Background:Glioma-induced refractory epilepsy can be alleviated through conventional exercise,providing a potential therapeutic approach to manage this condition.This study aims to investigate the underlying mechanism...Background:Glioma-induced refractory epilepsy can be alleviated through conventional exercise,providing a potential therapeutic approach to manage this condition.This study aims to investigate the underlying mechanisms.Methods:Bioinformatics methodologies were employed to scrutinize gene expression data from public repositories such as GEO,with a specific focus on mobility-related genes in epilepsy.Through differential and enrichment analyses,differentially expressed genes(DEGs)were identified,while protein-protein interaction networks elucidated pivotal hub genes.Results:Our analysis revealed 32 DEGs,comprising 23 upregulated and 9 downregulated genes.Enrichment analysis underscored significant alterations in immune pathways in epilepsy.Two central hub genes,haptoglobin(HP)and prostaglandin-endoperoxide synthase 2(PTGS2),were found to be modulated by Arginase 1(ARG1)and Chemokine(C-X-C motif)ligand 8(CXCL8).GSVA analysis associated elevated PTGS2 expression with metabolic pathways,while increased HP expression was correlated with angiogenesis and inflammation.Subsequent experiments validated HP’s role in tumor cell proliferation,emphasizing its potential as a therapeutic target.Conclusion:This study highlights the crucial involvement of HP and PTGS2 genes in the etiology of epilepsy,linked to discrepancies in the immune system.These findings offer fresh perspectives on the management of epilepsy,emphasizing the neuroprotective possibilities of targeting specific gene pathway.展开更多
Background:Preeclampsia(PE),characterized by hypertension and proteinuria,leads to serious maternal and infant complications.Uridine-cytidine kinase 2(UCK2)belongs to the UCK family,a class of enzymes that catalyzes t...Background:Preeclampsia(PE),characterized by hypertension and proteinuria,leads to serious maternal and infant complications.Uridine-cytidine kinase 2(UCK2)belongs to the UCK family,a class of enzymes that catalyzes the conversion of uridine and cytidine to monophosphate form.However,the role of UCK2 in PE has not been reported.Methods:The expression of UCK2 was detected in the placenta of PE patients and N(ω)-nitro-L-arginine methyl esterinduced PE mouse model.Through forced up-regulation or down-regulation of UCK2 in vitro,we examined the effects of UCK2 on the proliferation,apoptosis,migration,and invasion of trophoblast cells.Stattic,the inhibitor of STAT3 pathway,was used to investigate whether the STAT3 pathway mediates the biological function of UCK2 in trophoblast cells.Results:The present study found that UCK2 showed low expression in the placenta of PE patients and PE mouse model.MTT(3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide)and flow cytometry assays verified that up-regulation of UCK2 promoted the proliferation of trophoblast cells,while the silence of UCK2 suppressed cell proliferation.Besides,flow cytometry and TdT-mediated dUTP Nick-End Labeling assays demonstrated that knockdown of UCK2 resulted in apoptosis of trophoblast cells.The wound healing and transwell assays showed that the migration and invasion activities of the trophoblast cells were facilitated by the overexpression of UCK2 and were blocked by the silence of UCK2.Furthermore,the expression of phosphorylated STAT3 was increased with the upregulation of UCK2 and decreased with the inhibition of UCK2.When the STAT3 pathway was blocked by its inhibitor stattic,the promotion effects of UCK2 on trophoblast cells were suppressed.Conclusion:UCK2 promotes the proliferation,migration,and invasion of trophoblast cells,and these effects may be partly mediated by the activation of the STAT3 pathway.展开更多
Background Mycoplasma pneumoniae(M.pneumoniae)is a significant contributor to community-acquired pneumonia among children.Since 1968,when a strain of M.pneumoniae resistant to macrolide antibiotics was initially repor...Background Mycoplasma pneumoniae(M.pneumoniae)is a significant contributor to community-acquired pneumonia among children.Since 1968,when a strain of M.pneumoniae resistant to macrolide antibiotics was initially reported in Japan,macrolide-resistant M.pneumoniae(MRMP)has been documented in many countries worldwide,with varying incidence rates.MRMP infections lead to a poor response to macrolide antibiotics,frequently resulting in prolonged fever,extended antibiotic treatment,increased hospitalization,intensive care unit admissions,and a significantly higher proportion of patients receiving glucocorticoids or second-line antibiotics.Since 2000,the global incidence of MRMP has gradually increased,especially in East Asia,which has posed a serious challenge to the treatment of M.pneumoniae infections in children and attracted widespread attention from pediatricians.However,there is still no global consensus on the diagnosis and treatment of MRMP in children.Methods We organized 29 Chinese experts majoring in pediatric pulmonology and epidemiology to write the world’s first consensus on the diagnosis and treatment of pediatric MRMP pneumonia,based on evidence collection.The evidence searches and reviews were conducted using electronic databases,including PubMed,Embase,Web of Science,CNKI,Medline,and the Cochrane Library.We used variations in terms for“macrolide-resistant”,“Mycoplasma pneumoniae”,“MP”,“M.pneumoniae”,“pneumonia”,“MRMP”,“lower respiratory tract infection”,“Mycoplasma pneumoniae infection”,“children”,and“pediatric”.Results Epidemiology,pathogenesis,clinical manifestations,early identification,laboratory examination,principles of antibiotic use,application of glucocorticoids and intravenous immunoglobulin,and precautions for bronchoscopy are highlighted.Early and rapid identification of gene mutations associated with MRMP is now available by polymerase chain reaction and fluorescent probe techniques in respiratory specimens.Although the resistance rate to macrolide remains high,it is fortunate that M.pneumoniae still maintains good in vitro sensitivity to second-line antibiotics such as tetracyclines and quinolones,making them an effective treatment option for patients with initial treatment failure caused by macrolide antibiotics.Conclusions This consensus,based on international and national scientific evidence,provides scientific guidance for the diagnosis and treatment of MRMP in children.Further studies on tetracycline and quinolone drugs in children are urgently needed to evaluate their effects on the growth and development.Additionally,developing an antibiotic rotation treatment strategy is necessary to reduce the prevalence of MRMP strains.展开更多
Our study was to investigate the epidemiological characteristics of M.tuberculosis from a national tuberculosis referral center in China. All strains isolated from TB patients, were genotyped by the RD105 deletion, 8 ...Our study was to investigate the epidemiological characteristics of M.tuberculosis from a national tuberculosis referral center in China. All strains isolated from TB patients, were genotyped by the RD105 deletion, 8 and 51 SNP loci and VNTR. The high differentiation SNPs of modern Beijing strains were analyzed for protein function and structure. 413 M. tuberculosis were included. Of 379 Beijing lineage M. tuberculosis, ＇modern＇ and ＇ancient＇ strains respectively represented 85.5% （324/379） and 14.5% （55/379）. Rv2494 （V48A） and Rv0245 （Sl03F） were confirmed as high differentiation SNPs associated with modern strains. In a word, Modern Beijing lineage M.tuberculosis was dominant and the structural models suggested that modern sub-lineage may more easily survive in ＇extreme＇ host condition.展开更多
The design of membrane pore is critical for membrane preparation. Polyvinylidene fluoride(PVDF) membrane exhibits outstanding properties in the water-treatment field. However, it is a huge challenge to prepare PVDF ma...The design of membrane pore is critical for membrane preparation. Polyvinylidene fluoride(PVDF) membrane exhibits outstanding properties in the water-treatment field. However, it is a huge challenge to prepare PVDF macro-pore plasma separation membrane by non-solvent induced phase separation(NIPS). Herein, a facile strategy is proposed to prepare PVDF macro-pore plasma separation membrane via macromolecular interaction. ATR-FTIR and ^(1)H NMR showed that the intermolecular interaction existed between polyethylene oxide(PEO) and polyvinylpyrrolidone(PVP). It could significantly affect the PVDF macro-pore membrane structure. The maximum pore of the PVDF membrane could be effectively adjusted from small-pore/medium-pore to macro-pore by changing the molecular weight of PEO. The PVDF macro-pore membrane was obtained successfully when PEO-200 k existed with PVP. It exhibited higher plasma separation properties than the currently used plasma separation membrane.Moreover, it had excellent hemocompatibility due to the similar plasma effect, hemolysis, prothrombin time, blood effect and complement C_(3a) effect with the current utilized plasma separation membrane,implying its great potential application. The proposed facile strategy in this work provides a new method to prepare PVDF macro-pore plasma separation membrane by NIPS.展开更多
BACKGROUND Pompe disease has a broad disease spectrum,including infantile-onset Pompe disease(IOPD)and late-onset Pompe disease(LOPD)forms.It is a type of glycogen storage disorder belonging to autosomal recessive gen...BACKGROUND Pompe disease has a broad disease spectrum,including infantile-onset Pompe disease(IOPD)and late-onset Pompe disease(LOPD)forms.It is a type of glycogen storage disorder belonging to autosomal recessive genetic disease,for an estimated incidence of 1/40000 among the neonatal population.In severe cases,the natural course is characterized by death due to cardiopulmonary failure in the first year after birth.However,the clinical outcomes have improved since the emergence of enzyme replacement therapy(ERT)was widely used.CASE SUMMARY The reported female case in China was an atypical IOPD,which demonstrates an unusual presentation of glycogen accumulation syndrome typeⅡwithout obvious skeletal muscle involvement,and reviewed physical examination,biochemical examinations,chest radiograph,and acidα-glucosidase(GAA)mutation analysis.After 4-mo specific ERT,the case received 12-mo follow-up.Moreover,the patient has obtained a very good prognosis under ERT.CONCLUSION For the atypical IOPD patients,early diagnosis and treatment may contribute to good prognosis.展开更多
Meningiomas occur in 1%-4% of primary intracranial tumors in the pediatric group, and is increasing in incidence with age. Some authors have reported that meningioma is more prevalent among adult males, but there is n...Meningiomas occur in 1%-4% of primary intracranial tumors in the pediatric group, and is increasing in incidence with age. Some authors have reported that meningioma is more prevalent among adult males, but there is no gender prevalence. The accepted origin of meningiomas is from the arachnoid ceils lining the meninges, or the choroid plexuses. Since Beckwith and Palmer introduced the term ＇rhabdoid tumor＇ in 1978 in reference to a subgroup of childhood malignant renal tumors, many tumors with a rhabdoid morphology have been reported in various sites, including the central nervous system. In 1998 Kepes et al.展开更多
Monkeypox is a zoonotic disease.Since the first human monkeypox case was detected in 1970,it has been prevalent in some countries in central and western Africa.Since May 2022,monkeypox cases have been reported in more...Monkeypox is a zoonotic disease.Since the first human monkeypox case was detected in 1970,it has been prevalent in some countries in central and western Africa.Since May 2022,monkeypox cases have been reported in more than 96 non-endemic countries and regions worldwide.As of September 14,2022,there have been more than 58,200 human monkeypox cases,and there is community transmission.The cessation of smallpox vaccination in 1980,which had some cross-protection with monkeypox,resulted in a general lack of immunity to monkeypox,which caused global concern and vigilance.As of Sep-tember 14,2022,there are four monkeypox cases in China,including three in Taiwan province and one in Hong Kong city.Previous foreign studies have shown that children are vulnerable to monkeypox and are also at high risk for severe disease or complications.In order to improve pediatricians'understanding of monkeypox and achieve early detection,early diagno-sis,early treatment,and early disposal,we have organized national authoritative experts in pediatric infection,respiratory,dermatology,critical care medicine,infectious diseases,and public health and others to formulate this expert consensus,on the basis of the latest"Clinical management and infection prevention and control for monkeypox"released by The World Health Organization,the"guidelines for diagnosis and treatment of monkeypox(version 2022)"issued by National Health Commission of the People's Republic of China and other relevant documents.During the development of this consensus,multidisciplinary experts have repeatedly demonstrated the etiology,epidemiology,transmission,clinical manifestations,laboratory examinations,diagnosis,differential diagnosis,treatment,discharge criteria,prevention,disposal process,and key points of prevention and control of suspected and confirmed cases.展开更多
Background Children as a population have high antimicrobial prescribing rates which may lead to high resistance of bacteria according to data from some single-center surveys of antibiotic prescribing rates in China. T...Background Children as a population have high antimicrobial prescribing rates which may lead to high resistance of bacteria according to data from some single-center surveys of antibiotic prescribing rates in China. The acquirement of baseline data of antibiotic prescribing is the basis of developing intervention strategies on inappropriate antimicrobial prescriptions. Few studies show clearly the pattern and detailed information on classes of antibiotics and distribution of indications of antibiotic prescriptions in children in China. This study aims to assess the antibiotic prescribing patterns among children and neonates hospitalized in 18 hospitals in China. Methods A 24-hour point prevalence survey on antimicrobial prescribing was conducted in hospitalized neonates and chil-dren in China from December 1st, 2016 to February 28th, 2017. Information on the antibiotic use of patients under 18 years of age who were administered one or more on-going antibiotics in the selected wards over a 24-hour period was collected. These data were submitted to the GARPEC (Global Antimicrobial Resistance, Prescribing and Efficacy in Children and Neonates) web-based application ( https ://pidrg -datab ase.sgul.ac.uk/redca p/ ). For statistical analysis, Microsoft Excel 2007 and SPSS 22.0 were used. Results The antibiotic data were collected in 35 wards in 18 hospitals from 9 provinces. In total, 67.76% (975/1439) of the patients (n = 1439) were given at least one antibiotic, including 58.1% (173/298) of neonates (n = 298) and 70.3% (802/1141) of children (n = 1141). In neonates, the three most frequently prescribed antibiotics were third-generation cephalosporins (41.7%), penicillins plus enzyme inhibitor (23.8%), and carbapenems (11.2%). In children, the three most frequently pre-scribed antibiotics were third-generation cephalosporins (35.5%), macrolides (23.2%), and penicillins plus enzyme inhibi-tors (15.9%). The most common indication for antibiotics was proven or probable bacterial lower respiratory tract infection (30.9% in neonates and 66.6% in children). Conclusions Antibiotics are commonly prescribed in the Chinese children population. It is likely that the third-generation cephalosporins and macrolides are currently overused in Chinese children. Efforts must be made to ensure safe and appropri-ate antibiotic prescribing to reduce and prevent the future development of antibiotic resistance.展开更多
To gain a broader appreciation of the clinical presentation,operative treatment,and outcome offibrous dysplasia involving the calvarium in children,we retro-spectively reviewed a series of cases offibrous dysplasia invo...To gain a broader appreciation of the clinical presentation,operative treatment,and outcome offibrous dysplasia involving the calvarium in children,we retro-spectively reviewed a series of cases offibrous dysplasia involving the calvarium(4 males and 2 females)with patients’age ranging from 5 to 12 years old.The clinical manifestation,radiographicfindings,surgical treatment,outcome and follow-up were evaluated on the basis of medical records.Fibrous dysplasia in the series was monostotic,involving frontal bone(2 cases),temporal bone(1 case),parietal bone(2 cases)and occipital bone(1 case).The patients most commonly presented with enlarging mass and cosmetic complaints.The treatment given,depending on clinical presentation,was simple biopsy with conservative follow-up(2 cases)to cranial resection(4 cases).All the cases were histopathologically confirmed asfibrous dysplasia.It was demonstrated thatfibrous dysplasia involving the calvarium is a typically benign but slowly progressive disorder of bone.Modern imaging modalities and histopathologic analysis have made diagnosis relatively straightforward.Surgery should be reserved for patients with functional impairment or cosmetic deformity.Because of the benign nature of the condition,the surgery itself should be contemplated with great caution in children.展开更多
Derived from neural stem cells(NSCs)and progenitor cells originated from the neuroectoderm,the nervous system presents an unprecedented degree of cellular diversity,interwoven to ensure correct connections for propaga...Derived from neural stem cells(NSCs)and progenitor cells originated from the neuroectoderm,the nervous system presents an unprecedented degree of cellular diversity,interwoven to ensure correct connections for propagating information and responding to environ-mental cues.NSCs and progenitor cells must integrate cell-intrinsic programs and environmental cues to achieve production of appropriate types of neurons and glia at appropriate times and places during develop-ment.These developmental dynamics are reflected in changes in gene expression,which is regulated by transcription factors and at the epigenetic level.From early commitment of neural lineage to functional plas-ticity in terminal differentiated neurons,epigenetic regulation is involved in every step of neural develop-ment.Here we focus on the recent advance in our un-derstanding of epigenetic regulation on orderly genera-tion of diverse neural cell types in the mammalian nervous system,an important aspect of neural devel-opment and regenerative medicine.展开更多
Background This study aimed to assess efficacy and safety of oxcarbazepine (OXC) oral suspension in pediatric patients aged 2-5 years with partial seizures (PS) and/or generalized tonic-clonic seizures (GTCS) in real-...Background This study aimed to assess efficacy and safety of oxcarbazepine (OXC) oral suspension in pediatric patients aged 2-5 years with partial seizures (PS) and/or generalized tonic-clonic seizures (GTCS) in real-world clinical practice in China. Methods This 26-week, prospective, single-arm, multicenter, observational study recruited pediatric patients aged 2-5 years with PS or GTCS suitable for OXC oral suspension treatment based on physicians' judgments from 11 medical centers in China. Enrolled subjects started OXC oral suspension treatment as monotherapy or in combination with other antiepileptic drugs. Primary efficacy outcome was the percentage of pediatric subjects achieving ≥ 50% seizure frequency reduction at the end of the 26-week treatment. Secondary efficacy-related parameters and safety parameters such as adverse events (AEs) and serious AEs (SAEs) were also monitored during the 26-week treatment period. Results Six hundred and six pediatric patients were enrolled and 531 (87.6%) completed the study. After 26 weeks of treat-ment, 93.3% subjects achieved ≥ 50% seizure frequency reduction, and 81.8% achieved 100% seizure frequency reduction compared to baseline. Among diff erent seizure types, OXC was eff ective in all subjects with simple PS and in > 90% of subject with other type of seizure present in the study. AEs were observed in 49 (8.1%) subjects. Only three subjects expe-rienced SAE. Rash (n = 18, 2.97%) was the most common AE. Only 17 subjects discontinued due to AEs. Conclusion This study, reporting the real-world data, further confi rms the efficacy and good safety profi le of OXC oral suspension in Chinese pediatric patients aged 2-5 years with PS and/or GTCS.展开更多
Vein of Galen malformations(VGMs)are rare,consisting approximately 1%of intracranial vascular mal-formations,but nearly one third of pediatric vascular mal-formations,which can cause severe morbidity and mortality in ...Vein of Galen malformations(VGMs)are rare,consisting approximately 1%of intracranial vascular mal-formations,but nearly one third of pediatric vascular mal-formations,which can cause severe morbidity and mortality in infants and older children,but more particu-larly in neonates.We presented 2 patients with VGMs which were confirmed by characteristic clinical symptoms and neuroradiological findings(including CT,MRI and/or angiography).One patient underwent embolization fol-lowed by CT,MRI and conventional angiography.The patient’s neurological and cognitive status was normal at present.The other neonate died of congestive heart failure 36 h after birth.展开更多
Though rare,angiomyolipomas(AMLs)are the most common mesenchymal tumors of kidney.In general,AMLs can always be associated with two conditions affecting other organ systems:tuberous sclero-sis complex(TSC)and sporadic...Though rare,angiomyolipomas(AMLs)are the most common mesenchymal tumors of kidney.In general,AMLs can always be associated with two conditions affecting other organ systems:tuberous sclero-sis complex(TSC)and sporadic lymphangioleiomyoma-tosis.This article presents a case of renal AML occurring in a 14-year-old girl with a definite diagnosis of TSC.She had been diagnosed with TSC at the age of three,and a schedule for close observation was disobeyed.At this time,she underwent a series of examinations:physical examina-tion,ultrasonography,angiography,computed tomography(CT),and magnetic resonance imaging(MRI)scans.The physical examination showed adenoma sebaceum in a butterfly paranasal distribution,and a mass was palpated in the left upper quadrant.There were no neurological deficits.Imaging studies(including ultrasonography,angiography,CT,and MRI)of the abdomen showed a large heterogeneous mass arising from the left kidney.Partial nephrectomy was performed.The pathological diagnosis was hemorrhagic renal AML.No recurrence was found in the three-year follow-up.We concluded that schedule of close observation on patients with TSC should be strictly abided by for the high morbidity of AMLs.The specific risks of renal AMLs are spontaneous hemorrhage and rupture.Treatment options for AMLs include conservative and interventional(total/partial nephrectomy,cryoptherapy,and embolization)treatments.展开更多
The spinal epidural space is an uncommon presenting site in primary non-Hodgkin’s lymphomas,especially for children.A boy suffered spinal cord compression syndrome caused by primary spinal epidural non-Hodgkin’s lym...The spinal epidural space is an uncommon presenting site in primary non-Hodgkin’s lymphomas,especially for children.A boy suffered spinal cord compression syndrome caused by primary spinal epidural non-Hodgkin’s lymphoma.Thoracolumbar magnetic reso-nance imaging(MRI)demonstrated an intraspinal mass.An operation was performed with gross total tumor removal.Histological examination revealed a non-Hodgkin’s B-cell lymphoma.Bone marrow aspiration was negative for lymphoma involvement.No other therapies(chemotherapy and/or radiotherapy)were per-formed according to the parents’opinion.The patient died approximately one year after the operation due to brain metastases.The clinical course and imaging features were discussed with a review of literatures.展开更多
background Biliary atresia(BA)is one of the most challenging hepatobiliary diseases in children.Notwithstanding the reasonable outcome of liver transplantation(LT),portoenteral anastomosis(Kasai operation)is still the...background Biliary atresia(BA)is one of the most challenging hepatobiliary diseases in children.Notwithstanding the reasonable outcome of liver transplantation(LT),portoenteral anastomosis(Kasai operation)is still the first choice of treatment for patients with BA.How to improve the survival rate of the patients with BA in the era of LT is a new challenge.Data sources Based on recent original publications and the experience with the BA in China,we review many factors that influence BA survival situation,including early diagnosis and screen plan,defects of early treatment,Kasai operation,and LT and indicate present questions about BA diagnosis and treatment in China.results BA diagnosis may also be delayed due to insufficient understanding of BA and lack of jaundice monitoring methods at different levels of the hospitals in China.Further education of the physicians at smaller city hospitals about BA,neonatal jaundice and cholestasis would be helpful in improving early diagnosis of BA.Early surgical intervention is still the only guarantee to improve the survival rate of BA with native liver.Conclusions In the era of LT,especially in China,Kasai operation can provide waiting time for living donor LT and improve the success rate of LT and minimize the rapid deterioration of liver function of the children with BA and decrease the mortality in patients with BA.展开更多
Tianjin Children’s Hospital,in North China、Tianjin Municipality,has 141 years of history.The hospitals director,Liu Wei,is known for her dedication to research into rare diseases,her humanistic approach to care,and ...Tianjin Children’s Hospital,in North China、Tianjin Municipality,has 141 years of history.The hospitals director,Liu Wei,is known for her dedication to research into rare diseases,her humanistic approach to care,and her ability to manage a children^hospital with a long history.In addition to concentrating on her work,Liu likes to draw,paint,read,write and listen to music.She has written several novels,which have won national-level awards.展开更多
基金supported by the Ningxia Natural Science Foundation(Grant No.2022AAC03741)the Ningxia Medical University Scientific Research Fund(Grant No.XZ2021025).
文摘Background:Glioma-induced refractory epilepsy can be alleviated through conventional exercise,providing a potential therapeutic approach to manage this condition.This study aims to investigate the underlying mechanisms.Methods:Bioinformatics methodologies were employed to scrutinize gene expression data from public repositories such as GEO,with a specific focus on mobility-related genes in epilepsy.Through differential and enrichment analyses,differentially expressed genes(DEGs)were identified,while protein-protein interaction networks elucidated pivotal hub genes.Results:Our analysis revealed 32 DEGs,comprising 23 upregulated and 9 downregulated genes.Enrichment analysis underscored significant alterations in immune pathways in epilepsy.Two central hub genes,haptoglobin(HP)and prostaglandin-endoperoxide synthase 2(PTGS2),were found to be modulated by Arginase 1(ARG1)and Chemokine(C-X-C motif)ligand 8(CXCL8).GSVA analysis associated elevated PTGS2 expression with metabolic pathways,while increased HP expression was correlated with angiogenesis and inflammation.Subsequent experiments validated HP’s role in tumor cell proliferation,emphasizing its potential as a therapeutic target.Conclusion:This study highlights the crucial involvement of HP and PTGS2 genes in the etiology of epilepsy,linked to discrepancies in the immune system.These findings offer fresh perspectives on the management of epilepsy,emphasizing the neuroprotective possibilities of targeting specific gene pathway.
基金supported by“Heart Health Research Fund,”Special Fund for Cardiovascular Disease Development of China,China International Medical Foundation(Grant No.Z-2019-42-1908-3),Tianjin Key Medical Discipline(Specialty)Construction Project,and the Young Talents Training Program of Tianjin First Central Hospital.
文摘Background:Preeclampsia(PE),characterized by hypertension and proteinuria,leads to serious maternal and infant complications.Uridine-cytidine kinase 2(UCK2)belongs to the UCK family,a class of enzymes that catalyzes the conversion of uridine and cytidine to monophosphate form.However,the role of UCK2 in PE has not been reported.Methods:The expression of UCK2 was detected in the placenta of PE patients and N(ω)-nitro-L-arginine methyl esterinduced PE mouse model.Through forced up-regulation or down-regulation of UCK2 in vitro,we examined the effects of UCK2 on the proliferation,apoptosis,migration,and invasion of trophoblast cells.Stattic,the inhibitor of STAT3 pathway,was used to investigate whether the STAT3 pathway mediates the biological function of UCK2 in trophoblast cells.Results:The present study found that UCK2 showed low expression in the placenta of PE patients and PE mouse model.MTT(3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide)and flow cytometry assays verified that up-regulation of UCK2 promoted the proliferation of trophoblast cells,while the silence of UCK2 suppressed cell proliferation.Besides,flow cytometry and TdT-mediated dUTP Nick-End Labeling assays demonstrated that knockdown of UCK2 resulted in apoptosis of trophoblast cells.The wound healing and transwell assays showed that the migration and invasion activities of the trophoblast cells were facilitated by the overexpression of UCK2 and were blocked by the silence of UCK2.Furthermore,the expression of phosphorylated STAT3 was increased with the upregulation of UCK2 and decreased with the inhibition of UCK2.When the STAT3 pathway was blocked by its inhibitor stattic,the promotion effects of UCK2 on trophoblast cells were suppressed.Conclusion:UCK2 promotes the proliferation,migration,and invasion of trophoblast cells,and these effects may be partly mediated by the activation of the STAT3 pathway.
基金supported by the grants from Key R&D Projects of Zhejiang Province(2023C03009 and 2024C03177).
文摘Background Mycoplasma pneumoniae(M.pneumoniae)is a significant contributor to community-acquired pneumonia among children.Since 1968,when a strain of M.pneumoniae resistant to macrolide antibiotics was initially reported in Japan,macrolide-resistant M.pneumoniae(MRMP)has been documented in many countries worldwide,with varying incidence rates.MRMP infections lead to a poor response to macrolide antibiotics,frequently resulting in prolonged fever,extended antibiotic treatment,increased hospitalization,intensive care unit admissions,and a significantly higher proportion of patients receiving glucocorticoids or second-line antibiotics.Since 2000,the global incidence of MRMP has gradually increased,especially in East Asia,which has posed a serious challenge to the treatment of M.pneumoniae infections in children and attracted widespread attention from pediatricians.However,there is still no global consensus on the diagnosis and treatment of MRMP in children.Methods We organized 29 Chinese experts majoring in pediatric pulmonology and epidemiology to write the world’s first consensus on the diagnosis and treatment of pediatric MRMP pneumonia,based on evidence collection.The evidence searches and reviews were conducted using electronic databases,including PubMed,Embase,Web of Science,CNKI,Medline,and the Cochrane Library.We used variations in terms for“macrolide-resistant”,“Mycoplasma pneumoniae”,“MP”,“M.pneumoniae”,“pneumonia”,“MRMP”,“lower respiratory tract infection”,“Mycoplasma pneumoniae infection”,“children”,and“pediatric”.Results Epidemiology,pathogenesis,clinical manifestations,early identification,laboratory examination,principles of antibiotic use,application of glucocorticoids and intravenous immunoglobulin,and precautions for bronchoscopy are highlighted.Early and rapid identification of gene mutations associated with MRMP is now available by polymerase chain reaction and fluorescent probe techniques in respiratory specimens.Although the resistance rate to macrolide remains high,it is fortunate that M.pneumoniae still maintains good in vitro sensitivity to second-line antibiotics such as tetracyclines and quinolones,making them an effective treatment option for patients with initial treatment failure caused by macrolide antibiotics.Conclusions This consensus,based on international and national scientific evidence,provides scientific guidance for the diagnosis and treatment of MRMP in children.Further studies on tetracycline and quinolone drugs in children are urgently needed to evaluate their effects on the growth and development.Additionally,developing an antibiotic rotation treatment strategy is necessary to reduce the prevalence of MRMP strains.
基金supported by the National Natural Science Foundation of China(No.81273144)Beijing Natural Science Foundation Program and Scientific Research Key Program of Beijing Municipal Commission of Education(KZ201510025024)Beijing Municipal Administration of Hospitals Clinical Medicine Development of Special Funding Support(ZYLX201304)
文摘Our study was to investigate the epidemiological characteristics of M.tuberculosis from a national tuberculosis referral center in China. All strains isolated from TB patients, were genotyped by the RD105 deletion, 8 and 51 SNP loci and VNTR. The high differentiation SNPs of modern Beijing strains were analyzed for protein function and structure. 413 M. tuberculosis were included. Of 379 Beijing lineage M. tuberculosis, ＇modern＇ and ＇ancient＇ strains respectively represented 85.5% （324/379） and 14.5% （55/379）. Rv2494 （V48A） and Rv0245 （Sl03F） were confirmed as high differentiation SNPs associated with modern strains. In a word, Modern Beijing lineage M.tuberculosis was dominant and the structural models suggested that modern sub-lineage may more easily survive in ＇extreme＇ host condition.
基金the National Natural Science Foundation of China (21776216)Tianjin Key Laboratory Project (16PTSYJC00210)+3 种基金Program for Innovative Research Team in University of Tianjin (TD13-5044)Science and technology support project of Tianjin (20YFZCSY00310, 21ZXGWSY00040)State Key Laboratory of Separation Membranes and Membrane Processes (Tiangong University), Youth Science Foundation of Tianjin (21JCQNJC00100)Tianjin Health Science and Technology Project (TJWJ2021MS014)。
文摘The design of membrane pore is critical for membrane preparation. Polyvinylidene fluoride(PVDF) membrane exhibits outstanding properties in the water-treatment field. However, it is a huge challenge to prepare PVDF macro-pore plasma separation membrane by non-solvent induced phase separation(NIPS). Herein, a facile strategy is proposed to prepare PVDF macro-pore plasma separation membrane via macromolecular interaction. ATR-FTIR and ^(1)H NMR showed that the intermolecular interaction existed between polyethylene oxide(PEO) and polyvinylpyrrolidone(PVP). It could significantly affect the PVDF macro-pore membrane structure. The maximum pore of the PVDF membrane could be effectively adjusted from small-pore/medium-pore to macro-pore by changing the molecular weight of PEO. The PVDF macro-pore membrane was obtained successfully when PEO-200 k existed with PVP. It exhibited higher plasma separation properties than the currently used plasma separation membrane.Moreover, it had excellent hemocompatibility due to the similar plasma effect, hemolysis, prothrombin time, blood effect and complement C_(3a) effect with the current utilized plasma separation membrane,implying its great potential application. The proposed facile strategy in this work provides a new method to prepare PVDF macro-pore plasma separation membrane by NIPS.
基金Supported by Tianjin Municipal Health Commission,China,No. ZC20060
文摘BACKGROUND Pompe disease has a broad disease spectrum,including infantile-onset Pompe disease(IOPD)and late-onset Pompe disease(LOPD)forms.It is a type of glycogen storage disorder belonging to autosomal recessive genetic disease,for an estimated incidence of 1/40000 among the neonatal population.In severe cases,the natural course is characterized by death due to cardiopulmonary failure in the first year after birth.However,the clinical outcomes have improved since the emergence of enzyme replacement therapy(ERT)was widely used.CASE SUMMARY The reported female case in China was an atypical IOPD,which demonstrates an unusual presentation of glycogen accumulation syndrome typeⅡwithout obvious skeletal muscle involvement,and reviewed physical examination,biochemical examinations,chest radiograph,and acidα-glucosidase(GAA)mutation analysis.After 4-mo specific ERT,the case received 12-mo follow-up.Moreover,the patient has obtained a very good prognosis under ERT.CONCLUSION For the atypical IOPD patients,early diagnosis and treatment may contribute to good prognosis.
文摘Meningiomas occur in 1%-4% of primary intracranial tumors in the pediatric group, and is increasing in incidence with age. Some authors have reported that meningioma is more prevalent among adult males, but there is no gender prevalence. The accepted origin of meningiomas is from the arachnoid ceils lining the meninges, or the choroid plexuses. Since Beckwith and Palmer introduced the term ＇rhabdoid tumor＇ in 1978 in reference to a subgroup of childhood malignant renal tumors, many tumors with a rhabdoid morphology have been reported in various sites, including the central nervous system. In 1998 Kepes et al.
基金National Natural Science Foundation of China(72174138)High-level Public health Talents Training Program of Beijing Municipal Health Commission(2022-2-002).
文摘Monkeypox is a zoonotic disease.Since the first human monkeypox case was detected in 1970,it has been prevalent in some countries in central and western Africa.Since May 2022,monkeypox cases have been reported in more than 96 non-endemic countries and regions worldwide.As of September 14,2022,there have been more than 58,200 human monkeypox cases,and there is community transmission.The cessation of smallpox vaccination in 1980,which had some cross-protection with monkeypox,resulted in a general lack of immunity to monkeypox,which caused global concern and vigilance.As of Sep-tember 14,2022,there are four monkeypox cases in China,including three in Taiwan province and one in Hong Kong city.Previous foreign studies have shown that children are vulnerable to monkeypox and are also at high risk for severe disease or complications.In order to improve pediatricians'understanding of monkeypox and achieve early detection,early diagno-sis,early treatment,and early disposal,we have organized national authoritative experts in pediatric infection,respiratory,dermatology,critical care medicine,infectious diseases,and public health and others to formulate this expert consensus,on the basis of the latest"Clinical management and infection prevention and control for monkeypox"released by The World Health Organization,the"guidelines for diagnosis and treatment of monkeypox(version 2022)"issued by National Health Commission of the People's Republic of China and other relevant documents.During the development of this consensus,multidisciplinary experts have repeatedly demonstrated the etiology,epidemiology,transmission,clinical manifestations,laboratory examinations,diagnosis,differential diagnosis,treatment,discharge criteria,prevention,disposal process,and key points of prevention and control of suspected and confirmed cases.
文摘Background Children as a population have high antimicrobial prescribing rates which may lead to high resistance of bacteria according to data from some single-center surveys of antibiotic prescribing rates in China. The acquirement of baseline data of antibiotic prescribing is the basis of developing intervention strategies on inappropriate antimicrobial prescriptions. Few studies show clearly the pattern and detailed information on classes of antibiotics and distribution of indications of antibiotic prescriptions in children in China. This study aims to assess the antibiotic prescribing patterns among children and neonates hospitalized in 18 hospitals in China. Methods A 24-hour point prevalence survey on antimicrobial prescribing was conducted in hospitalized neonates and chil-dren in China from December 1st, 2016 to February 28th, 2017. Information on the antibiotic use of patients under 18 years of age who were administered one or more on-going antibiotics in the selected wards over a 24-hour period was collected. These data were submitted to the GARPEC (Global Antimicrobial Resistance, Prescribing and Efficacy in Children and Neonates) web-based application ( https ://pidrg -datab ase.sgul.ac.uk/redca p/ ). For statistical analysis, Microsoft Excel 2007 and SPSS 22.0 were used. Results The antibiotic data were collected in 35 wards in 18 hospitals from 9 provinces. In total, 67.76% (975/1439) of the patients (n = 1439) were given at least one antibiotic, including 58.1% (173/298) of neonates (n = 298) and 70.3% (802/1141) of children (n = 1141). In neonates, the three most frequently prescribed antibiotics were third-generation cephalosporins (41.7%), penicillins plus enzyme inhibitor (23.8%), and carbapenems (11.2%). In children, the three most frequently pre-scribed antibiotics were third-generation cephalosporins (35.5%), macrolides (23.2%), and penicillins plus enzyme inhibi-tors (15.9%). The most common indication for antibiotics was proven or probable bacterial lower respiratory tract infection (30.9% in neonates and 66.6% in children). Conclusions Antibiotics are commonly prescribed in the Chinese children population. It is likely that the third-generation cephalosporins and macrolides are currently overused in Chinese children. Efforts must be made to ensure safe and appropri-ate antibiotic prescribing to reduce and prevent the future development of antibiotic resistance.
文摘To gain a broader appreciation of the clinical presentation,operative treatment,and outcome offibrous dysplasia involving the calvarium in children,we retro-spectively reviewed a series of cases offibrous dysplasia involving the calvarium(4 males and 2 females)with patients’age ranging from 5 to 12 years old.The clinical manifestation,radiographicfindings,surgical treatment,outcome and follow-up were evaluated on the basis of medical records.Fibrous dysplasia in the series was monostotic,involving frontal bone(2 cases),temporal bone(1 case),parietal bone(2 cases)and occipital bone(1 case).The patients most commonly presented with enlarging mass and cosmetic complaints.The treatment given,depending on clinical presentation,was simple biopsy with conservative follow-up(2 cases)to cranial resection(4 cases).All the cases were histopathologically confirmed asfibrous dysplasia.It was demonstrated thatfibrous dysplasia involving the calvarium is a typically benign but slowly progressive disorder of bone.Modern imaging modalities and histopathologic analysis have made diagnosis relatively straightforward.Surgery should be reserved for patients with functional impairment or cosmetic deformity.Because of the benign nature of the condition,the surgery itself should be contemplated with great caution in children.
基金supported by grants from the National Natural Science Foundation of China(Grant No.2012CB966701)the Ministry of Science and Technology of the People’s Republic of China.
文摘Derived from neural stem cells(NSCs)and progenitor cells originated from the neuroectoderm,the nervous system presents an unprecedented degree of cellular diversity,interwoven to ensure correct connections for propagating information and responding to environ-mental cues.NSCs and progenitor cells must integrate cell-intrinsic programs and environmental cues to achieve production of appropriate types of neurons and glia at appropriate times and places during develop-ment.These developmental dynamics are reflected in changes in gene expression,which is regulated by transcription factors and at the epigenetic level.From early commitment of neural lineage to functional plas-ticity in terminal differentiated neurons,epigenetic regulation is involved in every step of neural develop-ment.Here we focus on the recent advance in our un-derstanding of epigenetic regulation on orderly genera-tion of diverse neural cell types in the mammalian nervous system,an important aspect of neural devel-opment and regenerative medicine.
文摘Background This study aimed to assess efficacy and safety of oxcarbazepine (OXC) oral suspension in pediatric patients aged 2-5 years with partial seizures (PS) and/or generalized tonic-clonic seizures (GTCS) in real-world clinical practice in China. Methods This 26-week, prospective, single-arm, multicenter, observational study recruited pediatric patients aged 2-5 years with PS or GTCS suitable for OXC oral suspension treatment based on physicians' judgments from 11 medical centers in China. Enrolled subjects started OXC oral suspension treatment as monotherapy or in combination with other antiepileptic drugs. Primary efficacy outcome was the percentage of pediatric subjects achieving ≥ 50% seizure frequency reduction at the end of the 26-week treatment. Secondary efficacy-related parameters and safety parameters such as adverse events (AEs) and serious AEs (SAEs) were also monitored during the 26-week treatment period. Results Six hundred and six pediatric patients were enrolled and 531 (87.6%) completed the study. After 26 weeks of treat-ment, 93.3% subjects achieved ≥ 50% seizure frequency reduction, and 81.8% achieved 100% seizure frequency reduction compared to baseline. Among diff erent seizure types, OXC was eff ective in all subjects with simple PS and in > 90% of subject with other type of seizure present in the study. AEs were observed in 49 (8.1%) subjects. Only three subjects expe-rienced SAE. Rash (n = 18, 2.97%) was the most common AE. Only 17 subjects discontinued due to AEs. Conclusion This study, reporting the real-world data, further confi rms the efficacy and good safety profi le of OXC oral suspension in Chinese pediatric patients aged 2-5 years with PS and/or GTCS.
文摘Vein of Galen malformations(VGMs)are rare,consisting approximately 1%of intracranial vascular mal-formations,but nearly one third of pediatric vascular mal-formations,which can cause severe morbidity and mortality in infants and older children,but more particu-larly in neonates.We presented 2 patients with VGMs which were confirmed by characteristic clinical symptoms and neuroradiological findings(including CT,MRI and/or angiography).One patient underwent embolization fol-lowed by CT,MRI and conventional angiography.The patient’s neurological and cognitive status was normal at present.The other neonate died of congestive heart failure 36 h after birth.
文摘Though rare,angiomyolipomas(AMLs)are the most common mesenchymal tumors of kidney.In general,AMLs can always be associated with two conditions affecting other organ systems:tuberous sclero-sis complex(TSC)and sporadic lymphangioleiomyoma-tosis.This article presents a case of renal AML occurring in a 14-year-old girl with a definite diagnosis of TSC.She had been diagnosed with TSC at the age of three,and a schedule for close observation was disobeyed.At this time,she underwent a series of examinations:physical examina-tion,ultrasonography,angiography,computed tomography(CT),and magnetic resonance imaging(MRI)scans.The physical examination showed adenoma sebaceum in a butterfly paranasal distribution,and a mass was palpated in the left upper quadrant.There were no neurological deficits.Imaging studies(including ultrasonography,angiography,CT,and MRI)of the abdomen showed a large heterogeneous mass arising from the left kidney.Partial nephrectomy was performed.The pathological diagnosis was hemorrhagic renal AML.No recurrence was found in the three-year follow-up.We concluded that schedule of close observation on patients with TSC should be strictly abided by for the high morbidity of AMLs.The specific risks of renal AMLs are spontaneous hemorrhage and rupture.Treatment options for AMLs include conservative and interventional(total/partial nephrectomy,cryoptherapy,and embolization)treatments.
文摘The spinal epidural space is an uncommon presenting site in primary non-Hodgkin’s lymphomas,especially for children.A boy suffered spinal cord compression syndrome caused by primary spinal epidural non-Hodgkin’s lymphoma.Thoracolumbar magnetic reso-nance imaging(MRI)demonstrated an intraspinal mass.An operation was performed with gross total tumor removal.Histological examination revealed a non-Hodgkin’s B-cell lymphoma.Bone marrow aspiration was negative for lymphoma involvement.No other therapies(chemotherapy and/or radiotherapy)were per-formed according to the parents’opinion.The patient died approximately one year after the operation due to brain metastases.The clinical course and imaging features were discussed with a review of literatures.
基金This study was supported by National Natural Fund:81570471.Tianjin Institutes of Health grants:14KG129
文摘background Biliary atresia(BA)is one of the most challenging hepatobiliary diseases in children.Notwithstanding the reasonable outcome of liver transplantation(LT),portoenteral anastomosis(Kasai operation)is still the first choice of treatment for patients with BA.How to improve the survival rate of the patients with BA in the era of LT is a new challenge.Data sources Based on recent original publications and the experience with the BA in China,we review many factors that influence BA survival situation,including early diagnosis and screen plan,defects of early treatment,Kasai operation,and LT and indicate present questions about BA diagnosis and treatment in China.results BA diagnosis may also be delayed due to insufficient understanding of BA and lack of jaundice monitoring methods at different levels of the hospitals in China.Further education of the physicians at smaller city hospitals about BA,neonatal jaundice and cholestasis would be helpful in improving early diagnosis of BA.Early surgical intervention is still the only guarantee to improve the survival rate of BA with native liver.Conclusions In the era of LT,especially in China,Kasai operation can provide waiting time for living donor LT and improve the success rate of LT and minimize the rapid deterioration of liver function of the children with BA and decrease the mortality in patients with BA.
文摘Tianjin Children’s Hospital,in North China、Tianjin Municipality,has 141 years of history.The hospitals director,Liu Wei,is known for her dedication to research into rare diseases,her humanistic approach to care,and her ability to manage a children^hospital with a long history.In addition to concentrating on her work,Liu likes to draw,paint,read,write and listen to music.She has written several novels,which have won national-level awards.
