Objective This study aimed to investigate the antimicrobial resistance profiles of bacterial strains isolated from pediatric intensive care units(PICU)in China for better antimicrobial therapy.Methods Clinical isolates were collected from 17 institutions,including tertiary care children's hospitals and pediatric department of tertiary general hospitals in China from January 1,2020 to December 31,2022.Antimicrobial susceptibility testing was carried out according to a unified protocol using Kirby-Bauer method or automated systems.Results were interpreted according to the breakpoints released by the Clinical and Laboratory Standards Institute(CLSI)in 2020.Results A total of 10 688 isolates were collected,including gram-positive organisms(39.2％)and gram-negative organisms(60.8％).The top three organisms were S.aureus(13.6％,1 453/10 688),A.baumannii(10.0％,1 067/10 688),and coagulase-negative Staphylococcus(9.9％,1 058/10 688).Multi-drug resistant organisms(MDROs)were very common in children.The prevalence of methicillin-resistant Staphylococcus aureus(MRSA),carbapenem-resistant Enterobacterales(CRE),carbapenem-resistant E.coli,carbapenem-resistant K.pneumoniae(CRKP),carbapenem-resistant A.baumannii(CRAB),and carbapenem-resistant P.aeruginosa(CRPA)was 41.1％,19.4％,8.8％,30.9％,67.4％,and 28.8％,respectively.Overall,more than 50％of Enterobacteriales isolates were resistant to cephalosporins,while nearly 25％of Enterobacteriales isolates were resistant to carbapenems.MDROs were highly resistant to commonly used antibiotics.More than 80％of CRE and CRAB strains were resistant to all beta-lactam antibiotics.CRE and CRAB showed low resistance rates to tigecycline and polymyxin.CRPA showed lower resistance rates to piperacillin,beta-lactamase inhibitor combinations than the resistance rates to third and fourth generation cephalosporins.All of the Staphylococcus and Enterococcus isolates were susceptible to vancomycin and tigecycline.None of PRSP strains isolated from meningitis and nonmeningitis samples were resistant to rifampicin,vancomycin,or linezolid.The prevalence of β-lactamase-negative ampicillin-resistant(BLNAR)strains was 43.3％in Haemophilus influenzae.Conclusions MDROs were prevalent in PICU.It is necessary to establish an effective multidisciplinary team(MDT)to control the antimicrobial resistance.

OBJECTIVE To analyze the clinical characteristics and the correlation between laboratory indicators and prognosis of severe Mycoplasma pneumoniae pneumonia(SMPP)in children.METHODS A total of 85 children with SMPP admitted to Anhui Provincial Children's Hospital from Nov.2021 to May 2024 were selected as the study subjects.Based on clinical typing at admission,they were divided into a high-risk group(n=59)and a low-risk group(n=26).The clinical manifestations,laboratory indicators and outcomes at 28 days of treatment were compared between the two groups.RESULTS The duration of fever and cough before admission in the high-risk group was(7.17±1.09)days and(6.79±1.25)days,respectively,which was longer than that in the low-risk group(P＜0.05).There were no statistically significant differences in pulmonary auscultation(wheezing rales,moist rales)and extrapulmonary complications between the two groups.The levels of C-reactive protein(CRP),serum amyloid A(SAA),platelets(PLT),fibrinogen(FIB),D-dimer(DD)and N-terminal pro-brain natriuretic peptide(NT-proBNP)in the high-risk group were(11.62±1.45)mg/L,(226.88±36.83)mg/L,(3 18.57±39.82)×109/L,(4.28±0.74)g/L,(0.81±0.12)μg/ml and(2 295.48±413.75)pg/ml,respectively,all of which were higher than those in the low-risk group(P＜0.05).Within 28 days after treatment of children in both groups,one patient in the high-risk group died.CONCLUSIONS Compared with children with SMPP in the low-risk group,those in the high-risk group have a higher risk of prognostic mor-tality,suggesting a correlation between the children's blood CRP,SAA,PLT,FIB,DD and NT-proBNP levels and the prognosis of children with SMPP.

OBJECTIVE To analyze the clinical characteristics and the correlation between laboratory indicators and prognosis of severe Mycoplasma pneumoniae pneumonia(SMPP)in children.METHODS A total of 85 children with SMPP admitted to Anhui Provincial Children's Hospital from Nov.2021 to May 2024 were selected as the study subjects.Based on clinical typing at admission,they were divided into a high-risk group(n=59)and a low-risk group(n=26).The clinical manifestations,laboratory indicators and outcomes at 28 days of treatment were compared between the two groups.RESULTS The duration of fever and cough before admission in the high-risk group was(7.17±1.09)days and(6.79±1.25)days,respectively,which was longer than that in the low-risk group(P＜0.05).There were no statistically significant differences in pulmonary auscultation(wheezing rales,moist rales)and extrapulmonary complications between the two groups.The levels of C-reactive protein(CRP),serum amyloid A(SAA),platelets(PLT),fibrinogen(FIB),D-dimer(DD)and N-terminal pro-brain natriuretic peptide(NT-proBNP)in the high-risk group were(11.62±1.45)mg/L,(226.88±36.83)mg/L,(3 18.57±39.82)×109/L,(4.28±0.74)g/L,(0.81±0.12)μg/ml and(2 295.48±413.75)pg/ml,respectively,all of which were higher than those in the low-risk group(P＜0.05).Within 28 days after treatment of children in both groups,one patient in the high-risk group died.CONCLUSIONS Compared with children with SMPP in the low-risk group,those in the high-risk group have a higher risk of prognostic mor-tality,suggesting a correlation between the children's blood CRP,SAA,PLT,FIB,DD and NT-proBNP levels and the prognosis of children with SMPP.

Objective:To investigate the clinical and genetic characteristics of a family with cataplexy and epilepsy caused by KCNA1 gene mutations. Methods:The clinical data of a family with KCNA1 gene mutations leading to cataplexy and epilepsy who hospitalized in the Department of Pediatric Neurology at Anhui Children's Hospital in August 2022 were collected，and their clinical manifestations，imaging，electroencephalogram，gene testing results and treatment were analyzed. A total of 68 pathogenic or potentially pathogenic variants of the KCNA1 gene were identified by searching the database of CNKI，Wanfang Data Knowledge Service Platform, ClinVar, dbSNP and PubMed using the keyword‘KCNA1’between the establishment and August 2023. Results:The proband was a 9 years and 8 months old boy who initially presented with cataplexy induced by strenuous exercise or fatigue，followed by focal epilepsy. The whole exome sequencing detected heterozygous variation of exon 2 c.1006G>A（p.Gly336Arg）of KCNA1 gene，which was a missense mutation and was not reported in the country or abroad. Both the mother and brother of the proband detected heterozygous mutations at the same locus，and both had cataplexy induced by strenuous exercise or fatigue，but the type of seizure was generalized tonic-clonic seizure. The proband's grandmother，aunt，and brother all had seizures or cataplexy. Affected patients receiving different or the same anti-seizure drugs（sodium valproate，lamotrigine，phenytion sodium and carbamazepine）had varying degrees of relief，and those treated with sodium channel blockers had varying degrees of relief. A total of 68 mutation sites of KCNA1 gene were retrieved from domestic and foreign literature，mainly missense mutations，and most patients showed episodic ataxia type 1（EA1），and there was genetic heterogeneity between the genotype and phenotype of the variable patients. Conclusion:We have reported a heterozygous mutation in the KCNA1 gene c.1006G>A（p.Gly336Arg），which is a missense mutation and is easy to misdiagnose in patients with cataplexy and epilepsy as the main phenotypes. Patients with the KCNA1 mutation have different degrees of efficacy on sodium channel blockers.

Objective:To investigate the clinical and genetic characteristics of a family with cataplexy and epilepsy caused by KCNA1 gene mutations. Methods:The clinical data of a family with KCNA1 gene mutations leading to cataplexy and epilepsy who hospitalized in the Department of Pediatric Neurology at Anhui Children's Hospital in August 2022 were collected，and their clinical manifestations，imaging，electroencephalogram，gene testing results and treatment were analyzed. A total of 68 pathogenic or potentially pathogenic variants of the KCNA1 gene were identified by searching the database of CNKI，Wanfang Data Knowledge Service Platform, ClinVar, dbSNP and PubMed using the keyword‘KCNA1’between the establishment and August 2023. Results:The proband was a 9 years and 8 months old boy who initially presented with cataplexy induced by strenuous exercise or fatigue，followed by focal epilepsy. The whole exome sequencing detected heterozygous variation of exon 2 c.1006G>A（p.Gly336Arg）of KCNA1 gene，which was a missense mutation and was not reported in the country or abroad. Both the mother and brother of the proband detected heterozygous mutations at the same locus，and both had cataplexy induced by strenuous exercise or fatigue，but the type of seizure was generalized tonic-clonic seizure. The proband's grandmother，aunt，and brother all had seizures or cataplexy. Affected patients receiving different or the same anti-seizure drugs（sodium valproate，lamotrigine，phenytion sodium and carbamazepine）had varying degrees of relief，and those treated with sodium channel blockers had varying degrees of relief. A total of 68 mutation sites of KCNA1 gene were retrieved from domestic and foreign literature，mainly missense mutations，and most patients showed episodic ataxia type 1（EA1），and there was genetic heterogeneity between the genotype and phenotype of the variable patients. Conclusion:We have reported a heterozygous mutation in the KCNA1 gene c.1006G>A（p.Gly336Arg），which is a missense mutation and is easy to misdiagnose in patients with cataplexy and epilepsy as the main phenotypes. Patients with the KCNA1 mutation have different degrees of efficacy on sodium channel blockers.

Objective This study aimed to investigate the antimicrobial resistance profiles of bacterial strains isolated from pediatric intensive care units(PICU)in China for better antimicrobial therapy.Methods Clinical isolates were collected from 17 institutions,including tertiary care children's hospitals and pediatric department of tertiary general hospitals in China from January 1,2020 to December 31,2022.Antimicrobial susceptibility testing was carried out according to a unified protocol using Kirby-Bauer method or automated systems.Results were interpreted according to the breakpoints released by the Clinical and Laboratory Standards Institute(CLSI)in 2020.Results A total of 10 688 isolates were collected,including gram-positive organisms(39.2％)and gram-negative organisms(60.8％).The top three organisms were S.aureus(13.6％,1 453/10 688),A.baumannii(10.0％,1 067/10 688),and coagulase-negative Staphylococcus(9.9％,1 058/10 688).Multi-drug resistant organisms(MDROs)were very common in children.The prevalence of methicillin-resistant Staphylococcus aureus(MRSA),carbapenem-resistant Enterobacterales(CRE),carbapenem-resistant E.coli,carbapenem-resistant K.pneumoniae(CRKP),carbapenem-resistant A.baumannii(CRAB),and carbapenem-resistant P.aeruginosa(CRPA)was 41.1％,19.4％,8.8％,30.9％,67.4％,and 28.8％,respectively.Overall,more than 50％of Enterobacteriales isolates were resistant to cephalosporins,while nearly 25％of Enterobacteriales isolates were resistant to carbapenems.MDROs were highly resistant to commonly used antibiotics.More than 80％of CRE and CRAB strains were resistant to all beta-lactam antibiotics.CRE and CRAB showed low resistance rates to tigecycline and polymyxin.CRPA showed lower resistance rates to piperacillin,beta-lactamase inhibitor combinations than the resistance rates to third and fourth generation cephalosporins.All of the Staphylococcus and Enterococcus isolates were susceptible to vancomycin and tigecycline.None of PRSP strains isolated from meningitis and nonmeningitis samples were resistant to rifampicin,vancomycin,or linezolid.The prevalence of β-lactamase-negative ampicillin-resistant(BLNAR)strains was 43.3％in Haemophilus influenzae.Conclusions MDROs were prevalent in PICU.It is necessary to establish an effective multidisciplinary team(MDT)to control the antimicrobial resistance.

Familial acute necrotizing encephalopathy，also known as acute necrotizing encephalopathy type 1（ANE1），is a rapidly progressing rare encephalopathy with high morbidity and mortality rates.Although the etiology and pathogenesis remain unclear，mutations in genes such as RANBP2 and CPTⅡ are known to be related to its occurrence and development.Additionally，pathogen infections and cytokine storms are also considered potential influencing factors.Compared to isolated acute necrotizing encephalopathy，the clinical manifestations of ANE1 are more diverse，making diagnosis and treatment more complex.Currently，there are no specific therapeutic strategies for ANE1，and the effectiveness of immunomodulatory therapy remains controversial.Mitochondrial cocktail therapy and interleukin inhibitors have shown some therapeutic potential.The prognosis for ANE1 is generally poor，with survivors likely to experience severe neurological deficits and have a risk of recurrence.Early diagnosis and treatment are crucial for improving prognosis.

In recent years, as researchers delve deeply into the cell death mechanisms, ferroptosis, as a new iron-dependent cell death mode, has gradually become a research hotspot.It is mainly triggered by iron overload and lipid peroxidation in organisms.Sepsis-induced liver injury is an important pathophysiological process in the systemic inflammatory response caused by infection, which is closely related to various cell death modes.This review aims to explore the research progress on ferroptosis in sepsis-induced liver injury, including the mechanism of ferroptosis occurrence in sepsis-induced liver injury, its relationship with sepsis-induced liver injury, and possible therapeutic strategies for ferroptosis.

Hemophagocytic lymphohistiocytosis（HLH）is a complex，rapidly progressing，and highly fatal disease that can lead to during multiple organ dysfunctions.Coagulation disorders frequently occur during the course of HLH and are a significant cause of early mortality.Early diagnosis of coagulation disorders or disseminated intravascular coagulation（DIC）in such patients is particularly challenging，as HLH itself interferes with the diagnostic markers associated with coagulation disorders or DIC.This article summarized the current state of research on HLH complicated by coagulation disorders，including its pathogenesis and the common and novel coagulation markers used in the collaborative assessment of HLH complicated by coagulation disorders or DIC.The goal was to provide more opportunities for subsequent treatment of the underlying disease.

Objective:To investigate the clinical and genetic characteristics of a child with microcephaly-capillary malformation syndrome due to a STAMBP mutation. Methods:The clinical data of a case of microcephaly-capillary malformation syndrome caused by STAMBP gene mutation admitted to Anhui Children′s Hospital in August 2023 were collected. The genes of the child and his parents were detected by whole exome sequencing, and the risk was predicted by biological software. At the same time, the clinical characteristics of the child were analyzed and the literature was reviewed. Results:The patient is a male child aged 2 years and 7 months. The patient had special features, microcephaly, refractory epilepsy, severe comprehensive developmental delay, and capillary malformations. The results of genetic testing showed that the STAMBP gene in the child had complex heterozygous mutations NM_001353967: c.367delG[p.E123fs *27(p.Glu123fsTer27) and c.159A>C(p.Glu53Asp)], inherited from his mother and father respectively. The mutations have not been recorded in the HGMD, dbSNP and gnomAD databases,etc. The protein structure of the STAMBP gene was modeled that predicted c.367del(p.Glu123Lysfs *27) and c.159A>C (p.Glu53Asp) had a negative effect on the function of STAMBP protein. Conclusion:The STAMBP gene complex heterozygous mutations c.367delG(p.Glu123fsTer27) and c.159A>C(p.Glu53Asp) may be the pathogenic factor of this child, which further expands the variation spectrum of the STAMBP gene and the genotype of microcephaly-capillary malformation syndrome, and provides guidance for family genetic counseling.