular dystrophy (DMD) gene were confirmed by genetic testing. The 4 children were diagnosed with DMD or Becker muscular dystrophy combined with a second genetic disease, including hypertrophic osteoarthropathy, spinal muscular atrophy, fragile X syndrome, and cerebral cavernous malformations type 3, respectively. Cases 5-9 were clinically and genetically diagnosed as COL9A1 gene-related multiple epiphyseal dysplasia type 6 combined with NF1 gene-related neurofibromatosis type 1, COL6A3 gene-related Bethlem myopathy with WNT1 gene-related osteogenesis imperfecta type XV, Turner syndrome (45, X0/46, XX chimera) with TH gene-related Segawa syndrome, Chromosome 22q11.2 microduplication syndrome with DYNC1H1 gene-related autosomal dominant lower extremity-predominant spinal muscular atrophy-1, and ANKRD11 gene-related KBG syndrome combined with IRF2BPL gene-related neurodevelopmental disorder with regression, abnormal movement, language loss and epilepsy. DMD was the most common, and there were 6 autosomal dominant diseases caused by de novo heterozygous pathogenic variations. Conclusions: Pediatric patients with coexistence of double genetic diagnoses show complex phenotypes. When the clinical manifestations and progression are not fully consistent with the diagnosed rare genetic disease, a second rare genetic disease should be considered, and autosomal dominant diseases caused by de novo heterozygous pathogenic variation should be paid attention to. Trio-based whole-exome sequencing combining a variety of molecular genetic tests would be helpful for precise diagnosis.

Mechanism of Sijunzi Decoction in treatment of Alzheimer's disease based on UPLC-Q-TOF-MS, network pharmacology, and experimental verification / 中国中药杂志

The study identified the blood-entering components of Sijunzi Decoction after gavage administration in rats by UPLC-Q-TOF-MS/MS, and investigated the mechanism of Sijunzi Decoction in treating Alzheimer's disease by virtue of network pharmacology, molecular docking, and experimental verification. The blood-entering components of Sijunzi Decoction were identified based on the mass spectra and data from literature and databases. The potential targets of the above-mentioned blood-entering components in the treatment of Alzheimer's disease were searched against PharmMapper, OMIM, DisGeNET, GeneCards, and TTD. Next, STRING was employed to establish a protein-protein interaction(PPI) network. DAVID was used to perform the Gene Ontology(GO) annotation and the Kyoto Encyclopedia of Genes and Genomes(KEGG) pathway enrichment. Cytoscape 3.9.0 was used to carry out visual analysis. AutoDock Vina and PyMOL were used for molecular docking of the blood-entering components with the potential targets. Finally, the phosphatidylinositol 3-kinase(PI3K)/protein kinase B(Akt) signaling pathway enriched by the KEGG analysis was selected for validation by animal experiments. The results showed that 17 blood-entering components were detected in the serum samples after administration. Among them, poricoic acid B, liquiritigenin, atractylenolide Ⅱ, atractylenolide Ⅲ, ginsenoside Rb_1, and glycyrrhizic acid were the key components of Sijunzi Decoction in treating Alzheimer's disease. HSP90AA1, PPARA, SRC, AR, and ESR1 were the main targets for Sijunzi Decoction to treat Alzheimer's disease. Molecular docking showed that the components bound well with the targets. Therefore, we hypothesized that the mechanism of Sijunzi Decoction in treating Alzheimer's disease may be associated with the PI3K/Akt, cancer treatment, and mitogen-activated protein kinase(MAPK) signaling pathways. The results of animal experiments showed that Sijunzi Decoction significantly attenuated the neuronal damage in the hippocampal dentate gyrus area, increased the neurons, and raised the ratios of p-Akt/Akt and p-PI3K/PI3K in the hippocampus of mice. In conclusion, Sijunzi Decoction may treat Alzheimer's disease by activating the PI3K/Akt signaling pathway. The findings of this study provide a reference for further studies about the mechanism of action and clinical application of Sijunzi Decoction.

Analysis of 4 children with DYNC1H1 gene related spinal muscular atrophy with lower extremity predominant 1 / 中华儿科杂志

Objective: To investigate the clinical features and gene variation characteristics of children with dynein cytoplasmic 1 heavy chain 1 (DYNC1H1) gene associated spinal muscular atrophy with lower extremity predominant (SMALED) 1. Methods: The clinical data of 4 SMALED1 children admitted to Peking University First Hospital from December 2018 to May 2021, who were found to have pathogenic variation of DYNC1H1 gene through genetic testing, except for other genes known to be related to motor retardation, were retrospectively summarized to analyze the phenotype and genotype characteristics. Results: There were 3 males and 1 female. The age of onset was 1 year, 1 day, 1 day and 4 months, respectively. The age of diagnosis was 4 years and 10 months, 9 months, 5 years and 9 months, and 3 years and 1 month, respectively. The clinical manifestations were muscle weakness and muscular atrophy of lower limbs, 2 cases with foot deformity, 1 case with early non progressive joint contracture, 1 case with hip dislocation and 1 case with mental retardation. De novo heterozygous missense variations in DYNC1H1 gene were found in all 4 children. According to the rating of American College of medical genetics and genomics, they were all possible pathogenic and pathogenic variations, with p.R598C, p.P776L, p.Y1109D variations had been reported, and p.I1086R variation had not been reported. Conclusions: For those with unexplained lower limb muscle weakness, muscle atrophy, joint contracture and foot deformity, upper limb motor ability related retention, with or without mental retardation, as well as the motor ability progresses slowly, it is necessary to consider the possibility of SMALED1 and the detection of DYNC1H1 gene when necessary.

Transrectal ultrasound examination of prostate cancer guided by fusion imaging of multiparametric MRI and TRUS: avoiding unnecessary mpMRI-guided targeted biopsy / 亚洲男科学杂志(英文版)

The purpose of this study was to explore transrectal ultrasound (TRUS) findings of prostate cancer (PCa) guided by multiparametric magnetic resonance imaging (mpMRI) and to improve the Prostate Imaging Reporting and Data System (PI-RADS) system for avoiding unnecessary mpMRI-guided targeted biopsy (TB). From January 2018 to October 2019, fusion mpMRI and TRUS-guided biopsies were performed in 162 consecutive patients. The study included 188 suspicious lesions on mpMRI in 156 patients, all of whom underwent mpMRI-TRUS fusion imaging-guided TB and 12-core transperineal systematic biopsy (SB). Univariate analyses were performed to investigate the relationship between TRUS features and PCa. Then, logistic regression analysis with generalized estimating equations was performed to determine the independent predictors of PCa and obtain the fitted probability of PCa. The detection rates of PCa based on TB alone, SB alone, and combined SB and TB were 55.9% (105 of 188), 52.6% (82 of 156), and 62.8% (98 of 156), respectively. The significant predictors of PCa on TRUS were hypoechogenicity (odds ratio [OR]: 9.595, P = 0.002), taller-than-wide shape (OR: 3.539, P = 0.022), asymmetric vascular structures (OR: 3.728, P = 0.031), close proximity to capsule (OR: 3.473, P = 0.040), and irregular margins (OR: 3.843, P = 0.041). We propose subgrouping PI-RADS score 3 into categories 3a, 3b, 3c, and 3d based on different numbers of TRUS predictors, as the creation of PI-RADS 3a (no suspicious ultrasound features) could avoid 16.7% of mpMRI-guided TBs. Risk stratification of PCa with mpMRI-TRUS fusion imaging-directed ultrasound features could avoid unnecessary mpMRI-TBs.

Expert consensus for Jiegu Qili Tablets (Capsules) in treatment of fracture in clinical practice / 中国中药杂志

Fracture is one of the common diseases in the department of orthopaedics and traumatology. Jiegu Qili Tablets(Capsules) are a Chinese patent medicine commonly used to treat fractures in clinical practice, which has been included in the Class A drugs of the catalog of medicines covered by the National Medical Insurance System. However, no consensus or guideline has yet been developed to guide clinicians based on an evidence-based approach in detail, which has severely limited the clinical value of this drug. According to the guiding principle of evidence as the key, consensus as the supplement, and experience as the reference, a consensus was developed in strict accordance with the steps stipulated in the expert consensus on clinical applications of proprietary Chinese medicines. Based on literature review and questionnaire survey, the consensus was a timely summary of the existing clinical evidence on the treatment of fractures with Jiegu Qili Tablets(Capsules), and incorporated the treatment experience of a number of clinical experts. The preparation process took more than a year and the consensus(GS/CACM 293-2021) was officially released by the China Association of Chinese Medicine in September 2021, with the participation of multidisciplinary experts from 27 organizations of Chinese and Western medicine and research institutions. This article introduces the background and objectives of the consensus in detail, and describes the main process of proposal, drafting, expert consensus, and consultation. In particular, 5 consensus recommendations and 12 consensus suggestions are formed with regard to the key issues of indications, treatment timing, dose, duration, and safety in the clinical application of Jiegu Qili Tablets(Capsules) for the treatment of fractures, which guide and standardize the rational use by clinicians and improve the accuracy and safety of drugs.

Distinguishment of XU Qiu-fu's thirteen ghost points / 中国针灸

In addition to the well-known thirteen ghost points in Qianjin Fang (Important Formulas worth a Thousand Gold Pieces), "XU Qiu-fu's thirteen ghost points" is recorded in other medical books. The contents of "XU Qiu-fu's thirteen ghost points" are not exactly same among these medical books, which mainly focus on the nomination and selection of the ghost points. Even though, there are many similarity when compared with the thirteen ghost points in Qianjin Fang in terms of acupoint selection, acupoint location and the sequence of needle insertion, as well as the therapeutic methods. The authors believe that "XU Qiu-fu's thirteen ghost points" is actually adapted based on the framework of the thirteen ghost points in Qianjin Fang.

Added value of shear-wave elastography in the prediction of extracapsular extension and seminal vesicle invasion before radical prostatectomy / 亚洲男科学杂志(英文版)

The purpose of this study was to analyze the value of transrectal shear-wave elastography (SWE) in combination with multivariable tools for predicting adverse pathological features before radical prostatectomy (RP). Preoperative clinicopathological variables, multiparametric magnetic resonance imaging (mp-MRI) manifestations, and the maximum elastic value of the prostate (Emax) on SWE were retrospectively collected. The accuracy of SWE for predicting adverse pathological features was evaluated based on postoperative pathology, and parameters with statistical significance were selected. The diagnostic performance of various models, including preoperative clinicopathological variables (model 1), preoperative clinicopathological variables + mp-MRI (model 2), and preoperative clinicopathological variables + mp-MRI + SWE (model 3), was evaluated with area under the receiver operator characteristic curve (AUC) analysis. Emax was significantly higher in prostate cancer with extracapsular extension (ECE) or seminal vesicle invasion (SVI) with both P < 0.001. The optimal cutoff Emax values for ECE and SVI were 60.45 kPa and 81.55 kPa, respectively. Inclusion of mp-MRI and SWE improved discrimination by clinical models for ECE (model 2 vs model 1, P = 0.031; model 3 vs model 1, P = 0.002; model 3 vs model 2, P = 0.018) and SVI (model 2 vs model 1, P = 0.147; model 3 vs model 1, P = 0.037; model 3 vs model 2, P = 0.134). SWE is valuable for identifying patients at high risk of adverse pathology.

Multiparametric ultrasound for the assessment of testicular lesions with negative tumoral markers / 亚洲男科学杂志(英文版)

The purpose of this study was to evaluate the diagnostic performance of multiparametric ultrasound (mpUS; grayscale US, color Doppler US, strain elastography, and contrast-enhanced US) in the assessment of testicular lesions with negative tumoral markers. MpUS imaging data, patient age, serum tumor markers, scrotal pain, cryptorchidism, and related clinical information were retrospectively collected for patients who underwent mpUS examination between January 2013 and December 2019. Histologic results or follow-up examinations were used as the reference standard. In total, 83 lesions from 79 patients were included in the analysis. Fifty-six patients were finally diagnosed with benign tumors, and 23 patients were ultimately diagnosed with malignant tumors. Chi-square tests or Fisher's exact tests were used to assess the difference between the two groups. Stepwise multivariate logistic regression analysis showed that lesion diameter (odds ratio [OR] = 1.072, P = 0.005), vascularization on color Doppler US (OR = 4.066, P = 0.001), and hyperenhancement during the early phase (OR = 6.465, P = 0.047) were significant independent risk factors for malignancy; however, when compared with neoplastic lesions, pain (OR = 0.136, P < 0.001), absence of vascularization on color Doppler US (OR = 1.680, P = 0.042), and nonenhancement during the late phase (OR = 3.461, P = 0.031) were strongly associated with nonneoplastic lesions. MpUS features are useful for differentiating testicular lesions with negative tumoral markers and improving the preoperative diagnosis, which may avoid inappropriate radical orchiectomy.

Changes in retinal nerve fiber layer and macular retinal thickness and predictive value of outcome in children with abnormal refractive amblyopia / 国际眼科杂志(Guoji Yanke Zazhi)

AIM: To investigate the changes in retinal nerve fiber layer(RNFL)and macular retinal thickness(MRT)in children with refractive abnormalities and amblyopia, and their predictive value of outcome.METHODS: A total of 168 children with myopic refractive abnormalities and monocular amblyopia admitted to our hospital from January 2020 to October 2022 were selected as the observation group, with 118 cases of mild to moderate amblyopia and 50 cases of severe amblyopia, and 168 children with normal vision were included as the control group in a 1:1 ratio during the same period. The changes of RNFL and MRT in two groups of children were statistically counted, and the correlation between the severity of refractive abnormalities and RNFL and MRT in children with amblyopia was analyzed. Additionally, the observation group was divided into effective subgroup and ineffective subgroup based on the therapeutic effect. The general information, as well as RNFL and MRT of the effective subgroup and the ineffective subgroups before and after treatment were compared. Logistic was used to analyze the factors influencing efficacy, and ROC curves was plotted to analyze the predictive value of RNFL and MRT alone or in combination for efficacy.RESULTS: RNFL and MRT of cases of severe amblyopia were higher than those of the mild to moderate amblyopia and the control groups(all P&#x0026;#x003C;0.05); the severity of amblyopia in children with refractive abnormalities is positively correlated with RNFL and MRT(rs=0.745 and0.724, both P&#x0026;#x003C;0.001); among patients of mild to moderate and severe, there were statistically significant differences between the effective and ineffective subgroups in terms of initial treatment age, fixation form, treatment compliance, as well as RNFL, MRT, and their differences before and 1mo postoperatively(all P&#x0026;#x003C;0.05). Logistic analysis showed that initial treatment age, fixation nature, treatment compliance, RNFL and MRT before and 1mo postoperatively were all factors influencing the therapeutic effect of amblyopia with refractive abnormalities in children(all P&#x0026;#x003C;0.05); after 1mo of treatment, the combined prediction of RNFL and MRT was significantly better than that of single prediction in children with mild to severe amblyopia.CONCLUSION:There are differences in RNFL and MRT in children with abnormal refractive amblyopia, and they are closely related to the different degrees and curative effects of children. The combination of RNFL and MRT after 1mo of treatment has certain value in predicting children with different degrees of abnormal refractive amblyopia.

Synchronous tele-ultrasonography is helpful for a naive operator to perform high-quality thyroid ultrasound examinations

Purpose@#This study investigated the value of synchronous tele-ultrasonography (TUS) for naive operators in thyroid ultrasonography (US) examinations. @*Methods@#Ninety-seven patients were included in this prospective, parallel-controlled trial. Thyroid scanning and diagnosis were completed by resident A independently, resident B with guidance from a US expert through synchronous TUS, and an on-site US expert. The on-site expert’s findings constituted the reference standard. Two other off-site US experts analyzed all data in a blind manner. Inter-operator consistency between the two residents and the on-site US expert for thyroid size measurements, nodule measurements, nodule features, American College of Radiology (ACR) Thyroid Imaging Reporting and Data System (TI-RADS) categories, and image quality was compared. Two questionnaires were completed to evaluate the clinical benefit. @*Results@#Resident B detected more nodules consistent with the on-site expert than resident A did (89.4% vs. 56.5%, P0.75), while resident A achieved lower consistency in ACR TI-RADS categories, composition, echogenicity, margin, echogenic foci, and vascularity (all ICCs 0.40-0.75). Residents A and B had excellent consistency in target nodule measurements (all ICCs >0.75). Resident B achieved better performance than resident A for gray values, time gain compensation, depth, color Doppler adjustment, and the visibility of key information (all P<0.05). Furthermore, 61.9% (60/97) of patients accepted synchronous TUS, and 59.8% (58/97) patients were willing to pay for it. @*Conclusion@#Synchronous TUS can help inexperienced residents achieve comparable thyroid diagnostic capability to a US expert.

Primary practice of transcatheter edge-to-edge repair for mitral regurgitation: Early results of MitraClip in multiple centers / 中国胸心血管外科临床杂志

@#Objective    To investigate the early clinical results of MitraClip system in domestic patients. Methods     We retrospectively analyzed the clinical data of 36 patients who underwent transcatheter edge-to-edge repair procedure using MitraClip system in Beijing Fuwai Hospital, Shenzhen Fuwai Hospital and Fuwai Yunnan Cardiovascular Hospital between January and June 2021. There were 24 males and 12 females, with a median age of 70 (47-86) years. Ten (27.8%) patients had 3+ mitral regurgitation (MR) and 26 (72.2%) patients had 4+ MR preoperatively. Results    All procedures were successfully performed. The reduction in MR was 2+ at least immediately after surgery, and 91.7% of patients had MR≤2+ at 3 days postoperatively. There was no statistical difference in left ventricular ejection fraction change postoperatively. Forward velocity and peak gradient of mitral valve were increased after the procedure. Mean gradient of mitral valve were increased at 3 days postoperatively than immediately after surgery (P<0.001). Two patients had acute pericardial effusion intraoperatively, and received pericardial puncture and drainage immediately. Conclusion    MitraClip system has been applied well in domestic patients and can significantly improve MR. This sutdy has a good consistency with foreign studies, and the early results are satisfactory.

Role of complement system in the pathogenesis of uveitis / 国际眼科杂志(Guoji Yanke Zazhi)

Uveitis is a group of inflammation diseases involving the iris, ciliary body, choroid, vitreous body, retina and retinal vessels, which can lead to visual deterioration and even visual loss. The pathogenesis of uveitis is complex and diverse, including infection, autoimmunity, trauma, physical and chemical injury, immune genetic mechanism and so on. Recent studies have shown that the activation of complement system is one of the pathogenesis of uveitis. Various complement proteins, including CFH, CFB, CFI, MAC, CD59 and so on, regulate host tissue damage through a rigorous mechanism mediated by the complement system. And studies have found that those complement proteins are involved in the occurrence and development of uveitis at the gene level and biological function. In addition, complement components like C2, C3, C4 and C5 can affect the pathogenesis of uveitis in terms of copy number variation, gene polymorphism and the regulation of T-cell-mediated autoimmune response. Therefore, complement inhibition therapy and related gene therapy provide new ideas and targets for the treatment of uveitis.

Influencing factors of nucleic acid negative conversion in patients with mild and common COVID-19 induced by the Omicron variant of SARS-COV-2 / 浙江大学学报·医学版

OBJECTIVE@#To observe the clinical characteristics of mild and common COVID-19 patients infected with the Omicron variant, and to analyze related factors affecting the time to negative conversion of viral nucleic acid detection.@*METHODS@#Clinical data of 1781 patients with coronavirus disease 2019 (COVID-19) admitted to a cabin hospital in Shanghai from April 12 to May 26, 2022, were retrospectively analyzed, including age, gender, height, weight, clinical symptoms, comorbid diseases, COVID-19 vaccination, treatment, and nucleic acid negative conversion time. Univariate and multivariate logistic regression analyses were used to analyze the influencing factors of nucleic acid negative conversion time.@*RESULTS@#Among the 1781 patients, 995 were male and 786 were female, with a median age of 39 (30, 52) years. There were 727 patients (40.8%) with overweight and obesity [body mass index (BMI) > 24 kg/cm 2) and 413 patients (23.2%) had comorbid diseases. 205 cases (11.5%) were not vaccinated while 1576 cases were vaccinated. There were 1233 cases (69.2%) with one or more symptoms. The main clinical symptoms were cough (60.3%), expectoration (50.4%) and fever (36.9%). 1444 cases (81.0%) were treated with Chinese medicine, 78 cases (4.4%) were treated with western medicine, 14 cases (0.8%) were treated with integrated Chinese and western medicine, and 245 cases (13.8%) did not receive any medical treatment. All patients improved and were discharged. The median nucleic acid negative conversion time was 10.3 (7.4, 12.4) d. Univariate and multivariate analysis showed that, age ≥ 60 years ( OR＝1.537, 95% CI: 1.116 - 2.115, P<0.01), BMI > 24 kg/cm 2 ( OR＝1.344, 95% CI: 1.106 - 1.634, P<0.01 ) and hypertension ( OR＝1.518, 95% CI: 1.094 - 2.106, P<0.05) were independent risk factors for prolonged nucleic acid negative conversion. COVID-19 vaccination ( OR=0.548, 95% CI: 0.398 - 0.755, P<0.01) was a protective factor, that is, vaccination shortened the time for the nucleic acid test to become negative.@*CONCLUSIONS@#The symptoms of the Omicron variant infection were relatively mild and occult. Age ≥ 60 years old, comorbid hypertension, no vaccination and BMI > 24 kg/cm 2 are independent influencing factors for prolonged nucleic acid negative conversion.

Effect of Taohong Siwu Decoction() early intervention on mesenchymal stem cells homing in fracture healing in rats / 中国骨伤

OBJECTIVE@#To observe the effects of Taohong Siwu Decoction(, THSWD) on the mesenchymal stem cells(MSCs) migration, homing number and cytokine expression in callus during the early process of fracture healing, and to explore the mechanism of THSWD on accelerationg fracture healing by regulating the homing of MSCs in rats.@*METHODS@#A rat model of right femoral shaft open fracture was established. Thirty-two 5-week-old male Sprague-Dawley rats, weighting 110 to 130 g, were divided into control group, low-dose group, medium-dose group and high-dose group by using random number table. Distilled water was given to the control group, and the other groups were given Taohong Siwu Decoction. The rats were gavaged twice a day for 5 consecutive days after surgery. Bone volume/tissue volume(BV/TV) and bone mineral density(BMD) were observed using micro-computed tomography (micro-CT) at 21 days after surgery. At 5 days post-fracture, peripheral blood MSCs from THSWD treated and untreated rats were cultured in vitro. Subsequently, the migration ability of MSCs was observed by cell migration assay. The number of MSCs homing to the callus at the early stage of fracture (5 d) was detected by Immunohistochemistry (IHC). Protein chip was used to detect the expression of cytokines in callus.@*RESULTS@#Micro-CT results showed that BV/TV was higher in the high-dose group than in the medium-dose group (P=0.032), and higher in the medium-dose group than in the low-dose group(P=0.041), with no difference between the control and low-dose group (P=0.651). In addition, there was no difference in BMD between low-dose group and the model group (P=0.671), and lower in the low-dose group than in the medium-dose group(P=0.018), and the medium-dose group was lower than the high-dose group(P=0.008). Cell migration assay showed that THSWD promotes enhanced the migration ability of peripheral blood MSCs. IHC assay revealed that CD45-, CD90+, CD29+ MSCs significantly increased in bone callus after THSWD intervention compared with the control group. Protein chip showed that THSWD promoted the upregulation of CINC-1(×2.91), CINC-3(×1.59), LIX(×1.5), Thymus Chemokine (×2.55), VEGF (×1.22) and the down-regulation of TIMP-1 (×2.98).@*CONCLUSION@#THSWD, a representative formula of "promoting blood circulation and removing blood stasis", can significantly accelerate fracture healing, and its mechanism may be related to enhancing the migration ability of peripheral blood MSCs and up-regulating CINC-1, CINC-3, LIX, Thymus Chemokine, VEGF and down-regulating TIMP-1 in bone callus, which promotes the peripheral blood MSCs homing in the early stage of fracture.

Application of modified Delphi method in the construction of curriculum of emergency professional postgraduates / 中华医学教育探索杂志

Objective:At present, the postgraduate education of emergency medicine in China mainly relies on the internal medicine system and lacks specialty pertinence. The purpose of this study is to establish a compulsory curriculum system for professional postgraduates of emergency medicine.Methods:Modified Delphi method was used to subscribe questionnaires to experts, and the contents were mainly about medical education management and research of emergency in the affiliated teaching hospitals of Peking University. Microsoft Excel 2016 was used for double entry of questionnaire contents, and SPSS 22.0 software was used for statistics.Results:The panel was consisted of 14 experts in total and two rounds of Delphi questionnaires were completed. The response rate of the two rounds were both 100%. The authority coefficient of experts was > 0.9 and the average score of each evaluation index was 4.07-5.00 points. The coefficient of variation of each index was 0-0.22 and the Kendall coefficient of concordance was 0.07-0.10.Conclusion:As a result, a comprehensive emergency professional postgraduate curriculum system has been established.

Clinical study of linear scleroderma " en coup de sabre " with central nervous system involvement in children / 中华实用儿科临床杂志

Objective:To investigate the clinical features, imaging features, treatment options and prognosis of linear scleroderma with central nervous system involvement.Methods:One case of linear scleroderma " en coup de sabre" (LSES) school-age child suffering from dizziness, vomiting and blurred vision was admitted to the Department of Pediatrics, Peking University First Hospital on March 25, 2019.The curative effect was observed after treatment.The relevant literature was searched, and the characteristics of cases and therapeutic effects were reviewed.Results:The clinical features of this case included recurrent and transient dizziness, vomiting, and blurred vision.Cranial imaging indicated abnormal signals in the left frontotemporal lobe white matter, cingulate gyrus, basal ganglia region, and corpus callosum proximal pressure part, multiple soft meningeal line enhancement and abnormal brain substance enhancement on the brain surface in the lesion area.After 2 months of combined treatment with Methotrexate(MTX) and corticosteroids, some symptoms such as dizziness and vomiting disappeared.Three months after the treatment, in the primary cerebral hemisphere and multiple calcifications in the brain parenchyma, the lesions significantly reduced in cranial imaging.The child was followed up for 11 months and displayed no clinical symptoms.New hair was dense at the alopecia area, and skin color, texture and grain were close to normal at the damaged area.In the review of domestic literature, treatment and prognosis were not involved.Foreign literatures reported 5 cases of children, with the first choice of Methylprednisolone being combined with MTX treatment, significant effect was observed, and consistent with the treatment of this case.Conclusions:In order to detect and treat them as early as possible and improve the prognosis, LSES patients should undergo cranial integrity assessment and neurological imaging examination at an early stage, regardless of clinical manifestations of nervous system involvement.

Long-term rituximab treatment of refractory idiopathic inflammatory myopathy: A report of 3 cases / 北京大学学报(医学版)

Idiopathic inflammatory myopathies are a group of rare but serious diseases. The treatment of refractory idiopathic inflammatory myopathy is always challenging, especially in children. Three cases of refractory idiopathic inflammatory myopathy treated by rituximab were reported and discussed with the review of relevant literature. All were female with on-set age of 8 years and 6 months, 11 years and 7 months, 4 years and 2 months old, respectively. All had acute onset, presenting with progressive and severe muscle weakness. All lost ambulation within 1 or 2 months, with difficult swallowing and low voice. Respiratory distress occurred in case 2 after an attack of asphyxia due to an aspiration of sputum, and ventilator support was required for 1 month. Rashes were detected at the initial stage of the disease in cases 2 and 3. Patient 2 showed facial erythematous papules, spreading to her neck and hands. Patient 3 showed purplish eyelids with peri-orbital swelling, generalized edema involving all her limbs. Creatine kinase (CK) levels were markedly elevated in all the patients, ranging from 6 000 IU/L to 28 819 IU/L. Anti-SRP antibody was identified in cases 1, and anti-NXP2 antibodies were confirmed in cases 2 and 3. MRI of both thighs in all the patients showed profound muscle and fascial edema. Muscle pathology of patient 1 showed prominent fiber variation and endomysial fibrosis, with overexpression of MHC-Ⅰ. While muscle pathology in patients 2 and 3 showed scattered fiber necrosis, regeneration, endomysial edema without inflammatory cell infiltration. All the patients were diagnosed with idiopathic inflammatory myopathy and failed to the initial treatment including adequate glucocorticoids and high-dose immunoglobulin therapy. Other immunosuppressants (methotrexate, cyclophosphamide) were also tried in cases 2 and 3 with poor response. Then all the patients were treated with rituximab combined with glucocorticoids. Patient 1 regained normal strength and discontinued rituximab at the end of her last follow-up (2 years and 7 mouths). Though calcinosis developed during the follow-up period, significant improvement was noticed in cases 2 and 3 (both regained the ability to walk independently) at the end of their last follow-up after 2 years and 8 months, 3 years and 2 months respectively. Long-term rituximab therapy may improve the prognosis of refractory idiopathic inflammatory myopathy, especially with positive anti-SRP and anti-NXP2 antibodies.