Objective:To investigate the correlation between oxygen metabolism index and prognosis of children with severe sepsis.Methods:A retrospective study was conducted.Children with severe sepsis admitted to the Department of Pedaitric Intensive Care Unit, Children′s Hospital Affiliated to Xi′an Jiaotong University from April 2016 to April 2019 were enrolled.Demographic data, hemodynamic-related indexes and oxygen metabolism indexes on admission were collected, and 28-day mortality was our primary outcome.According to the prognosis, the children were divided into survival group and death group.Baseline data, hemodynamic-related indexes and oxygen metabolism indexes on admission were compared between two groups.Binary Logistic regression was used to analyze the risk factors that may affect the prognosis, and relevant risk factors were analyzed by the receiver operator characteristic(ROC)curve to verify the predictability in prognosis.Results:A total of 170 children with severe sepsis were selected, including 79 died in 28-days with a 28-day mortality of 46.47%.There were no statistically significant differences in age, gender( P>0.05). Compared with survival group, the bloodstream infection and intracranial infection incidence were increased in the death group(all P<0.05). Compared with the survival group, blood lactate(Lac) and oxygen extraction ratio(ERO 2) were all increased in the death group[Lac: (7.58±2.64)mmol/L vs.(3.14±1.16) mmol/L, ERO 2: (45.12±11.39)% vs.(32.19±6.24)%, all P<0.05]; Oxygenation index(PO 2/FiO 2), mean arterial pressure(MAP), saturation of arterial blood oxygen(SaO 2), saturation of venous blood oxygen(SvO 2), cardiac index(CI) were all decreased[ PO 2/FiO 2: (237.75±130.37)mmHg vs.(319.25±150.85) mmHg, 1 mmHg=0.133 kPa; MAP: (49±4)mmHg vs.(61±15) mmHg; SaO 2: (62.29±15.16)%vs.(83.21±16.09) %; SvO 2: (57.28±24.02)% vs.(65.32±13.15) %; CI: (1.68±0.76)mL/(min·m 2) vs.(2.56±0.25) mL/(min·m 2), all P<0.05]. The binary Logistic regression showed that Lac and ERO 2 were independent risk factors affecting the prognosis of children with severe sepsis, and the difference was statistically significant[ OR(95% CI) were 2.00(1.14-3.51)and 1.83(1.09-4.05), respectively, all P<0.05]. ROC curve analysis showed that the area under ROC curve of Lac and ERO 2 were 0.675 and 0.789, respectively.Sensitivity to predict death in children with severe sepsis were 93.75% and 85.31%, respectively, whose specificity were 87.85% and 78.39%, respectively.The combined prediction area under ROC curve of Lac and ERO 2 was 0.946, with a sensitivity of 89.15% and specificity of 88.76%, and the differences were statistically significant(all P<0.05). Conclusion:Lac and ERO 2 are independent risk factors affecting children with severe sepsis, and their combination has a good predictive value for the prognosis of children with severe sepsis.

Objective To investigate the relationship between the incidence of deep vein thrombosis (DVT) during hospitalization and the energy of injury in tibial plateau fractures (TPFs).Methods 140 patients were enrolled in the study between September,2014,and October,2017 in Honghui Hospital,Xi'an Jiaotong University.According to the Schatzker classification,the low-energy group (Schatzker Ⅰ-Ⅲ) X-ray showed a simple or lateral fracture or compression fracture of the lateral plateau (n =63);the high-energy group (Schatzker Ⅳ-Ⅵ) X-ray showed the medial tibia or double of the tibial plateau (n =77).Duplex Ultrasonography was performed in lower extremities before and after surgical intervention for DVT evaluation and record the incidence of DVT,the days during hospitalization,the blood loss during surgery and the level of D-Dimer.All patients received mechanical and chemical thromboprophylaxis.The patient was followed up for one month after surgery,and the lower extremity ultrasound was reviewed and recorded.The measurement data were expressed as ((x) ± s),and the categorical variables were expressed in composition ratio.The measurement data were all in accordance with the normal distribution and the variance was uniform.The t-test was used,and the Chi-square test was used for comparison between groups.Results The incidence of thrombosis of tibial plateau fracture in TPFs was 36.43％ and 46.43％ pre-operation and post-operation respectively.The thrombosis incidence was 31.75 ％ (20/63) in the low-energy group and 40.26％ (31/77) in the high-energy group pre-operation.The thrombosis incidence was 44.44％ (28/63) in the low-energy group and 48.05％ (37/77) in the high-energy group post-operation.Statistical analysis showed no significant difference between the two groups pre-operation (P =0.298) and post-operation(P =0.785).The days during hospitalization [(11.94 ± 4.18) d,(9.56 ± 2.54) d],the blood loss during surgery [(208.96 ±224.43) ml,(129.68 ± 142.14) ml] and the level of D-Dimer [(2.39 ± 3.38) mg/L,(1.21 ± 2.32) mg/L] at pre-operation showed differences in the high energy group and the low energy group,respectively.Conclusions The incidence of DVT in TPFs is high during the hospital,but most of DVT is concentrated in the distal to the knee.Although the incidence of high energy injury and low energy injury DVT is not significantly different in TPFs,we should also be alert to the occurrence of DVT.

Objective:To investigate the lymphocyte subsets and clinical characteristics of children with abnormal reaction to Bacillus Calmette-Guérin(BCG)vaccination.Methods:A total of 35 children with BCG disease diagnosed in the Children′s Hospital Affiliated to Xi′an Jiaotong University from January 2013 to December 2019 were enrolled retrospectively.Patients with strong local reaction and lymphadenitis after vaccine injection were selected as the localized group, and with lymphadenitis complicated with distant organ involvement were classified as the disseminated group.The differences in clinical infection indicators, demographic data, lymphocyte subsets and prognosis between the two groups were compared.Results:There are 25 cases in the localized group and 10 cases in the disseminated group, male 20 cases and female 15 cases.Compared with the localized group, the incidence of cough, fever and growth retardation all increased in the disseminated group, with statistical significance(all P<0.05). Lymphocyte ratio[(61.14±18.61)% vs.(39.64±31.45)%], T lymphocytes [CD3 + (×10 6/L): (1 821±487)vs.(1 065±539)], helper/inducible T lymphocytes[CD3 + CD4 + (×10 6/L): (1 058±357)vs.(445±140)], double positive T lymphocytes[CD3 + CD4 + CD8 + (×10 6/L): (24.07±7.17)vs.(14.10±8.89)], CD4 + /CD8 + ratio[CD4 + /CD8 + (%): (1.65±0.73)vs.(1.00±0.25)], natural killer cells[CD16 + CD56 + (×10 6/L): (19.70±2.34)vs.(12.76±7.01)]were lower in the disseminated group than those in the localized group and the differences were significant(all P<0.05). In the disseminated group, 6 cases were diagnosed with immunodeficiency disease and 7 cases died during the follow-up period.All the children in the localized group were cured. Conclusion:Most BCG reaction have a good prognosis, while disseminated children combined with primary immune deficiency have worst prognosis.Early lymphocyte subsets analysis is effective for BCG disease screening.

OBJECTIVE: To explore the genetic basis for a child with optic atrophy and global developmental delay. METHODS: A child who had presented at the Guangzhou Women and Children's Medical Center in January 2022 was selected as the study subject. Clinical data were collected. Whole exome sequencing (WES) was carried out for the child. Candidate variant was validated by Sanger sequencing and bioinformatic analysis. RESULTS: The child, a nine-month-old female, had manifested dysopia and global developmental delay. Genetic testing revealed that she has harbored a de novo c.425G>C (p.Arg142Pro) variant of the NR2F1 gene, which has been associated with Bosch-Boonstra-Schaaf syndrome. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), the variant was classified as pathogenic (PS2+PM1+PM2_Supporting+PM5+PP3+PP4). CONCLUSION The c.425G>C (p.Arg142Pro) variant of the NR2F1 gene probably underlay the pathogenesis in this child. Above finding has enriched the genotypic and phenotypic spectrum of the NR2F1 gene.
Female ; Humans ; Infant ; Computational Biology ; COUP Transcription Factor I/genetics* ; Genetic Testing ; Genomics ; Genotype ; Optic Atrophy/genetics*

Objective:A clinical prediction model was constructed based on the related factors affecting neonatal early-onset sepsis (EOS).Methods:A retrospective study was conducted. The patients with EOS amditted to the neonatal intensive care unit of Children′s Hospital Affiliated to Xi′an Jiaotong University from April 2015 to April 2020 were enrolled. The demographic data and the clinical indicators within 8 hours after admission were collected. The death 7 days after admission was taken as the end event. The differences of various indexes between the survival group and the death group were compared. After univariate analysis of the indexes that may have an impact on the prognosis, binary logistic regression analysis was performed; The predictive model was established for the factors that may affect the prognosis; the predictive value of the relevant models was analyzed by recevier operating characteristic (ROC) curve, and the model was verified by independent clinical medical records.Results:A total of 139 children were enrolled, and 41 died within 7 days, with a fatality rate of 29.50%. Compared with the survival group, the dead group had higher white blood cells (WBC), serum procalcitonin (PCT), lactic acid (Lac), creatinine (Scr), D-dimer and Paediatric Risk of Mortality (PRISM) score {WBC(×10 9/L): 24.15[4.36, 29.36] vs 21.21[19.14, 28.36], PCT: (67.32±40.36)ng/L vs (37.76±25.11)ng/L, Lac: (8.69±6.17)mmol/L vs (2.34±1.11)mmol/L, Scr: (239.99±68.46)μmol/L vs (65.31±34.34)μmol/L, D-dimer(mg/L): 5.21[2.06, 21.49] vs 0.34[0.26, 0.45], PRISM Ⅲ: (19.52±6.25)s vs (10.63±2.05)s, all P<0.05}, and lower fibrinogen (Fib), platelet count (PLT) and hemoglobin concentration (Hb) [Fib: (1.48±1.19)g/L vs (2.44±0.83)g/L, PLT: (154±58)×10 9/L vs (189±29)×10 9/L, Hb: (169±49)g/L vs (182±52)g/L, all P<0.05]. The incidence of placental/umbilical cord lesions, amniotic fluid pollution, asphyxia, premature delivery, premature rupture of membranes, positive etiology and maternal infection in the death group were higher than those in the survival group, while the gestational age and weight were lower than those in the survival group (all P<0.05); Binary logistic regression analysis showed that Lac, PCT and premature rupture of membranes were independent risk factors for the prognosis of EOS [odds ratio ( OR) and 95% confidence interval (95% CI): Lac was 1.23(1.00-2.05), PCT was 1.05(1.03-1.85), premature rupture of membranes was 2.59(1.89-3.32), all P<0.05]; ROC curve analysis showed that the area under the curve (AUC) of the prediction model was 0.967; the predicted sensitivity was 88.70%; and the specificity was 78.20% respectively. Conclusions:PCT, Lac and premature rupture of membranes are independent risk factors affecting the prognosis of EOS. The clinical prognosis prediction model constructed by combining PLT, gestational age and weight has good prediction efficiency.

Objective:To explore the effectiveness and safety of plasma exchange in the treatment of neonatal extremely severe hyperbilirubinemia.Methods:A retrospective analysis was performed on the data of 18 cases, who were all newborns with extremely severe hyperbilirubinemia and treated with plasma exchange in the NICU at Xi ′an Children′s Hospital from April 2019 to December 2019.The changes of serum total bilirubin, indirect bilirubin, albumin, white blood cells, red blood cells, platelets, hematocrit, hemoglobin, serum sodium, serum potassium, serum calcium, blood glucose, blood coagulation and mean arterial pressure were compared before and after plasma exchange.Results:A total of 18 eligible children were included.The peak value of total bilirubin was (571.2±113.3) μmol/L before treatment, and the value after treatment was (235.8±66.7) μmol/L, whose difference was statistically significant ( P<0.05). The exchange rate of bilirubin was (58.5±8.4)%.There were no statistically significant differences in the changes of white blood cells, platelets, hemoglobin, hematocrit, serum sodium, serum potassium, serum chloride, serum calcium, serum glucose, and albumin before and after plasma exchange (all P>0.05). There were no allergic reactions, hypotension, plasma separator or pipeline coagulation and other adverse reactions during plasma exchange. Conclusion:Plasma exchange therapy can remove serum bilirubin quickly, effectively and safely, and may be a new treatment for neonatal hyperbilirubinemia.

Objective To evaluate the feasibility and safety of laparoscopic choledochoscopy for common bile duct exploration in patients of common bile duct stones with a history of previous abdominal surgery.Methods From March 2015 to May 2016,100 cases were divided into laparoscopic and open common bile duct exploration in our department.Hospital stay,costs and complications were compared.Results The blood loss,cost,fasting time and hospital stay in the laparoscopy group were less than those in the open group.There were no significant differences in alanine aminotransferase,total bilirubin,albumin,prothrombin time,leukocyte,C-reactive protein,operative time and complications between the two groups.Conclusion Laparoscopic choledochoscopy for common bile duct exploration is an effective and safe method for the treatment of common bile duct stones in patients with previous history of abdominal surgery.

Objective:To investigate the prognostic value of the ratio of veno-arterial carbon dioxide partial pressure difference to arterio-venous oxygen content difference (Pv-aCO 2/Ca-vO 2) in children with primary peritonitis-related septic shock. Methods:A retrospective study was conducted. Sixty-three children with primary peritonitis-related septic shock admitted to department of intensive care unit of the Children's Hospital Affiliated to Xi'an Jiaotong University from December 2016 to December 2021 were enrolled. The 28-day all-cause mortality was the primary endpoint event. The children were divided into survival group and death group according to the prognosis. The baseline data, blood gas analysis, blood routine, coagulation, inflammatory status, critical score and other related clinical data of the two groups were statistics. The factors affecting the prognosis were analyzed by binary Logistic regression, and the predictability of risk factors were tested by the receiver operator characteristic curve (ROC curve). The risk factors were stratified according to the cut-off, Kaplan-Meier survival curve analysis compared the prognostic differences between the groups.Results:A total of 63 children were enrolled, including 30 males and 33 females, the average age (5.6±4.0) years old, 16 cases died in 28 days, with mortality was 25.4%. There were no significant differences in gender, age, body weight and pathogen distribution between the two groups. The proportion of mechanical ventilation, surgical intervention, vasoactive drug application, and procalcitonin, C-reactive protein, activated partial thromboplastin time, serum lactate (Lac), Pv-aCO 2/Ca-vO 2, pediatric sequential organ failure assessment, pediatric risk of mortality Ⅲ in the death group were higher than those in the survival group. Platelet count, fibrinogen, mean arterial pressure were lower than those in the survival group, and the differences were statistically significant. Binary Logistic regression analysis showed that Lac and Pv-aCO 2/Ca-vO 2 were independent risk factors affecting the prognosis of children [odds ratio ( OR) and 95% confidence interval (95% CI) were 2.01 (1.15-3.21), 2.37 (1.41-3.22), respectively, both P < 0.01]. ROC curve analysis showed that the area under curve (AUC) of Lac, Pv-aCO 2/Ca-vO 2 and their combination were 0.745, 0.876 and 0.923, the sensitivity were 75%, 85% and 88%, and the specificity were 71%, 87% and 91%, respectively. Risk factors were stratified according to cut-off, and Kaplan-Meier survival curve analysis showed that the 28-day cumulative probability of survival of Lac ≥ 4 mmol/L group was lower than that in Lac < 4 mmol/L group [64.29% (18/28) vs. 82.86% (29/35), P < 0.05]. Pv-aCO 2/Ca-vO 2 ≥ 1.6 group 28-day cumulative probability of survival was less than Pv-aCO 2/Ca-vO 2 < 1.6 group [62.07% (18/29) vs. 85.29% (29/34), P < 0.01]. After a hierarchical combination of the two sets of indicator variables, the 28-day cumulative probability of survival of Pv-aCO 2/Ca-vO 2 ≥ 1.6 and Lac ≥ 4 mmol/L group significantly lower than that of the other three groups (Log-rank test, χ2 = 7.910, P = 0.017). Conclusion:Pv-aCO 2/Ca-vO 2 combined with Lac has a good predictive value for the prognosis of children with peritonitis-related septic shock.

OBJECTIVE: To explore the genetic basis for a child featuring global developmental disorder with epilepsy. METHODS: A child who had presented at Guangzhou Women and Children's Medical Center in July 2022 was selected as the study subject. Clinical data was collected. Potential variant was detected by whole exome sequencing (WES). Candidate variant was validated by Sanger sequencing and bioinformatic analysis. RESULTS: The child, a three-year-old ethnic Zhuang Chinese girl, had presented with global developmental disorder and epilepsy, for which rehabilitation therapy was ineffective. Genetic testing revealed that she has harbored a homozygous c.821T>C (p.Leu274Pro) missense variant of the PIGW gene, for which both of her parents and sister were heterozygous carriers. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), the variant was classified as variant of uncertain significance. CONCLUSION The homozygous c.821T>C (p.Leu274Pro) variant of the PIGW gene probably underlay the onset of disease in this child. Above finding has enriched the mutational spectrum of the PIGW gene.
Child, Preschool ; Female ; Humans ; Computational Biology ; Developmental Disabilities ; Epilepsy/genetics* ; Genetic Testing ; Homozygote

Hepatocellular carcinoma(HCC)is one of the most common malignancies with high morbidity and mortality rates worldwide.With the advances in molecular biology and tumor immunology,molecular-targeted agents represented by tyrosine kinase inhibitors(such as sorafenib and lenvatinib)and immunotherapy represented by PD-1/PD-L1 monoclonal antibodies have brought hope for patients with advanced HCC.The combination of immunotherapy and anti-angiogenic therapy can further improve the treatment outcome of patients.In addition,the optimization and integration of stereotactic body radiotherapy,local treatment,and systemic treatment may maximize the benefits of patients.In the future,through a deep understanding of the heterogeneity of HCC,the development of precision molecular subtyping and individualized treatment,and the establishment of a multidisciplinary collaborative diagnosis and treatment system,systemic therapy is expected to achieve long-term management of advanced HCC.This article reviews the current status and advances in systemic therapy for advanced HCC.