Developmental epileptic encephalopathy associated with the CACNA1E gene mutation / 中华实用儿科临床杂志

One of the important causes of developmental epileptic encephalopathy (DEE) is the mutation of ion channel genes, including the mutation of the CACNA1E gene. CACNA1E-related DEE cases were first reported in 2018.The mutation types include new missense mutations, nonsense mutations and frameshift mutations, but the correlation between mutation sites and types with the phenotype of DEE is not clear.This review aims to summarize the reported CACNA1E-related DEE cases, and explore the correlation between the clinical phenotype of CACNA1E-related DEE and gene mutation sites and mutation types.Meanwhile, possible pathogenesis of CACNA1E-related DEE and the progress of drug intervention were reviewed to provide references for the diagnosis and precise treatment of DEE.

Research progress of ADPRHL2 gene related stress-induced childhood-onset neurodegeneration with variable ataxia and seizures / 中华实用儿科临床杂志

Stress-induced childhood-onset neurodegeneration with variable ataxia and seizures caused by ADPRHL2 gene mutation is a recently diagnosed hereditary neurodegenerative disease, featured by a small number of reported cases, early age of onset, and high mortality.Therefore, to attract the attention of clinicians, the research progress of ADPRHL2 gene, its protein structure and function, as well as the hereditary mode, pathogenic mechanism, clinical features, and treatment prognosis of stress-induced childhood-onset neurodegeneration with variable ataxia and seizures were reviewed in this paper.

Advances in the levels of neurotransmitters associated with attention deficit hyperactivity disorder in children with tic disorders / 国际儿科学杂志

Tic disorders(TD)including Tourette syndrome are childhood-onset neurodevelopmental disorders characterized by motor and/or vocal tics that commonly affect children′s physical and mental health.More than half of TD patients also have attention deficit hyperactivity disorder(ADHD). The pathogenesis of TD is not clear, and it is believed to be related to the abnormal level of neurotransmitters caused by the dysfunction of the cortical-striatal-thalamic-cortical circuitry, and the disorder of neurotransmitters is also related to the pathogenesis of ADHD, but the current research results on the level of neurotransmitters in TD with comorbid ADHD are controversial, and even some neurotransmitters show completely opposite changes in the two diseases.This paper reviews the research progress of neurotransmitters in children with TD co-suffering from ADHD in recent years, in order to provide ideas for exploring its pathogenesis.

A diabetic foot classification model based on radiomics features of fundus photographs / 中华内分泌代谢杂志

Objective:To construct a diabetic foot classification prediction model based on radiomics features of fundus photographs.Methods:A total of 2 035 fundus photographs of patients with type 2 diabetes diagnosed at Nanfang Hospital between December 2011 and December 2018 were retrospectively collected [282 photographs from patients with diabetic foot(DF), and 1 753 from patients with diabetes mellitus(DM)]. All fundus photographs were randomly divided into a training set(1 424 photos) and a test set(611 photos) using a computer generated random number at 7∶3. After pre-processing the fundus photographs, a total of 4 128 texture features based on the gray matrix were extracted by the Radiomic toolkit, and 11 339 other features were extracted using the ToolboxDESC toolkit. The LASSO algorithm was used to select the 30 features most relevant to DF, and then the Bootstrap + 0.632 self-sampling method was used to further select the 7 best combinations. Logistic regression analysis was used to obtain the regression coefficients and establish the final diabetic foot classification prediction model. ROC curve was drawn, and AUC, sensitivity, specificity, and accuracy of the training and test sets were calculated to verify its prediction performance. Results:We screened 7 fundus radiomics markers for diabetic foot patients, and based on this established a DF/DM classification prediction model. The AUC, sensitivity, specificity, and accuracy of the model were 0.958 6, 0.984 0, 0.920 0, and 0.928 0 in the training set, and 0.927 1, 0.988 9, 0.881 0, and 0.896 9 in the test set, respectively.Conclusion:In this study, seven DF fundus markers were screened using radiomics technology. Based on this, a highly accurate and easy-to-use DF/DM classification model was constructed. This technology has the potential to increase the efficiency of DF screening programs.

Establishment of the normal reference range of neurotransmitters in Han-nationality children aged 3-12 in Hubei province / 中华实用儿科临床杂志

Objective:To establish the normal reference range of neurotransmitters in Han-nationality children aged 3-12 in Hubei province.Methods:A prospective study was conducted on healthy Han-nationality children aged 3-12 who took physical examination in Wuhan Children′s Hospital, Hubei province from January to August 2021.The children were asked for their medical histories, and those with neurological diseases, psychiatric diseases, infection, trauma, and a drug history in the past 2 weeks were excluded.The plasma of 324 children (262 males, 62 females; 217 cases in the 3-7 years old, 107 cases in the 8-12 years old) and urine of 391 children (302 males, 89 females; 266 cases in the 3-7 years old, 125 cases in the 8-12 years old) were collected.They ultra performance liquid chromatography-mass spectrometry multiple techniques (UPLC-MS/MS) were used to detect 10 kinds of neurotransmitters (e.g., dopamine, epinephrine, glutamic acid, etc.) in plasma and 8 kinds of neurotransmitters (e.g., dopamine, epinephrine, 5-hydroxyindoleacetic acid, etc.) in random urine.The normal reference range of neurotransmitters in Han-nationality children aged 3-12 in Hubei province was established.The Kruskal- Wallis H test was made for statistical analysis of the differences in neurotransmitter levels among different age groups and gender groups.The neurotransmitter levels between different groups were compared by the Nemenyi test. Results:There were no significant differences in the levels of various neurotransmitters in children of different genders(all P>0.05). There were significant differences in the levels of dopamine, methoxy-norepinephrine, tryptophan and γ-aminobutyric acid in the plasma of children aged 3-7 years and 8-12 years.There were significant differences in the levels of dopamine, epinephrine, norepinephrine, methoxy-norepinephrine, high vanillic acid and 5-hydroxyindoleacetic acid in the random urine between the 3-7 years old group and the 8-12 years old group. Conclusions:The normal reference range of neurotransmitters in Han-nationality children aged 3-12 in Hubei province is established.This study provides reference for clinical practice and lays a foundation for the study of neurotransmitter-related diseases in children.

Research progress of refractory tic disorders / 中华实用儿科临床杂志

Refractory tic disorders (RTD) are a recently emerging concept gradually formed in pediatric neurology/psychiatry.Currently, the concept and diagnostic criteria of RTD remain unclarified, and it is extremely difficult to treat RTD.In this article, the definition, clinical features, influence factors, diagnosis, differential diagnosis, pharmacological treatment and non-pharmacological treatment of RTD in children were reviewed.

Interpretation to the European clinical guidelines of psychological interventions for Tourette syndrome and other tic disorders in 2021 / 中华实用儿科临床杂志

Tic disorders are one of the common neurodevelopmental disorders in children, which are mainly managed by pharmacological and non-pharmacological treatment.Psychological interventions belong to the non-pharmacological treatment, which, however, have not yet been fully recognized and understood in China.The analyses on clinical effectiveness and availability to pediatric patients are limited.This study aims to interpret the clinical guidelines of psychological interventions for Tourette syndrome and other tic disorders by European Society for the Study of Tourette syndrome in the journal of European Child and Adolescent Psychiatry in 2021, aiming to provide reference for the psychological interventions of tic disorders in China.

Clinical features and genetic analysis of the early-onset epileptic encephalopathy caused by sodium channel mutations / 中华实用儿科临床杂志

Objective:To explore the clinical phenotype characteristics of early-onset epileptic encephalopathy (EOEE) caused by sodium channel mutations.Methods:A retrospective study was used.A total of 52 EOEE patients treated in the Department of Neurology, Children′s Hospital of Fudan University and Department of Neurology, Wuhan Children′s Hospital, Tongji Medical College, Huazhong University of Science & Technology from June 2016 to June 2019 were recruited.Peripheral blood samples of 52 patients and their parents were collected for analyzing pathogenic mutations by the next generation sequencing and copy number variations of whole exons in family. Chi- square test was used to compare seizure control data among different voltage-gated sodium channel α1 subunit ( SCN1A) mutation types. Results:A total of 35/52 cases (67.3%) were diagnosed as Dravet syndrome, 3/52 cases (5.8%) were West syndrome, and 14/52 cases (26.9%) were non-symptomatic EOEE.The electroencephalogram (EEG) findings showed a large number of multifocal spikes, spike-slow waves, sharp waves, and sharp-slow waves.A total of 45/52 cases (86.5%) showed normal brain magnetic resonance imaging(MRI), 1 case had slightly widened bilateral frontal sulcus, 1 case had widened bilateral temporal pole and frontal top subarachnoid space, and the remaining 5 cases had widened extracerebral space and slightly larger ventricles.Thirteen cases were re-examined with brain MRI, and 3 cases had mild brain atrophy.A total of 43/52 cases (82.7%) were examined with SCN1A gene mutations, of which 28/52 cases (53.8%) were missense mutations, 5/52 cases (9.6%) were nonsense mutations, 7/52 cases (13.5%) were frameshift mutations and 3/52 cases (5.8%) were splice site mutations.A total of 3/52 cases (5.8%) had SCN2A mutations, of which 2/52 cases (3.8%) were missense mutations, and 1/52 case (1.9%) was a frameshift mutation, 1/52 cases (1.9%) carrying the missense mutation of the SCN3A gene.A total of 5/52 cases (9.6%) had missense mutations of the SCN8A gene.After an average of 1-year follow-up, a total of 13/52 cases (25.0%) had more than 1-year control of seizure, of which 6/52 cases (11.5%) with seizure control for more than 2 years, and 4/52 cases (7.7%) with more than 3-year control.Children carrying SCN1A missense mutations were relatively easier to be controlled for seizures than those carrying SCN1A truncation mutations (nonsense mutations+ frameshift mutations) ( P<0.05). In 5 children carrying SCN8A mutations, 2 cases of them had seizures control for more than 1 year after adding Oxcarbazepine, but the improvement of mental motor function was not obvious. Conclusions:In children with EOEE associated with sodium channel gene mutations, SCN1A, SCN2A, SCN3A, and SCN8A mutations were pathogenic factors.Among them, SCN1A was the most common pathogenic gene for EOEE, with the mutation rate of 82.7%.Dravet syndrome was the most common clinical phenotype of EOEE associated with sodium channel gene mutations.Epileptic seizures in children carrying SCN1A missense mutations were easier to be controlled than those with truncated mutation (nonsense mutations + frameshift mutations), suggesting that the gene mutation type was related to the degree of seizures control.Oxcarbazepine was effective in the treatment of EOEE with SCN8A gene mutations, indicating that the combination therapy using anti-epilepsy drugs can be applied to EOEE patients according to the type of gene function.

Influencing factors of image cleanliness scores of magnetically-controlled capsule endoscopy / 中华消化内镜杂志

Objective:To explore the influencing factors of the image cleanliness scores of magnetically-controlled capsule endoscopy (MCE) and the methods to improve cleanliness scores.Methods:Data of patients undergoing MCE from October 2017 to August 2020 in Peking University First Hospital were analyzed retrospectively. The cleanliness scores at six regions of the stomach (cardia, fundus, body, angularis, antrum, and pylorus) were recorded (1-4 points), and the sum of the scores from these six regions was also calculated. Clinical features [gender, age, body mass index, indication, use of esophagus cap, and medication of proton pump inhibitors(PPI)], and gastric preparation regimens (one dose of pronase: simethicone 5 mL + pronase 20 000 U + sodium bicarbonate 1 g; or two doses of pronase: simethicone 5 mL + pronase 40 000 U + sodium bicarbonate 2 g) were collected. Cleanliness scores were defined as poor (group P, total cleanliness scores≤18) and as good (group G, total cleanliness scores>18). Clinical features, indication, use of esophagus cap and PPI, and preparation regimes were compared. Factors influencing cleanliness scores were analyzed.Results:A total of 238 consecutive patients with median age of 61.5 (54.0, 76.3) years were recruited according to inclusion criteria, among whom 68(28.6%) were female. There were 35 patients (14.7%) in group P with median age of 67.0 (56.0, 83.0) years. There were 203 patients(85.3%)in group G with median age of 61.0 (53.0, 75.0) years. No significant differences were found in age, sex, body mass index or esophagus cap use( P>0.05). Indications of abdominal symptoms or digestive system diseases( OR= 2.899, 95% CI: 1.258-6.681, P=0.012) and PPI use ( OR=3.168, 95% CI: 1.261-7.959, P=0.014) were more likely to yield a low cleanliness score. Gastric preparation regimen with two doses of pronase ( OR=0.201, 95% CI: 0.067~0.603, P=0.004)was more likely to avoid a low cleanliness score. Conclusions:Indications of digestive system diseases or abdominal symptoms and PPI use may decrease the cleanliness scores of MCE, while gastric preparation with double doses of pronase may improve it.

Interpretation of expert consensus for diagnosis and treatment of tic disorders in China / 中华实用儿科临床杂志

Tic disorders (TD) is a kind of neuropsychiatric disorders developing during childhood and cha-racterized by tics.In August 2020, the TD Consortium Neurology Group of Chinese Pediatric Society, Chinese Medical Association has developed an English version of Expert Consensus on Diagnosis and Treatment of Tic disorders in children to help improve the diagnosis, treatment and long-term management of TD, as well as international communication.Now the consensus is interpreted as follows, including its pathophysiology, clinical characteristics, diagnosis, comorbidity, treatment and prognosis.

Clinical value of TDI combined with 2D-STI on evaluating the microcirculation dysfunction and left ventricular dysfunction in patients with non-obstructive coronary angina / 中华心血管病杂志

Objective: To explore the value of tissue Doppler imaging (TDI) combined with two-dimensional speckle tracking imaging (2D-STI) at rest on evaluating microcirculation dysfunction and left ventricular dysfunction in patients with angina and no obstructive coronary artery disease(ANOCA). Methods: This retrospective study recruited 78 ANOCA patients, who hospitalized in the People's Hospital of Liaoning Province from August 2019 to July 2021. These patients underwent conventional echocardiography examination, including TDI and 2D-STI, to evaluate the left ventricular dysfunction, and adenosine stress echocardiography (SE) to evaluate the coronary flow velocity reserve (CFVR). ANOCA patients were divided into coronary microcirculation dysfunction CMD group (CFVR<2) and control group (CFVR≥2) according to CFVR. Clinical data, routine echocardiographic parameters, TDI parameters including isovolumic contraction time (IVCT), isovolumic relaxation time (IVRT), ejection time (ET), and STI parameters including global longitudinal peak strain (GLS), time to peak (TTP); peak strain dispersion (PSD) were compared between the two groups. Binary logistic regression was used to analyze the risk factors of CMD and the predictive value of each parameter to construct a joint prediction model for the diagnosis of CMD in this patient cohort. Results: The mean age was (55.5±11.2) years, 43 (55%) patients were females in this patient cohort, 38 (49%) patienst were didvided into the CMD group and 40 (51%) into the control group. Age, prevalence of hypertension, diabetes, dyslipidemia, and smokers were significantly higher in the CMD group than in the control group (all P<0.05). Tei index was higher, IVCT and TTP were longer, PSD was higher, ET was shorter, and absolute GLS was lower in the CMD group than in the control group (all P<0.05). The results of logistic regression analysis showed that longer IVCT, higher Tei index, higher time to PSD and lower absolute GLS were the independent risk factors of CMD. The ROC curve revealed that the predicting efficacy on CMD was satisfactiory with the combined predictors: AUC=0.884, sensitivity of 82% and specificity of 80%. Conclusions: TDI combined with 2D-STI is associated with a good diagnostic value on the diagnosis of CMD and left ventricular dysfunction in patients with ANOCA, which provides a feasible non-invasive tool for the diagnosis of CMD and risk stratification of patients with ANOCA.

Research on regularity of traditional Chinese medicine in treatment of Alzheimer's disease based on data mining / 中国中药杂志

To explore prescription medication regularity in the treatment of Alzheimer's disease with traditional Chinese medicine(TCM). With Alzheimer's disease or senile dementia as the subject, collecting and sorting out the journal papers in CNKI were collected as the data source to establish the literature research database of Alzheimer's disease prescriptions, and then the association rule analysis, factor analysis and systematic cluster analysis on the included TCM were conducted. Among the 113 prescriptions included in the standard, the single herb Acori Tatarinowii Rhizoma was the most common. The herbs were mainly warm and flat among four pro-perties, mainly sweet, bitter and spicy among five flavors. The drugs were mainly distributed in five internal organs, and the most commonly used drugs were deficiency tonifying drugs as well as blood activating and stasis removing drugs. In the association rule analysis, it was found that there were 6 drug pairs with the highest association strength. Eight common factors were extracted from the factor analysis, and they were classified into 6 categories in the systematic cluster analysis. The results have shown that the overall principles in treating Alzheimer's disease with modern Chinese medicine are tonifying deficiency, invigorating circulation, activating blood and dispelling phlegm.

Strategies and progresses of stereotactic radiosurgery for intractable brain metastases / 中华放射医学与防护杂志

Brain metastases are common intracranial diseases featured with high incidence rate, difficult clinical treatment and poor prognosis. Especially for refractory brain metastases (large volume, multiple recurrence and metastatic tumors), it is difficult for conventional therapies to achieve ideal prognosis. Compared with conventional therapies, stereotactic radiosurgery (SRS) has the advantages of high accuracy, large fractional dose and low damage to the surrounding tissues, and has gradually become the primary choice for the treatment of brain metastases. SRS has a good effect in the treatment of refractory brain metastases, and can effectively inhibit tumor proliferation and improve the quality of life of patients. SRS combined with surgery, chemotherapy, molecular targeted therapy and immunotherapy can improve the local tumor control rate of patients with intractable brain metastasis, but whether it can improve the prognosis of patients is still controversial. The adverse reactions of combined therapies should also be concerned. Therefore, this article reviews the therapeutic effects of SRS in the treatment of refractory brain metastases, the strategies and progresses of combined treatment.

Progress in applying surfactants to lignocellulose hydrolysis for sugar production / 生物工程学报

Lignocellulose is a major biomass resource for the production of biofuel ethanol. Due to its abundance, environmental friendliness and renewability, the utilization of lignocellulose is promising to solve energy shortage. Surfactant can effectively promote the enzymatic hydrolysis of lignocellulose. By discussing the influence and mechanism of different surfactants on the enzymatic hydrolysis, we provide references for finding appropriate surfactants in enzymatic hydrolysis process.

Clinical phenotypes of epilepsy associated with GABRA1 gene variants / 中华儿科杂志

Objective@#To summarize the clinical phenotypes of epilepsy in patients with GABRA1 gene variants.@*Methods@#A total of 11 epileptic patients (4 boys and 7 girls) who were treated in the Department of Pediatrics, Peking University First Hospital from March 2016 to July 2019 and detected with GABRA1 gene heterozygous pathogenic variants by targeted next-generation sequencing were enrolled. The features of clinical manifestations, electroencephalogram (EEG), and neuroimaging were analyzed retrospectively.@*Results@#A total of 11 epileptic patients carried GABRA1 gene pathogenic variants, of whom 10 were de novo variants and the other one was inherited from the patient′s mother. Two patients had the same variants. Six variants were novel. Ages at seizure onset ranged from 3 to 14 months, and the median age was 8 months. The seizure was first observed within 1 year in 10 patients and beyond 1 year of age in 1 patient. Multiple seizure types were observed, including focal seizures in 10 patients, generalized tonic clonic seizures (GTCS) in 3 patients, myoclonic seizures in 3 patients, and epileptic spasm in 2 patients. There were 5 patients with multiple seizure types. Sensitivity to fever was observed in 9 patients, among whom 6 patients had a history of status epilepticus. Two patients had photoparoxysmal response. Five patients had abnormal EEG background, and 6 patients had abnormal discharges in EEG during interictal phase. Brain magnetic resonance imaging (MRI) was normal in all patients. Developmental delay in various degrees was present in 9 patients. Among the 11 patients, Dravet syndrome was diagnosed in 5 patients, West syndrome in 2 patients, undiagnosed early-onset epileptic encephalopathy in 1 patient, and focal epilepsy in the other 3 patients. The ages at the last follow-up ranged from 8 months to 12 years. During follow-up, 8 patients were seizure-free for 6 months to 8 years, and 1 patient had discontinuation of medication.@*Conclusions@#In epilepsy associated with GABRA1 gene variants, de novo pathogenic variants are more common than inherited. Most epilepsy caused by GABRA1 gene variants occurs in infancy. Most patients have multiple seizures and focal seizures are common. Most patients have a comparatively favorable prognosis, but they may still have varied degrees of developmental delay.

Study on the efficacy and influencing factors of Dabigatran in elderly patients / 中华老年医学杂志

Objective:To observe the efficacy of Dabigatran etexilate anticoagulation therapy and analyze its influencing factors in elderly patients.Methods:The clinical data of the elderly patients using Dabigatran etexilate anticoagulation for prevention or treatment of thrombosis in the Department of Geriatrics, Peking University First Hospital were collected.Changes of coagulation indexes and its influencing factors were analyzed when taking Dabigatran in different doses.Results:A total of 142 patients receiving Dabigatran therapy were collected, with an average age of(79.0±7.5)years.Among them, 128 patients had atrial fibrillation and 14 had deep venous thrombosis and/or pulmonary embolism.After taking Dabigatran, the trough values of activated partial thromboplastin time(APTT)and thrombin time(TT)were increased significantly in all patients compared with that before administration( P<0.001). In patients taking Dabigatran 110 mg once a day, APTT was(32.4±3.0)s before administration, with a trough value of(41.4±5.4)s and a peak value of(53.9±9.8)s.In patients taking Dabigatran 110 mg twice a day, APTT was(31.0±3.1)s before administration, with a trough value of(42.4±5.5)s and a peak value of(48.7±7.3)s.Compared with patients taking Dabigatran 220 mg/d, those who took Dabigatran 110 mg/d were older( P<0.0001), often women( P<0.0001), had lower body weight( P=0.001), body mass index( P=0.018)and creatinine clearance rate( P<0.001), and higher basal( P=0.008)and peak values( P=0.002)of APTT, but with similar trough values.Multivariate analysis showed that the APTT trough values were significantly related with APTT basal value( β=0.885, P<0.001), creatinine clearance rate( β=-0.121, P<0.001), dosage of Dabigatran( β=0.037, P<0.001)and combined amiodarone( β=3.784, P=0.022). Conclusions:There are individual differences in the anticoagulant effect of Dabigatran etexilate.The APTT trough value is higher in patients with high APTT basal value, high Dabigatran dose, low creatinine clearance rate and combined use of amiodarone.Dabigatran 110 mg/d was often used in women, elderly patients, and those with low body weight, and decreased renal function, and with a similar trough value and a high peak value of APTT compared with patients on Dabigatran 220 mg/d.

Application experience of integrated nursing mode for extremely severe burn patients in August 2nd Kunshan factory aluminum dust explosion accident / 中华烧伤杂志

Objective@#To explore the application experience of integrated nursing mode in the treatment of extremely severe burn patients in August 2nd Kunshan factory aluminum dust explosion accident.@*Methods@#On August 2nd, 2014, 35 extremely severe burn patients involved in the August 2nd Kunshan factory aluminum dust explosion accident were admitted to Wuxi Third People′s Hospital, including 18 males and 17 females, aged 21-50 years. According to the characteristics of the wounded, the situation of the nursing staff, and the characteristics of the nursing work, the integrated nursing mode was constructed and implemented to improve overall nursing quality. The standardized management measures such as cluster management of facilities and equipments in wards, improving and unifying nursing system, standardized training, drawing up " Nurses Compulsory Reading" , optimizing nursing shift handover and so on were taken. Professional quality control groups such as continuous renal replacement therapy (CRRT) group, static therapy group, airway group, and burn group were established, and standardized writing nursing group, wound nursing group, psychological nursing group, and enteral nutrition nursing group were set up under burn group. The treatment outcomes of patients and effects of nursing management, nursing methods, and specialty nursing were recorded.@*Results@#Twenty-seven patients survived the shock period, infection period, and recovery period smoothly. The success rate of rescue was 77.14%. During the treatment, the ward was in good order. The implementation rate of disinfection and isolation system, the completion rate of shift handover, the standard rate of intravenous therapy, the implementation rate of bed head elevation, the correct rate of posture placement, and the success rate of CRRT were all 100%. Successful turn over of rotating bed without interruption of CRRT for 24 hours was implemented in two patients. In many cases, the single filter for hemodialysis continuously run for more than 72 hours. The airway mucosa of patients healed around 20 days after injury. No adverse nursing events such as tracheal cannula detachment/blockage, respiratory distress, atelectasis, lung consolidation, aspiration by mistake, rotating bed rollover, ear chondritis, nasal septal pressure ulcer, vacuum sealing drainage (VSD) catheter blockage, VSD dressing leakage, severe abdominal distension/diarrhea, non-planned extubation/blockage of various intravenous treatment catheters implanted into deep veins and arteries were observed.@*Conclusions@#The integrated nursing mode significantly optimizes the nursing work process in the treatment of extremely severe mass burns, clarifies the duties of nursing staff, and improves the quality of nursing. This mode is worthy of taking reference by other burn treatment units.

Multi-disciplinary cooperative treatment and management experience of 35 patients with extremely severe burns involved in August 2nd Kunshan factory aluminum dust explosion accident / 中华烧伤杂志

On August 2nd, 2014, 35 patients with extremely severe burns involved in August 2nd Kunshan factory aluminum dust explosion accident, including 18 males and 17 females, aged from 21 to 50 years, were admitted to our unit. According to the patient′s condition, the rescue members divided the participants into groups according to their characteristics, and used the multi-disciplinary cooperative treatment and management mode of integrating critical care medicine, anesthesia, traditional Chinese medicine, rehabilitation, and nursing led by burn medicine. Totally 27 patients were successfully treated, with a success rate of 77.14%.

Comparative Study on Content of Medicinal Materials from Three Varieties of Sambucus williamsii / 中国实验方剂学杂志

Objective:To determine the content of active ingredients in root bark,stems,leaves and fruits of three varieties of Sambucus williamsii (Saline-toleratedvariety,Natural Red No. 1 variety,Qingzhou No.15 variety),and identify the chemical patterns of the chemical components. Method:The content of polysaccharides,flavonoids,phenolic acids and triterpene were determined by UV spectrophotometry (UV) colorimetry. The content of anthocyanin was determined by pH differential method. Ursolic acid,oleanolic acid,quercetin,protocatechuic acid,morroniside content were determined by high performance liquid chromatography (HPLC). The root bark,stems,leaves and fruits of three varieties were analyzed by chemical pattern recognition. Result:The quality of different samples was further evaluated by F3 and F6 comprehensive scores in principal component analysis. It was found that the root bark and leaves were higher in the comprehensive ranking,and the results were basically consistent with the quantitative results. The contents of polysaccharide and morroniside in Natural Red No. 1 variety root bark were the highest,which were 161.09 mg·g-1 and 8.33 mg·g-1,respectively. Flavonoids,rutin and quercetin were the highest in the Natural Red No. 1 variety leaf,which were 71.93,4.09 and 3.57 mg·g-1,respectively. Triterpene was the highest in Natural Red No. 1 variety fruit, which was 47.84 mg·g-1. Phenolic acid and anthocyanin were the highest in Saline-tolerated variety fruits,which were 11.25 mg·g-1 and 94.32 mg·g-1,respectively. The Saline-tolerated variety stem had the highest oleanolic acid content, which was 1.41 mg·g-1. Saline-tolerated variety leaf had the highest contents of ursolic acid and gallic acid,which were 2.12 and 0.34 mg·g-1, respectively. The highest content of protocatechuic acid in Qingzhou No. 15 variety root bark, which was 0.12 mg·g-1. Conclusion:The content of Natural Red No. 1 variety is higher than that of the other two varieties,with a good quality.

Clinical characteristics of PCDH19-female limited epilepsy / 中华儿科杂志

Objective@#To analyze the clinical characteristics of patients with PCDH19-female limited epilepsy (PCDH19-FE).@*Methods@#The clinical data of 60 female epilepsy patients with PCDH19 gene heterozygous variations at the Department of Pediatrics, Peking University First Hospital from October 2007 to December 2018 were collected and analyzed retrospectively, their clinical manifestations, accessory examination and follow-up treatment were summarized.@*Results@#Data of a total of 60 cases of PCDH19-FE were collected. The seizure onset occurred between 4 and 42 months of age (median: 11 months of age). Focal seizures occurred in 47 patients (78%), generalized tonic-clonic seizures (GTCS) occurred in 30 patients (50%), and other rare types of seizures included atypical absence, myoclonic, clonic, tonic, and atonic seizures. Two or more seizures types existed in 24 patients (40%), and seven patients (12%) had attacks of status epilepticus. Sensitivity to fever was observed in 47 out of them (78%) and clustering of seizures as found in all patients. During the interictal phase, focal discharges were monitored in 22 cases (22/45, 49%), multifocal discharges in 12 cases (12/45, 27%), widely discharging in 2 cases (4%), and both focal and widely discharging in 9 cases (20%). Clinical seizures were detected in 30 patients during the electroencephalogram (EEG) recording, including focal seizures in 22 cases, GTCS seizures in 8 cases, tonic seizure in three cases, myoclonic seizure followed by GTCS in one case, and two types of seizures in four cases. Before seizure onset, 57 patients had normal development and three patients had delayed language development. After seizure onset, varied degrees of intelligence disability were present in 38 cases (63%), language delay in 36 cases (60%), and gait instability in 10 cases (17%). Autistic features occurred in 17 cases (28%); and other behavioral problems like learning difficulties, personality, or emotional disorders existed in 33 cases (55%). Age at last follow-up ranged from one year and 3 months to 22 years and 3 months of age, 17 patients (28%) were seizure-free for more than 2 years (5 to 22 years at the last follow-up). The efficiency of antiepileptic drugs were 65% (33/51) in sodium valproate, 63% (27/43) in levetiracetam and 59% (20/34) in topiramate.@*Conclusions@#The clinical features of PCDH19-FE are characterized by clustering of seizures, focal seizures in most cases, sensitivity to fever mostly, focal discharges principally in EEG, varied degrees of intellectual disability or movement disorder, combined with autism spectrum disorders in partial and high efficiency in sodium valproate or levetiracetam treatment.