Artificial intelligence(AI)has shown great potential in the field of medical imaging in recent years,which mainly focused on identifying and analyzing images,detecting and classifying lesions,as well as establishing intelligent auxiliary diagnostic systems on pediatric bone and joint.With the continuous development and breakthrough of machine learning,radiomics combined with natural language processing and other technologies,AI is pushing pediatric osteoarticular imaging to a new height.The research progresses of AI in pediatric osteoarticular imaging were reviewed in this article.

Objective:To analyze the clinical characteristics of patients of chronic active Epstein-Barr virus infection (CAEBV) with intestinal involvement misdiagnosed as inflammatory bowel disease (IBD).Methods:A retrospective analysis was conducted on the clinical characteristics, laboratory results, digestive endoscopic findings, histological results, treatment and prognosis of 10 patients with CAEBV intestinal involvement who were misdiagnosed as IBD and treated at the Department of Hematology, Beijing Friendship Hospital, Capital Medical University from February 2019 to November 2022. Epstein-Barr virus-encoded small RNA (EBER) was detected by in situ hybridization. Results:Among the 10 patients with CAEBV, eight were males and two were females. Seven patients had been misdiagnosed as ulcerative colitis and three misdiagnosed as Crohn′s disease. The median age of onset was 36 years (ranged from 26 to 52 years), and the median time from onset to CAEBV diagnosis was 18.5 months (ranged from 2.0 to 96.0 months). The main clinical characteristics of these patients included fever >38.5 ℃ in 10 cases, diarrhea in seven cases, abdominal pain in seven cases, abdominal lymph node enlargement in six cases and hematochezia in seven cases. Six patients primarily presented with gastrointestinal symptoms, and seven patients had involvement of extraintestinal organs, three patients developed hemorrhagic shock due to gastrointestinal bleeding. The laboratory findings included anemia in seven cases, elevated erythrocyte sedimentation rate in six cases, decreased natural killer cell activity in five cases, and elevated ferritin in three cases. Epstein-Barr virus (EBV) DNA were detected in the peripheral blood mononuclear cells (PBMCs) of nine patients, with a median viral load of 23 000 copies/mL. Seven patients were tested positive for anti-EBV viral capsid antigen IgG and nuclear antigen 1 IgG. The main endoscopy findings were hyperemia, edema of the affected intestinal wall mucosa, which could be accompanied by erosion, multiple scattered shallow ulcers with varying sizes. There were six patients with total colon involvement. The rectum was involved in three patients, and the esophagus, gastric antrum, duodenum and small intestine were each involved in one patient. Seven patients underwent follow-up colonoscopy after diagnosis, and four cases progressed. All 10 patients showed active chronic inflammation in the histopathological examinations of their intestinal tissue, with crypt changes in four cases and granulomatous changes in one cases. The intestinal tissues of eight patients were positive for EBER staining, and EBER positive cells≥50 cells/high-power field in seven patients. Seven patients were treated with 5-aminosalicylic acid before the correct diagnosis. Five patients had not improved or progressed upon the follow-up colonoscopy. Two patients died of uncontrolled massive hemorrhage of digestive tract.Conclusions:The clinical, endoscopic and pathological findings of patients with CAEBV intestinal involvement lack specificity. For IBD patients initially diagnosed accompanied by fever and evidence of extraintestinal organ involvement, it is recommended to simultaneously detect EBV DNA in PBMCs and blood plasma, EBER in intestinal tissue, and identify the main EBV-infected cells in peripheral blood and/or tissue, to distinguish CAEBV.

Objective:To explore the feasibility of constructing an objective tinnitus subtype model based on peripheral blood differentially expressed genes (DEGs) using a combination of Weighted Gene Co-expression Network Analysis (WGCNA) and Random Forest algorithm (RF).Methods:From October 2019 to June 2020, peripheral blood DEGs were obtained from 37 patients (from the Third Affiliated Hospital of Sun Yat-sen University)with chronic subjective high-frequency tinnitus (21 unbothersome type, 16 bothersome type) and 20 healthy volunteers through high-throughput sequencing. WGCNA was used to construct gene modules with different expression patterns and analyze their relationships with tinnitus characteristics. Subsequently, RF was employed to build subtype models, which were evaluated by the area under the receiver operating characteristic curve (AUC), accuracy, and F1-score.Results:A total of 12 351 intergroup DEGs were divided into 9 gene modules. Among them, MEblue, MEgreen, and MEbrown showed significant negative correlations with the healthy volunteer group, while MEpink showed a significant positive correlation with the tinnitus distress group. The "Tinnitus vs. Normal" and "Compensatory vs. Decompensatory" subtype models, based on MEblue and MEpink respectively, both had AUCs greater than 0.80, accuracies above 90%, and F1-scores above 0.90, indicating good performance.Conclusions:Peripheral blood DEGs are potential biological indicators for objective classification of subjective tinnitus. The combined application of WGCNA and the Random Forest algorithm should be a viable approach to constructing an objective tinnitus subtype model. However, further exploration and refinement are needed to validate the model′s generalizability, cross-dataset performance, and algorithm optimization.

Objective:To investigate the effectiveness of multimodal magnetic resonance imaging (MRI)-based radiomics models in the auxiliary diagnosis of neonatal cerebral white matter lesion (WML).Methods:Clinical and MRI imaging data were collected from 91 children diagnosed with WML in Dalian Women and Children′s Medical Center from April 2018 to December 2021, including 58 cases in the good prognosis group and 33 cases in the poor prognosis group. The region of interest (ROI) was drawn for the lesion of each sequence image (T 1WI, T 2WI, DWI and SWI). Three models, T 1WI+T 2WI (model 1), T 1WI + T 2WI + DWI (model 2) and T 1WI + T 2WI + SWI (model 3), were created. Resample of the ROI, pre-processing of images and extraction of features were performed by using PyRadiomics, and the extracted features were standardized on the Dr.Wise research platform. A logistic regression classifier was used to create the radiomics model and 5-fold cross-validation was carried out 10 times. Key features were screened, and the receiver operating characteristic (ROC) curve was drawn to evaluate the predictive ability of the models. And DeLong was used to compare the effectiveness of different models. Results:The AUC values of model 2 (0.95) and model 3 (0.93) in the validation set were higher than that of model 1 (0.90). The accuracy, sensitivity and specificity of model 1, model 2, and model 3 were (86%, 89% and 87%), (79%, 88% and 88%) and (90%, 90% and 86%), respectively. The differences between model 1 and model 2 by DeLong′s test was statistically significant ( P<0.05), whereas there was no statistically significant differences between model 3 and model 1 or between model 3 and model 2 ( P>0.05). Conclusions:Multimodal MRI-based radiomics model was proved to be an effective tool in the auxiliary diagnosis of neonatal WML.

Ischemia reperfusion injury (IRI) of organs is a major challenge for clinicians, but the mechanism is still not elucidated, and the clinical treatment effect is still unsatisfactory. PARP-1-dependent cell death (parthanatos) is a non-apototic programmed cell death pathway involved in the development of the occurrence of IRI of organs. At the same time, parthanatos is also a multi-step pathway. There are many molecules in the parthanatos cascade that can be exploited to create therapeutic interventions for IRI, including PARP1, apoptosis inducing factor (AIF), and macrophage migration inhibitory factor (MIF). These critical molecules are involved in DNA damage, energy depletion and homeostasis imbalance. Therefore, these molecular signals in the parthanatos cascade represent promising therapeutic targets for the treatment of IRI. In the following, we will elaborate on the mechanisms and molecular characteristics of the parthanatos pathway and the relation between parthanatos pathway and IRI of vital organs. It aims to explore the posibility of IRI mechanism research and clinical treatment and to provide new ideas for clinicians and researchers.

Objective:To investigate the efficacy of liposomal doxombicin combined with etoposide and high dose methylprednisolone (DEP) as a salvage therapy for refractory macrophage activation syndrome (MAS).Methods:Totally 38 patients with refractory MAS were enrolled in this study from January 2016 to January 2022 in Beijing Friendship Hospital, including clinical characteristics and laboratory test results before and after DEP treatment, were retrospectively collected. The efficacy was evaluated every 2 weeks according to the United States Midwest Cooperative HLH Group. Relevant samples were statistically analyzed using non-parametric tests.Results:Of 38 refractory MAS patients, 8 males and 30 females were included into this study.The median age was 30(15-69) years old. The underlying disease were adult onset Still's disease in 29 cases, Systemic lupus erythematosus in 6 cases, Rheumatoid arthritis in 1 case and Undifferentiated Connective-Tissue disease in 2. The overall response rate was 95% (36/38), including 9 patients (24%) achieved complete remission and 27 patients (71%) achieved partial remission after 2 weeks of treatment. The overall response rate was 97% (34/35), including 16 (46%) complete remission and 18 (51%) partial remission after 4 weeks of treatment(due to lack of data in some patients). The overall response rate was 97% (34/35), including 17 (49%) complete remission and 17 (49%) partial remission after 6 weeks of treatment. Patients who achieved partial remission or complete remission were actively treated for the underlying diseases after induction, and their conditions were in persistent remission.Conclusion:The DEP regimen may be an effective salvage therapy for the treatment of refractory MAS.

Objective:This study aimed to investigate the clinical characteristics of patients diagnosed with primary hemophagocytic lymphohistiocytosis (pHLH) associated with perforin gene deficiency.Methods:We retrospectively analyzed the clinical data of 16 pHLH patients associated with perforin gene deficiency at Beijing Friendship Hospital, Capital Medical University, from April 2014 to August 2021. The mutation sites, mutation types, family history, clinical characteristics, and prognosis of the patients were assessed.Results:A total of 16 patients, including ten males and six females, with a median onset age of 17.5 years (range: 4-42 years), were enrolled in this study. Sixteen different mutations were identified, consisting of 11 missense mutations, one nonsense mutation, two frameshift mutations, and two in-frame mutations. All patients harbored at least one deleterious missense mutation, with the most common mutation sites being c.1349C>T (p.T450M) and c.503G>A (p.S168N). Decreased natural killer (NK) cell activity was observed in 11 patients, reduced perforin protein expression in ten patients, concurrent Epstein-Barr virus (EBV) infection at onset in eight patients, a family history in two patients, and central nervous system involvement in four patients. Eleven cases underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT), with eight cases surviving. The median survival time of non-transplanted patients was eight months (range: 4-18 months), while that of transplanted patients was reported as "not reached".Conclusions:Emphasizing the diagnosis of pHLH in adults with perforin gene deficiency. In addition, it should be noted that EBV infection can potentially act as a triggering factor in such disease, and allo-HSCT exerts a substantial effect on the prognosis of patients.

Objective:To observe the effect of esketamine on cardiac index in patients undergoing lumbar surgery in prone position under general anesthesia.Methods:Forty-five patients with prone lumbar surgery after general anesthesia in Hunan Provincial People′s Hospital from March to July 2021 were divided into observation group (24 cases, group A) and control group (21 cases, group B) according to random number table method. Group A received 0.5 mg/kg esketamine intravenously during induction, and 0.15 mg/(kg·h) esketamine intravenously for 2 h after prone position. Group B received the same amount of normal saline. Both groups were given sevoflurane and remifentanil during operation to maintain anesthesia, and sufentanil was given intermittently during operation. The mean arterial pressure (MAP), systolic blood pressure (SBP), diastolic pressure (DBP), cardiac index (CI), and heart rate (HR) before induction (T 0), during endotracheal intubation (T 1), 5 minutes after intubation (T 2), 5 minutes after prone position (T 3), 10 minutes after prone position (T 4), 30 minutes after prone position (T 5), 45 minutes after prone position (T 6), 60 minutes after prone position (T 7), 90 minutes after prone position (T 8), and 120 minutes after prone position (T 9) were recorded; The total dosage of norepinephrine 2 hours after anesthesia to prone position and extubation time after operation were also recorded. The Visual Analogue Scale (VAS) was performed 15 minutes after extubation, 6 and 24 hours after operation. Results:There was no significant difference in CI between T 3-T 9 and T 2 in group A ( P>0.05); the CI of group B at T 3-T 7 was significantly lower than that at T 2 (all P<0.05); there was no significant difference in CI between T 8-T 9 and T 2 in group B (all P>0.05); There was no significant difference in CI between group A and group B at T 0-T 2 (all P>0.05). The CI of group A at T 3-T 9 was significantly higher than that of group B (all P<0.05); The dosage of norepinephrine in group A was significantly lower than that in group B ( P<0.05); There was no significant difference in HR, MAP, SBP and DBP between the two groups at different time points (all P>0.05); there was also no significant difference in extubation time and VAS scores at 15 minutes, 6 hours and 24 hours after extubation between the two groups (all P>0.05). Conclusions:Intraoperative application of esketamine can increase CI after prone position and reduce the amount of norepinephrine during lumbar surgery.

Objective:To explore the predictive value of N-terminal-pro-B-type brain natriaretic peptide (NT-pro BNP) and B-type brain natriaretic peptide (BNP) of symptomatic patent ductus arteriosus (sPDA) in premature infants.Methods:Sixty premature infants were recruited. The gestational age was 26-32 weeks and the birth weight was less than 1 500 g. According to the echocardiography measured on the 3rd day, the infants were divided into PDA group (40 cases) and control group (20 cases). PDA group was divided into sPDA group (20 cases) and asPDA group (20 cases). The sPDA group was divided into treatment group (10 cases) and non-treatment group (10 cases). The asPDA group was further divided into self-closed group (10 cases) and non- self-closed group (10 cases). NT-pro BNP and BNP were examined on the 3rd and 7th day.Results:The level of NT-pro BNP among sPDA group, asPDA group and control group had significant difference on the 3rd and 7th day ( P<0.05). On the 7th day, the level of NT-pro BNP between treatment group and non-treatment group had significant difference [4 082(111 - 7 566) ng/L vs. 9 643 (2 362 - 25 000) ng/L, P<0.05] between self-closed group and non- self-closed group [603 (27 - 1 698) ng/L vs. 4 595 (896 - 9 873) ng/L, P<0.05]. The level of NT-pro BNP on the 3rd day was positively correlated with of ductus arteriosus diameter, ratio of left atrium diameter and aortic root diameter, ratio of ductus arteriosus diameter and left pulmonary artery diameter ( r=0.358 - 0.455, P<0.05). The area under the curve drawn by NT-pro BNP on the 3rd day was 0.783. Conclusions:The level of NT-pro BNP on the 3rd day can be used as a predictor of sPDA.

Objective:This study was designed to evaluate the efficacy of haploidentical hematopoietic stem cell transplantation (haplo-HSCT) for adult-onset primary hemophagocytic lymphohistiocytosis (HLH) .Method:A retrospective study was carried out to analyze the clinical data of 15 adult patients with primary HLH who received haplo-HSCT from January 2013 to October 2019 in Beijing Friendship Hospital, Capital Medical University, Beijing, China.Results:Among the 15 patients included in the study, ten were males and five were females, with a median age of 21 years old (18-52) . Eight of the patients had familial hemophagocytic lymphohistiocytosis type 2 (FHL-2) , four had FHL-3, one had Griscelli syndrome type 2 (GS-2) , one had X-linked lymphoproliferative disease type 1 (XLP-1) , and the other had XLP-2. The median time from HLH diagnosis to transplantation was 7 months (2-46 months) . Seven patients were treated with Bu/Cy condition regimen prior to transplantation. Meanwhile, the other eight cases were treated with TBI/Cy. The median concentration of mononuclear cell (MNC) infusion was 12.6 (9.2-20.3) ×10 8/kg and CD34 + cells was 4.91 (2.51-8.37) ×10 6/kg. The median time of leukocyte engraftment was on day 13 following transplantation (10-23 days) , and the platelet engraftment was on day 12 (9-36) . Graft failure (GF) finally occurred in two patients (one primary GF and one secondary GF) . The cumulative incidence of acute graft-versus-host-disease (GVHD) grades 2 to 4 was 71.4% (10/14) and chronic GVHD was 30.8% (4/13) , respectively. The five-year overall survival (OS) for all 15 cases of primary HLH was 65.5% (95% CI, 34.9%-73.3%) and the transplant-related mortality (TRM) was 26.7% (4/15) . The five-year OS was 87.5% (95% CI, 38.7%-66.3%) in eight patients who received haplo-HSCT subsequent to initial therapy and 42.9% (95% CI, 8.5%-65.2%) in patients seven patients who needed salvage therapy prior to haplo-HSCT ( χ2=2.387, P=0.122) . The five-year OS was 85.7% (95% CI, 50.4%-89.8%) in eight patients who achieved complete response before haplo-HSCT and 42.9% (95% CI, 6.4%-53.0%) in seven patients with partial response ( χ2=3.185, P=0.074) . Conclusion:The results indicated that haplo-HSCT is a promising method for the treatment of primary HLH in adults.