Tuberous sclerosis complex (TSC) is a rare autosomal dominant disorder.Primarily involving the skin and central nervous system, it also impacts the heart, lungs, kidneys, and other organs.The vast majority of TSC patients may experience neuropsychiatric symptoms during their lifetime, including behavioral, mental, intellectual, academic, neuropsychological, and psychosocial disorders, which are collectively referred to as TSC-associated neuropsychiatric disorders(TAND).The TAND Checklist is a screening tool designed to identify potential neuropsychiatric disorders by facilitating dialogues between TSC patients, their families, and clinicians.This article focuses on the concept and research history of TAND and the application of the TAND Checklist, contributing to the comprehensive and systematic clinical evaluation and understanding of the prognosis of children with TAND.

Objective:To analyze the difference of clinical characteristics and outcomes of infants with moderate and severe pediatric acute respiratory distress syndrome(PARDS)diagnosed according to baseline oxygenation index(OI) in pediatric intensive care unit(PICU).Methods:Second analysis of the data collected from the "Efficacy of pulmonary surfactant (PS) in the treatment of children with moderate and severe ARDS" program.Retrospectively compare of the differences in clinical data such as general condition, underlying diseases, OI, mechanical ventilation, PS administration and outcomes among infants with moderate and severe PARDS divided by baseline OI who admitted to PICUs at 14 participating tertiary hospitals from 2016 to December 2021.Results:Among the 101 cases, 55 cases (54.5%) were moderate and 46 cases (45.5%) were severe PARDS.The proportion of male in the severe group (50.0% vs.72.7%, P=0.019) and the pediatric critical illness score(PCIS)[72 (68, 78) vs.76 (70, 80), P=0.019] were significantly lower than those in the moderate group, while there was no significant difference regarding age, body weight, etiology of PARDS and underlying diseases.The utilization rate of high-frequency ventilator in the severe group was significantly higher than that in the moderate group (34.8% vs.10.9%, P=0.004), but there was no significant difference in PS use, fluid load and pulmonary complications.The 24 h OI improvement (0.26±0.33 vs.0.04±0.34, P=0.001) and the 72 h OI improvement[0.34 (-0.04, 0.62) vs.0.15 (-0.14, 0.42), P=0.029)]in the severe group were significantly better than those in the moderate group, but there was no significant difference regarding mortality, length of hospital stay and intubation duration after diagnosis of PARDS between the two groups. Conclusion:In moderate and severe(divided by baseline OI) PARDS infants with invasive mechanical ventilation, children in severe group have better oxygenation improvement in the early stage after PARDS identified and are more likely to receive high frequency ventilation compared to those in moderate group.Baseline OI can not sensitively distinguish the outcomes and is not an ideal index for PARDS grading of this kind of patient.

Objective To evaluate the clinical efficacy and safety of preoperative endovascular embolization of Solid Hemangioblastomas.Methods The data bases including Wan Fang,CNKI(China National Knowledge Infrastructure),VIP Database,PubMed、Medline、Springer were searched for the related studies.Two independent surgeons assessed trails for eligibility and quality,and all data marching the standards were abstracted for Meta-analysis by RevMan 5.3.Results 8 randomized controlled trails(RCT)were included.Selected analysis of embolized and non-embolized groups of Solid Hemangioblastomas were observed for variables of clinical efficacy in surgery time,number of blood loss and transfusions,complete resection,there were statistical difference.(P＜0.000 01,WMD=-1.18,95％CI[-1.16,-0.71];P＜0.000 01,WMD=-464.17,95％CI[-492.17,-437.24];P＜0.000 01,WMD=-238.81,95％CI[-282.84,-194.77];P＜0.006,RR=1.17,95％CI[1.05,1.31]).Conclusion The preoperative endovascular embolization is beneficial for Hemangioblastomas because it can shorten the time of surgery,diminish the necessity of intra-operative blood loss and transfusion,it also raises the ratio of complete resection of Solid Hemangioblastomas.

Enterotoxigenic Escherichia coli（ETEC）infection can induce watery diarrhea，leading to dehydration，electrolyte disturbance，and even death in severe cases. Recombinant B subunit/inactivated whole-cell cholera（rBS/WC）vaccine is effective in preventing ETEC infectious diarrhea. On the basis of the latest evidence on etiology and epidemiology of ETEC，as well as the effectiveness，safety，and health economics of rBS/WC vaccine，National Clinical Research Center for Child Health（The Children’s Hospital，Zhejiang University School of Medicine）and Zhejiang Provincial Center for Disease Control and Prevention invited experts to develop expert consensus on rBS/WC vaccine in prevention of ETEC infectious diarrhea. It aims to provide the clinicians and vaccination professionals with guidelines on using rBS/WC vaccine to reduce the incidence of ETEC infectious diarrhea.

ObjectiveTo identify potential distribution areas for wild Panax ginseng cultivated under forest in Liaoning province， China， and analyze the ecological factors of spatial stratified heterogeneity affecting its ecological suitability and quality suitability. MethodWild Panax ginseng samples cultivated under forest were collected from 33 cultivation bases in Liaoning province. The Maxent maximum entropy model and ArcGIS were used to delineate the ecological suitability zones. Correlation analysis was performed on seven indicators and 110 ecological factors. Variables with significant correlation （P<0.05） were used to build partial least squares regression analysis models. A comprehensive quality zoning was conducted using the analytic hierarchy process （AHP）. The geographic detector was employed to analyze the interactions among dominant ecological factors of spatial stratified heterogeneity affecting habitat suitability， quality suitability， and the ecological driving factors. ResultVegetation type was the most influential ecological factor for delineating the ecological suitability zones for wild Panax ginseng in Liaoning province. The main ecological suitability areas for wild Panax ginseng cultivated under forest were located in the northeast， east， and southeast regions along the line from Xifeng County to Gaizhou City. The comprehensive quality suitability of wild Panax ginseng cultivated under forest was highest in Kuandian County and Huanren County and gradually decreased to the northwest and southwest. Within the delineated regions， the suitability conditions and comprehensive quality of wild Panax ginseng cultivated under forest were primarily influenced by the interactions between radiation and precipitation factors. The content of the measured samples was significantly higher than the standards in the 2020 edition of the Chinese Pharmacopoeia， indicating the high overall quality of wild Panax ginseng in Liaoning Province. ConclusionAccording to the zoning and prediction results， areas in Fengcheng City， Xiuyan County， Zhuanghe City， Liaoyang County， Tieling County， Xifeng County， Gaizhou City， Haicheng City， and Dashiqiao City showed large potential distribution areas with high quality， making them highly promising for wild Panax ginseng cultivation. However， further experimental verification is required. The zoning results can provide insights for research on habitat suitability and comprehensive quality accumulation of wild Panax ginseng cultivated under forest， as well as guidance for the search for potential cultivation areas and industrial development of wild Panax ginseng in Liaoning Province.

Cancer is the leading cause of death worldwide. Drugs play a pivotal role in cancer treatment, but the complex biological processes of cancer cells seriously limit the efficacy of various anticancer drugs. Autophagy, a self-degradative system that maintains cellular homeostasis, universally operates under normal and stress conditions in cancer cells. The roles of autophagy in cancer treatment are still controversial because both stimulation and inhibition of autophagy have been reported to enhance the effects of anticancer drugs. Thus, the important question arises as to whether we should try to strengthen or suppress autophagy during cancer therapy. Currently, autophagy can be divided into four main forms according to its different functions during cancer treatment: cytoprotective (cell survival), cytotoxic (cell death), cytostatic (growth arrest), and nonprotective (no contribution to cell death or survival). In addition, various cell death modes, such as apoptosis, necrosis, ferroptosis, senescence, and mitotic catastrophe, all contribute to the anticancer effects of drugs. The interaction between autophagy and these cell death modes is complex and can lead to anticancer drugs having different or even completely opposite effects on treatment. Therefore, it is important to understand the underlying contexts in which autophagy inhibition or activation will be beneficial or detrimental. That is, appropriate therapeutic strategies should be adopted in light of the different functions of autophagy. This review provides an overview of recent insights into the evolving relationship between autophagy and cancer treatment.
Antineoplastic Agents/therapeutic use* ; Apoptosis ; Autophagy/physiology* ; Humans ; Necrosis/drug therapy* ; Neoplasms/therapy*

Objective:To summarize the clinical characteristics and the features of electroencephalograph (EEG) of children with DEPDC5 gene variants related epilepsy.Methods:The clinical data, gene variation, EEG and head magnetic resonance image (MRI) of 20 epileptic children with DEPDC5 gene variants admitted to Department of Pediatrics, Peking University First Hospital from May 2017 to November 2020 were retrospectively analyzed.Results:Twenty patients with heterozygous DEPDC5 gene variants were enrolled, 8 of 20 patients were nonsense variants, 6 were missense variants, 3 were frame-shift variants, 2 were splicing variants, and 1 was large fragment deletion. Sixteen cases had hereditary variation and 4 had de novo variation. Fifteen of variations were novel. Nine were male, while 11 were female. Their latest follow-up age ranged from 10 months to 13 years and one month.The epilepsy onset age ranged from 3 hours to 11 years and 3 months, the median age was 10.5 months. Twelve (60%) patients had developmental delay. Nineteen patients had focal seizures, 7 had epileptic spasms, 1 had multiple seizure types including tonic, atypical absence, dystonic and myoclonic seizures. Epileptic form discharges were observed in 18 patients during the interictal phase, and 11 were focal discharges, 7 were multifocal discharges. Ten (50%) patients had abnormal brain MRI, including focal cortical dysplasia in 5 patients, undefined malformation of cortical development in 4 patients, hemimegalencephaly in 1 patient. Four patients were diagnosed as West syndrome and one patient was diagnosed as Lennox-Gastaut syndrome. Fourteen (70%) patients were diagnosed as drug-resistant epilepsy. Four patients became seizure-free by treatment with anti-epileptic drugs. Three children were treated with surgery, and 2 of them became seizure-free, 1 had more than 75% reduction in seizures.Conclusions:DEPDC5 gene variant epilepsy is inherited with incomplete penetrance and focal seizure is the major seizure type. However, epileptic spasms, generalized seizures can also be observed. Half of the patients brain malformations. Most of the patients are drug-resistant epilepsy. Patients with clear epileptogenic zones can be treated with surgery. Treatment-resistant patients are more likely to be complicated with developmental delay.

Objective:To summarize the clinical phenotype and gene mutation characteristics of male patients with epilepsy caused by mosaic PCDH19 mutation. Methods:The clinical data of 3 male patients with epilepsy caused by mosaic PCDH19 mutation were analyzed.Microdroplet digital polymerase chain reaction (mDDPCR) was used for the detection of mosaicism in the three probands and their family members.Relevant literatures were reviewed. Results:The seizure onset age were 5 months, 9 months and 6 months of life respectively.Focal seizures occurred in 2 cases and multiple seizure types occurred in 1 case.Three patients presented with clusters of seizures.Fever sensitivity was observed in 2 cases out of the 3 cases.Two patients had intellectual disability and 1 patient had autistic manifestation.The clinical phenotype in 2 patient fulfilled the diagnosis of Dravet syndrome. PCDH19 mosaic mutations c. 317T>A(p.M106K), c.158dupT(p.D54Gfs*35) and c. 1639G>C(p.A547P) were detected respectively, and were identified as de novo after parental validation.Mutant allele fractions (MAF) in the blood samples were identified as 81.18%, 37.08%, 77.64%, respectively.The MAF of multiple tissues in 1 patient varied from 78.67% to 98.46%.Review of literature revealed that a total of 11 cases with mosaic PCDH19 mutation were reported.Among them, seizure onset occurred between 5 and 31 months of age.Focal seizures occurred in 9 cases, 3 cases of the 9 cases had only focal seizures.Generalized tonic clonic seizures occurred in 4 cases.Two or more seizures were observed in 6 cases.Clustering of seizures was found in all patient and sensitivity to fever was observed in 9 patients.Seven patients had mild to severe intellectual disability and 5 patients had autistic features. Conclusions:The clinical phenotypes of male patients with epilepsy caused by PCDH19 mosaic mutation are characterized by clustering of seizures, sensitivity to fever, focal seizures in most cases, varied degree of intellectual disability and autistic features in partial.

Post-traumatic tetanus is the main type of non-neonatal tetanus.To reduce the incidence and mortality rateof tetanus and guide the primary medical institutions to prevent and control tetanus after trauma,the National Immunization Planning Technical Working Group of the Chinese Center for Disease Control and Prevention has compiled this document in the reference with Position Paper by World Health Organization,and the latest research progress both at home and abroad.The guidelines focus on the basic procedures for the prevention and treatment of post-traumatic tetanus,the application of tetanus vaccines and immune preparation,and pre-exposure immunization in high-risk populations of trauma.