Objective To observe the value of deep learning image reconstruction(DLIR)algorithm in brain CT perfusion(CTP)using a protocol of 70 kVp and 40 ml contrast agent dose.Methods Totally 105 patients with suspected acute ischemic stroke(AIS)were prospectively enrolled and randomly divided into 3 groups,who underwent standard dose CTP scanning with 80 kVp and 150 mA combined with reconstruction as adaptive statistic iterative reconstruction V(ASIR-V)at 50％level(CN group,n=35),low dose(LD)scanning with 70 kVp and 100 mA combined with DLIR reconstruction at the highest level(DLIR-H)(LD group,n=35),or ultra-low dose(ULD)scanning with 70 kVp and 70 mA combined with DLIR-H reconstruction(ULD group,n=35).Radiation doses were compared among 3 groups.CT values and standard deviations(SDCT)of ROI of gray matter and white matter in the frontal,parietal and temporal lobes were measured.Signal-to-noise ratio(SNR)and contrast-to-noise ratio(CNR)between gray and white matter were calculated and compared among groups.Then pseudo-color images of cerebral blood volume(CBV),cerebral blood flow(CBF),mean transit time(MTT)and time to maximum of the tissue residual function(Tmax)were generated.The imaging quality of CTP pseudo-color images was evaluated,and the compatibility of the subjective scores within every kind of CTP pseudo-color images were assessed using Kappa test.Quantitative perfusion parameters were measured and compared among groups.Results Compared with CN group,both LD and ULD groups demonstrated significantly reduced volume CT dose index(CTDIvol),dose-length product(DLP)and effective dose(ED)(all adjusted P＜0.05).In ULD group,SDCT of white matter in frontal,parietal and temporal lobes were higher than those in CN group,and SDCT of white matter in parietal lobe was also higher than that in LD group(all adjusted P＜0.05).No significant difference of SDCT of gray matter was observed among groups(all P＞0.05).SNR of white matter in parietal and temporal lobes in both LD and ULD groups were lower than those in CN group(all P＜0.05),while no significant difference of SNR of white matter in frontal lobe,nor of gray matter in frontal,parietal and temporal lobes was found among groups(all P＞0.05).CNR of gray and white matter in the frontal,parietal and temporal lobes were not significantly different among groups(all P＞0.05).High consistency of inter-observer subjective scores of CBV maps,CBF maps and Tmax maps(Kappa of 0.623,0.644 and 0.638,respectively)were noticed,which of MTT maps had moderate consistency(Kappa=0.560).No significant difference of intra-obsever subjective scores of CTP pseudo-color images was found among groups(all P＞0.05).CBV,CBF,MTT and Tmax values of gray and white matter in frontal,parietal and temporal lobes were not significantly different among groups(all P＞0.05).Conclusion DLIR algorithm applicated in low radiation dose and reduced contrast agent dosage might ensure imaging quality.

Objective To investigate the protective effect and mechanism of adenosine-lidocaine-magnesium sulfate(ALM)resuscita-tion solution against acute lung injury in sepsis were investigated through in vitro and in vivo experiments.Methods ALM resuscitation solution effects on septic lung injury were assessed in SD rats and rat pulmonary micro vascular endothelial cells(PMVEC)in vitro,and the effector mechanism was explored by using network pharmacology combined with molecular biology methods,detecting alterations in nuclear factor kappaB(NF-κB)signaling pathway and inflammatory cytokine key protein expressions.Results Based on in vivo experi-ments,ALM resuscitation solution treatment significantly improved lung histopathological injury in cecal ligation and puncture(CLP)model rats,reduced the lung injury score and lung dry-to-wet ratio(P＜0.01),and significantly suppressed pro-inflammatory cytokine expressions such as that of IL-1β,IL-6,and TNF-α(P＜0.01).Moreover,in vitro experiments confirmed that ALM resuscitation solution significantly reduced p-p65 and p-IκBα protein expressions in LPS-induced PMVEC(P＜0.05),while down-regulating IL-1β,IL-6,and TNF-α protein levels(P＜0.01).Conclusion ALM resuscitation solution exerts a protective effect against acute lung injury in sepsis by inhibiting NF-κB signaling pathway activation and reducing pro-inflammatory cytokine release.

Purpose To develop and validate a nomogram based on 18F-FDG PET/CT for predicting spread through air space(STAS)in stage IA lung adenocarcinoma(LUAD),providing imaging support for surgical decision-making.Materials and Methods This retrospective study analyzed 155 patients with pathologically confirmed stage IA LUAD from the Fifth People's Hospital of Chongqing,Affiliated Hospital of Zunyi Medical University and Cavalier Hospital(December 2019 to December 2023).Patients were stratified into STAS-positive and STAS-negative groups based on postoperative histopathological evaluation.Univariate and multivariate binary Logistic regression analyses identified risk factors for STAS from clinical characteristics,PET/CT semantic features,and metabolic parameters.A nomogram predicting preoperative STAS status was developed and internally validated using bootstrap resampling.Model performance was assessed in training and internal validation sets using receiver operating characteristic analysis,calibration curves and decision curve analysis.Results Among 155 stage IA LUAD patients,20(12.9%)were STAS-positive.Multivariate analysis identified tumor mean diameter(OR=0.618,P=0.024),solid component proportion(OR=2.678,P=0.033),and maximum standardized uptake value(OR=3.437,P=0.028)as independent STAS predictors.The nomogram demonstrated excellent discrimination,with areas under the curve of 0.912(95%CI 0.855-0.942)and 0.843(95%CI 0.805-0.923)in training and validation sets,respectively.Hosmer-Lemeshow goodness-of-fit test indicated satisfactory calibration(χ2=5.591,P=0.693).Decision curve analysis showed substantial net clinical benefit across threshold probabilities of 5%-91%.Conclusion The nomogram incorporating tumor diameter,solid component proportion,and maximum standardized uptake value from 18F-FDG PET/CT effectively predicts STAS status in stage IA LUAD,demonstrating high preoperative predictive performance.

Purpose To develop and validate a nomogram based on 18F-FDG PET/CT for predicting spread through air space(STAS)in stage IA lung adenocarcinoma(LUAD),providing imaging support for surgical decision-making.Materials and Methods This retrospective study analyzed 155 patients with pathologically confirmed stage IA LUAD from the Fifth People's Hospital of Chongqing,Affiliated Hospital of Zunyi Medical University and Cavalier Hospital(December 2019 to December 2023).Patients were stratified into STAS-positive and STAS-negative groups based on postoperative histopathological evaluation.Univariate and multivariate binary Logistic regression analyses identified risk factors for STAS from clinical characteristics,PET/CT semantic features,and metabolic parameters.A nomogram predicting preoperative STAS status was developed and internally validated using bootstrap resampling.Model performance was assessed in training and internal validation sets using receiver operating characteristic analysis,calibration curves and decision curve analysis.Results Among 155 stage IA LUAD patients,20(12.9%)were STAS-positive.Multivariate analysis identified tumor mean diameter(OR=0.618,P=0.024),solid component proportion(OR=2.678,P=0.033),and maximum standardized uptake value(OR=3.437,P=0.028)as independent STAS predictors.The nomogram demonstrated excellent discrimination,with areas under the curve of 0.912(95%CI 0.855-0.942)and 0.843(95%CI 0.805-0.923)in training and validation sets,respectively.Hosmer-Lemeshow goodness-of-fit test indicated satisfactory calibration(χ2=5.591,P=0.693).Decision curve analysis showed substantial net clinical benefit across threshold probabilities of 5%-91%.Conclusion The nomogram incorporating tumor diameter,solid component proportion,and maximum standardized uptake value from 18F-FDG PET/CT effectively predicts STAS status in stage IA LUAD,demonstrating high preoperative predictive performance.

Objective:To summarize the clinical phenotypes, genotypes, and treatment outcomes of NPRL2-related epilepsy. Methods:This was a case summary.Clinical data of patients with NRPL2 variants admitted to the Department of Pediatrics, Peking University People′s Hospital between October 1, 2013 and October 31, 2023 were retrospectively analyzed.Previous reports of patients with the same disease were reviewed. Results:Six cases of NPRL2-related epilepsy were collected, and 37 cases were reported in the previous literatures.The age of onset ranged from 3 days to 18 years with the median age of 24 months.There were 15 patients with onset in infancy.Among the 41 patients diagnosed with epilepsy, 73.1% (30/41) had focal seizures, 34.1% (14/41) had frontal lobe epilepsy, and 17.1% (7/41) had epileptic spasms.Among the patients with known cranial imaging, 58.6% (17/29) had cortical malformations. NPRL2 variants involved 11 nonsense mutations, 10 splice site mutations, 7 frameshift mutations, 1 large fragment deletion, and 14 missense mutations; among them, 39 mutations were pathogenic or likely pathogenic, while the rest 4 mutations had unclear pathogenicity.Among the 27 patients with known outcomes, 11 (40.7%) had no seizures after administration of 1 or 2 types of drugs, and 16 (59.2%) had drug-resistant epilepsy.Among the 16 patients, 1 had no seizures after treatment with 3 types of anti seizure medications, and 7 had no seizures after surgery.Most patients had varying degrees of delay in intellectual and motor development. Conclusions:Patients with NPRL2 variants usually present with frequent focal seizures and epileptic spasms, and the age of onset varies greatly.About half of the patients have drug-resistant epilepsy, half of whom have cortical malformations.For those with drug-resistant epilepsy and abnormal cranial imaging, surgery may be considered.

Objective:To summarize the phenotype and genotype of leukoencephalopathy with calcifications and cysts(LCC).Methods:A case summary.Clinical, imaging, and genetic data of 2 patients with early-onset LCC admitted to the Department of Pediatrics, Peking University People′s Hospital between December 2023 and August 2024 were retrospectively summarized.A review of the literature was also conducted.Results:Case 1: a 19-month-old female infant presented with febrile seizures in infancy and mild developmental delay.Trio whole-exome sequencing (trio-WES) identified compound heterozygous pathogenic variants in the SNORD118 gene: n.92C>T (paternally inherited) and n. 72A>G (maternally inherited). Case 2: an 11-year-and-4-month-old girl had non-specific encephalopathy in the neonatal period, developmental delay with regression, and seizures since early childhood.Trio-WES revealed compound heterozygous pathogenic variants in SNORD118: n.3C>T (paternally inherited) and n. 57G>C (maternally inherited). Both cases showed typical imaging findings of leukoencephalopathy, intracranial calcifications, and cysts.Case 2 has been treated with Bevacizumab for 3 months and remains under follow-up.Combining this 2 cases with previously reported genetically confirmed cases, a total of 97 LCC patients with identified SNORD118 variants were analyzed.The median age of onset was 5 years.Seventy-one cases had childhood onset, including 31 cases with onset at ≤1 year.The inaugural symptoms were: seizures in 40 patients (41.2%), motor disorders in 25 patients (25.8%), developmental delay or cognitive impairment in 19 patients (19.6%) and headaches or increased intracranial pressure in 13 patients (13.4%). Neurological dysfunctions progress during the course.All patients had typical leukoencephalopathy, intracranial calcifications and cysts, with varied imaging progress.A total of 61 variants of SNORD118 were reported and most were compound heterozygous variants.Treatment is primarily symptomatic.Three out of the 4 patients treated with Bevacizumab showed improvement. Conclusions:LCC is a rare autosomal recessive inherited cerebral microangiopathy, characterized by progressive neurological dysfunction and radiological triad of diffuse and asymmetric leukoencephalopathy, intracranial calcifications and cysts.Patients with pathogenic SNORD118 variants should definitely be diagnosed.Symptomatic treatment is the mainstay therapy and Bevacizumab may slow down the progression.

Objective To investigate the protective effect and mechanism of adenosine-lidocaine-magnesium sulfate(ALM)resuscita-tion solution against acute lung injury in sepsis were investigated through in vitro and in vivo experiments.Methods ALM resuscitation solution effects on septic lung injury were assessed in SD rats and rat pulmonary micro vascular endothelial cells(PMVEC)in vitro,and the effector mechanism was explored by using network pharmacology combined with molecular biology methods,detecting alterations in nuclear factor kappaB(NF-κB)signaling pathway and inflammatory cytokine key protein expressions.Results Based on in vivo experi-ments,ALM resuscitation solution treatment significantly improved lung histopathological injury in cecal ligation and puncture(CLP)model rats,reduced the lung injury score and lung dry-to-wet ratio(P＜0.01),and significantly suppressed pro-inflammatory cytokine expressions such as that of IL-1β,IL-6,and TNF-α(P＜0.01).Moreover,in vitro experiments confirmed that ALM resuscitation solution significantly reduced p-p65 and p-IκBα protein expressions in LPS-induced PMVEC(P＜0.05),while down-regulating IL-1β,IL-6,and TNF-α protein levels(P＜0.01).Conclusion ALM resuscitation solution exerts a protective effect against acute lung injury in sepsis by inhibiting NF-κB signaling pathway activation and reducing pro-inflammatory cytokine release.

Purpose To investigate the value of non-invasive preoperative prediction of programmed death ligand 1 expression status in extrahepatic cholangiocarcinoma using MRI radiomics combined with clinical features through nomograms.Materials and Methods A retrospective collection was made of 87 cases of extrahepatic cholangiocarcinoma diagnosed through surgical pathology in the Affiliated Hospital of Southwest Medical University from January 2011 to December 2021.These were randomly divided into training and testing sets at a 7∶3 ratio.Using 3D-Slicer software,regions of interest were manually delineated layer-by-layer on MRI images,and radiomic features were extracted.Data normalization,feature dimensionality reduction and selection were then performed.A Gaussian naive Bayes classifier was used to construct the radiomics model,and radiomics scores were obtained.Multivariate Logistic regression was used to screen clinical features,and individual clinical and combined models were constructed.The predictive performances of the three models were evaluated using the area under the receiver operating characteristic curve(AUC),and the goodness of fit and clinical net benefit of the combined model nomogram were assessed through calibration and decision curves.Results Nine radiomic features and three clinical features were finally selected.The clinical features included alanine transaminase(P=0.020),aspartate transaminase(P=0.025)and total bilirubin(P=0.026).The predictive performance of the combined model(training set AUC 0.813,testing set AUC 0.818)was superior to that of the individual clinical model(training set AUC 0.711,testing set AUC 0.705)and the radiomics model(training set AUC 0.769,testing set AUC 0.767).Calibration and decision curves indicated good fit and better clinical net benefit for the combined model nomogram.Conclusion Based on preoperative multi-sequence MRI images and the radiomics score,along with alanine transaminase,aspartate transaminase and total bilirubin as clinical features,the constructed combined model nomogram effectively predicts the programmed death ligand 1 expression status in extrahepatic cholangiocarcinoma.This provides guidance for the precise and personalized immunotherapy for patients.

Objective:To summarize the clinical phenotypes, genotypes, and treatment outcomes of NPRL2-related epilepsy. Methods:This was a case summary.Clinical data of patients with NRPL2 variants admitted to the Department of Pediatrics, Peking University People′s Hospital between October 1, 2013 and October 31, 2023 were retrospectively analyzed.Previous reports of patients with the same disease were reviewed. Results:Six cases of NPRL2-related epilepsy were collected, and 37 cases were reported in the previous literatures.The age of onset ranged from 3 days to 18 years with the median age of 24 months.There were 15 patients with onset in infancy.Among the 41 patients diagnosed with epilepsy, 73.1% (30/41) had focal seizures, 34.1% (14/41) had frontal lobe epilepsy, and 17.1% (7/41) had epileptic spasms.Among the patients with known cranial imaging, 58.6% (17/29) had cortical malformations. NPRL2 variants involved 11 nonsense mutations, 10 splice site mutations, 7 frameshift mutations, 1 large fragment deletion, and 14 missense mutations; among them, 39 mutations were pathogenic or likely pathogenic, while the rest 4 mutations had unclear pathogenicity.Among the 27 patients with known outcomes, 11 (40.7%) had no seizures after administration of 1 or 2 types of drugs, and 16 (59.2%) had drug-resistant epilepsy.Among the 16 patients, 1 had no seizures after treatment with 3 types of anti seizure medications, and 7 had no seizures after surgery.Most patients had varying degrees of delay in intellectual and motor development. Conclusions:Patients with NPRL2 variants usually present with frequent focal seizures and epileptic spasms, and the age of onset varies greatly.About half of the patients have drug-resistant epilepsy, half of whom have cortical malformations.For those with drug-resistant epilepsy and abnormal cranial imaging, surgery may be considered.

Objective:To summarize the phenotype and genotype of leukoencephalopathy with calcifications and cysts(LCC).Methods:A case summary.Clinical, imaging, and genetic data of 2 patients with early-onset LCC admitted to the Department of Pediatrics, Peking University People′s Hospital between December 2023 and August 2024 were retrospectively summarized.A review of the literature was also conducted.Results:Case 1: a 19-month-old female infant presented with febrile seizures in infancy and mild developmental delay.Trio whole-exome sequencing (trio-WES) identified compound heterozygous pathogenic variants in the SNORD118 gene: n.92C>T (paternally inherited) and n. 72A>G (maternally inherited). Case 2: an 11-year-and-4-month-old girl had non-specific encephalopathy in the neonatal period, developmental delay with regression, and seizures since early childhood.Trio-WES revealed compound heterozygous pathogenic variants in SNORD118: n.3C>T (paternally inherited) and n. 57G>C (maternally inherited). Both cases showed typical imaging findings of leukoencephalopathy, intracranial calcifications, and cysts.Case 2 has been treated with Bevacizumab for 3 months and remains under follow-up.Combining this 2 cases with previously reported genetically confirmed cases, a total of 97 LCC patients with identified SNORD118 variants were analyzed.The median age of onset was 5 years.Seventy-one cases had childhood onset, including 31 cases with onset at ≤1 year.The inaugural symptoms were: seizures in 40 patients (41.2%), motor disorders in 25 patients (25.8%), developmental delay or cognitive impairment in 19 patients (19.6%) and headaches or increased intracranial pressure in 13 patients (13.4%). Neurological dysfunctions progress during the course.All patients had typical leukoencephalopathy, intracranial calcifications and cysts, with varied imaging progress.A total of 61 variants of SNORD118 were reported and most were compound heterozygous variants.Treatment is primarily symptomatic.Three out of the 4 patients treated with Bevacizumab showed improvement. Conclusions:LCC is a rare autosomal recessive inherited cerebral microangiopathy, characterized by progressive neurological dysfunction and radiological triad of diffuse and asymmetric leukoencephalopathy, intracranial calcifications and cysts.Patients with pathogenic SNORD118 variants should definitely be diagnosed.Symptomatic treatment is the mainstay therapy and Bevacizumab may slow down the progression.