Objective:To summarize the clinical and genetic features of neuroinflammation, autoinflammation, splenomegaly and anemia (NASA) syndrome and investigate the pathogenic mechanism.Methods:The clinical data of 2 patients diagnosed with NASA syndrome at Department of Pediatrics, Peking Union Medical College Hospital were retrospectively analyzed. Variants were identified by gene panel sequencing and confirmed by Sanger sequencing. The function of IRAK4 gene variants was studied in vitro.Results:Among the 2 patients, case 1 was an 8-year-old girl and case 2 was a 10-year-old boy. Both patients presented in early childhood with anemia and hepatosplenomegaly. Case 1 was also experienced recurrent seizures. Laboratory examinations showed elevated inflammatory markers and neuroimaging revealed bilateral basal ganglia calcification. In case 2, anemia and inflammation markers were well controlled after treatment with tocilizumab, while case 1 succumbed to recurrent seizures. Genetic tests verified compound heterozygous variants in IRAK4 gene: case 1 carries a nonsense variant c.592G>T (p.G198X) and a missense variant c.248A>C (p.D83A), which were respectively from the parents; case 2 carries a c.831+3A>G variant and a frameshift variant c.540delT (p.F180Lfs*26), and the former was inherited from the father and the latter from the mother. The reverse transcription and Sanger sequencing results confirmed that c.831+3A>G variant led to exon 7 skipping. In vitro studies indicated that c.592G>T, c.540delT and c.831+3A>G variants resulted in truncated interleukin-1 receptor-associated kinase-4 (IRAK4) protein while c.248A>C do not cause changes in IRAK4 protein expression level and protein length.Conclusions:NASA syndrome should be considered in children with early-onset anemia, hepatosplenomegaly, recurrent seizures, elevated inflammatory markers and intracranial calcification. IRAK4 gene variants may lead to impaired anti-inflammatory function of IRAK4 protein, contributing to the autoinflammatory phenotype.

Objective To investigate the effect of miR-486-5P on ferroptosis in H9c2 cardiomyocytes after hypoxia/reoxygenation(H/R),and to analyze its mechanism.Methods Using H9c2 cardiomyocytes as the research object,a H/R injury model was established using cobalt chloride(CoCl2)and fresh culture medium.The cells were divided into control group,H/R group,H/R+miR-486-5P mimic NC group,H/R+miR-486-5P mimic group,H/R+miR-486-5P inhibitor NC group and H/R+miR-486-5P inhibitor group.The relative expression level of miR-486-5P was detected by quantitative reverse transcription-polymerase chain reaction(qRT-PCR).The cell viability was detected by CCK-8 method.The activities or levels of lactate dehydrogen-ase(LDH),glutathione(GSH),Fe2+and malondialdehyde(MDA)were detected by colorimetric method.The levels of reactive oxygen species(ROS)and mitochondrial membrane potential(MMP)were detected by DCFH-DA fluorescent probe and JC-1 assay,respectively.Western blot was used to detect the levels of AkT/mTOR signaling pathway proteins and ferroptosis related protein solute carrier family 7 member 11(SLC7A11),glutathione peroxidase 4(GPX4)and acyl-coa synthetase long chain family member 4(ACSL4).Results Compared with the control group,the level of miR-486-5P and cell viability in the H/R group de-creased significantly(P＜0.05),while LDH activity,MDA,Fe2+level,ROS level and ACSL4 protein level in-creased significantly(P＜0.05).The GSH,MMP,SLC7A11 and GPX4 levels and p-Akt/Akt and p-mTOR/mTOR ratios were significantly decreased(P＜0.05).After H/R treatment,compared with the H/R+miR-486-5P mimic NC group,the cell viability of the H/R+miR-486-5P mimic group was significantly increased(P＜0.05).The LDH activity,MDA,Fe2+level,ROS level and ACSL4 protein level were significantly de-creased(P＜0.05),while GSH,MMP,SLC7A11 and GPX4 levels and p-Akt/Akt and p-mTOR/mTOR ratios were significantly increased(P＜0.05).Compared with the H/R+miR-486-5P inhibitor NC group,the trend of the above indicators in the H/R+miR-486-5P inhibitor group was opposite.Conclusion miR-486-5P allevi-ates hypoxia/reoxygenation-induced ferroptosis in H9c2 cells by regulating Akt/mTOR signaling pathway,and thus alleviates hypoxia/reoxygenation induced cardiomyocyte injury.

Objective:To summarize the clinical and genetic features of neuroinflammation, autoinflammation, splenomegaly and anemia (NASA) syndrome and investigate the pathogenic mechanism.Methods:The clinical data of 2 patients diagnosed with NASA syndrome at Department of Pediatrics, Peking Union Medical College Hospital were retrospectively analyzed. Variants were identified by gene panel sequencing and confirmed by Sanger sequencing. The function of IRAK4 gene variants was studied in vitro.Results:Among the 2 patients, case 1 was an 8-year-old girl and case 2 was a 10-year-old boy. Both patients presented in early childhood with anemia and hepatosplenomegaly. Case 1 was also experienced recurrent seizures. Laboratory examinations showed elevated inflammatory markers and neuroimaging revealed bilateral basal ganglia calcification. In case 2, anemia and inflammation markers were well controlled after treatment with tocilizumab, while case 1 succumbed to recurrent seizures. Genetic tests verified compound heterozygous variants in IRAK4 gene: case 1 carries a nonsense variant c.592G>T (p.G198X) and a missense variant c.248A>C (p.D83A), which were respectively from the parents; case 2 carries a c.831+3A>G variant and a frameshift variant c.540delT (p.F180Lfs*26), and the former was inherited from the father and the latter from the mother. The reverse transcription and Sanger sequencing results confirmed that c.831+3A>G variant led to exon 7 skipping. In vitro studies indicated that c.592G>T, c.540delT and c.831+3A>G variants resulted in truncated interleukin-1 receptor-associated kinase-4 (IRAK4) protein while c.248A>C do not cause changes in IRAK4 protein expression level and protein length.Conclusions:NASA syndrome should be considered in children with early-onset anemia, hepatosplenomegaly, recurrent seizures, elevated inflammatory markers and intracranial calcification. IRAK4 gene variants may lead to impaired anti-inflammatory function of IRAK4 protein, contributing to the autoinflammatory phenotype.

With the continuous development of information technology,smart financial systems has gradually become an important tool for financial management in public hospitals.As an important component of the smart financial system,electronic accounting record management is of great practical significance in adapting to the trend of"green and carbon free"economic development,improving the efficiency of financial management in public hospitals,and promoting high-quality development of hospitals.Henan Provincial People's Hospital is taken as an example(hereinafter referred to as the sample hospital)to deeply interpret national policies,explore the difficulties in electronic accounting file management,and combine with the specific situation of the hospital.Guided by the overall strategic goals of the hospital,starting from the top-level design integration,it creates a set of replicable and promotable electronic accounting voucher electronic reimbursement,entry,and archiving progress,so as to provide theoretical and practical experience for the transformation and upgrading of electronic record management in public hospitals,and provide a new template for the development and reform of economic management in public hospitals in China.

With the continuous development of information technology,smart financial systems has gradually become an important tool for financial management in public hospitals.As an important component of the smart financial system,electronic accounting record management is of great practical significance in adapting to the trend of"green and carbon free"economic development,improving the efficiency of financial management in public hospitals,and promoting high-quality development of hospitals.Henan Provincial People's Hospital is taken as an example(hereinafter referred to as the sample hospital)to deeply interpret national policies,explore the difficulties in electronic accounting file management,and combine with the specific situation of the hospital.Guided by the overall strategic goals of the hospital,starting from the top-level design integration,it creates a set of replicable and promotable electronic accounting voucher electronic reimbursement,entry,and archiving progress,so as to provide theoretical and practical experience for the transformation and upgrading of electronic record management in public hospitals,and provide a new template for the development and reform of economic management in public hospitals in China.

With the continuous development of information technology,smart financial systems has gradually become an important tool for financial management in public hospitals.As an important component of the smart financial system,electronic accounting record management is of great practical significance in adapting to the trend of"green and carbon free"economic development,improving the efficiency of financial management in public hospitals,and promoting high-quality development of hospitals.Henan Provincial People's Hospital is taken as an example(hereinafter referred to as the sample hospital)to deeply interpret national policies,explore the difficulties in electronic accounting file management,and combine with the specific situation of the hospital.Guided by the overall strategic goals of the hospital,starting from the top-level design integration,it creates a set of replicable and promotable electronic accounting voucher electronic reimbursement,entry,and archiving progress,so as to provide theoretical and practical experience for the transformation and upgrading of electronic record management in public hospitals,and provide a new template for the development and reform of economic management in public hospitals in China.

With the continuous development of information technology,smart financial systems has gradually become an important tool for financial management in public hospitals.As an important component of the smart financial system,electronic accounting record management is of great practical significance in adapting to the trend of"green and carbon free"economic development,improving the efficiency of financial management in public hospitals,and promoting high-quality development of hospitals.Henan Provincial People's Hospital is taken as an example(hereinafter referred to as the sample hospital)to deeply interpret national policies,explore the difficulties in electronic accounting file management,and combine with the specific situation of the hospital.Guided by the overall strategic goals of the hospital,starting from the top-level design integration,it creates a set of replicable and promotable electronic accounting voucher electronic reimbursement,entry,and archiving progress,so as to provide theoretical and practical experience for the transformation and upgrading of electronic record management in public hospitals,and provide a new template for the development and reform of economic management in public hospitals in China.

With the continuous development of information technology,smart financial systems has gradually become an important tool for financial management in public hospitals.As an important component of the smart financial system,electronic accounting record management is of great practical significance in adapting to the trend of"green and carbon free"economic development,improving the efficiency of financial management in public hospitals,and promoting high-quality development of hospitals.Henan Provincial People's Hospital is taken as an example(hereinafter referred to as the sample hospital)to deeply interpret national policies,explore the difficulties in electronic accounting file management,and combine with the specific situation of the hospital.Guided by the overall strategic goals of the hospital,starting from the top-level design integration,it creates a set of replicable and promotable electronic accounting voucher electronic reimbursement,entry,and archiving progress,so as to provide theoretical and practical experience for the transformation and upgrading of electronic record management in public hospitals,and provide a new template for the development and reform of economic management in public hospitals in China.

With the continuous development of information technology,smart financial systems has gradually become an important tool for financial management in public hospitals.As an important component of the smart financial system,electronic accounting record management is of great practical significance in adapting to the trend of"green and carbon free"economic development,improving the efficiency of financial management in public hospitals,and promoting high-quality development of hospitals.Henan Provincial People's Hospital is taken as an example(hereinafter referred to as the sample hospital)to deeply interpret national policies,explore the difficulties in electronic accounting file management,and combine with the specific situation of the hospital.Guided by the overall strategic goals of the hospital,starting from the top-level design integration,it creates a set of replicable and promotable electronic accounting voucher electronic reimbursement,entry,and archiving progress,so as to provide theoretical and practical experience for the transformation and upgrading of electronic record management in public hospitals,and provide a new template for the development and reform of economic management in public hospitals in China.

With the continuous development of information technology,smart financial systems has gradually become an important tool for financial management in public hospitals.As an important component of the smart financial system,electronic accounting record management is of great practical significance in adapting to the trend of"green and carbon free"economic development,improving the efficiency of financial management in public hospitals,and promoting high-quality development of hospitals.Henan Provincial People's Hospital is taken as an example(hereinafter referred to as the sample hospital)to deeply interpret national policies,explore the difficulties in electronic accounting file management,and combine with the specific situation of the hospital.Guided by the overall strategic goals of the hospital,starting from the top-level design integration,it creates a set of replicable and promotable electronic accounting voucher electronic reimbursement,entry,and archiving progress,so as to provide theoretical and practical experience for the transformation and upgrading of electronic record management in public hospitals,and provide a new template for the development and reform of economic management in public hospitals in China.