OBJECTIVE To analyze the influential factors for clinical efficacy of polymyxin B combined with other antibiotics in the treatment of carbapenem-resistant Acinetobacter baumannii （CRAB） pulmonary infection. METHODS A retrospective analysis was conducted on the clinical data of patients with CRAB pulmonary infection in our hospital from May 2021 to October 2024. Information such as age， gender， admitting department， infection status， underlying medical conditions， mechanical ventilation time， combination anti-infective treatment regimens， and the Acute Physiology and Chronic Health Evaluation Ⅱ （APACHE-Ⅱ） score 24 h before medication was compiled. Based on the effectiveness of the treatment， patients were divided into treatment-effective group and treatment-ineffective group. Univariate analysis and multivariate Logistic regression analysis were employed to identify independent factors influencing clinical efficacy. RESULTS A total of 156 patients were included， and 108 patients were treated effectively， with an effective rate of 69.23%. The results of univariate analysis indicated that there were statistically significant differences between 2 groups in terms of the duration of mechanical ventilation time， APACHE-Ⅱ score 24 h before medication， the number of complication types， the proportion of abnormal coagulation function， anti-infective treatment course， and hospital stay before medication （P＜0.05）. The results of multivariate Logistic regression analysis showed that APACHE-Ⅱ score≥15 points 24 h before medication [OR＝2.965， 95%CI （1.284， 6.845）， P＝0.020]， mechanical 20251606） ventilation time≥10 d [OR＝3.577， 95%CI （1.185， 10.793）， P＝0.037] and hospital stay≥14 d before medication [OR＝2.422， 95%CI （1.036， 5.654）， P＝0.041] were independent 15120420253@139.com risk factors， and anti-infective treatment course＞7 d was a protective factor [OR＝0.445， 95%CI （0.221， 0.895）， P＝0.043]. CONCLUSIONS This study shows that the effective rate of polymyxin B combined with other antibiotics in the treatment of CRAB pulmonary infection is less than 70%. The mechanical ventilation time≥10 d， APACHE-Ⅱ score≥15 points 24 h before medication， and hospital stay≥14 d before medication may lead to treatment failure， whereas anti-infective treatment course＞7 d may be associated with treatment success.

Evidence-based medicine (EBM) is a science that uses the best available research data to make decisions, and the core is that clinical decision-making is supported by the best research evidence. Incorporating EBM into traditional standardized residency training in hematology can foster residents' professional theoretical knowledge and clinical skills, improve the quality of standardized training, and provide ideas and methods for standardized training of hematology residents, which is worthy of further research and exploration.

Objective:To evaluate the impact of cold-air anesthesia on patients′ comfort and side effects during ablative fractional carbon-dioxide (CO 2) laser treatment for acnescars. Methods:Eighteen patients with atrophic acne scars treated with CO 2 laser were used as split-face comparative study with a cold-air device combined with 5% lidocaine cream on the left side of the face, and the right side as a control group treated with 5% lidocaine cream only. Symptoms and signs of patients were observed during treatment, immediately after treatment, 15 minutes, 30 minutes and 1 month after treatment. The score of patient′s comfort and incidence of adverse events was evaluated. Results:The results were obvious difference between each group. The surface temperature in combined group (33.1±1.3) showed significantly better results compared with other groups (36.9±0.4) during the operative period while the same result after the treatment immediately shown in the combined group (35.1±1.3) compared with other groups (36.4±0.2). The score of erythema shown that combined group (48.7±8.4) was significantly lower than that of the control group (54.8±8.0) ( P<0.05) as the score of exudation in combined group (6.2±1.6) was lower than that in the control group (7.8±1.6). There was significant statistical difference regarding scores of erythema between combined group (46.9±6.6) and control group (51.8±6.9) a month after the treatment. The pigmentation score revealed the same result that the combined group (48.9±8.8) was lower than the other groups (51.4±7.0). No patients had severe adverse events. Conclusions:The cold-air cooling application is safe and effective to reduce the surface temperature, pain and side effects during fractional carbon-dioxide laser treatment.

Objective:To observe the clinical effect of anal fistula incision, muscle layer suture, and internal drainage surgery in the treatment of low anal fistula.Methods:A retrospective study was conducted on 65 patients with low anal fistula admitted to Fifth Hospital of Zhangjiakou City From March 2021 to March 2023, and they were divided into two groups based on different surgical methods. The control group consisted of 32 patients who underwent traditional anal fistula incision and thread drainage, while the study group consisted of 33 patients who underwent anal fistula incision and muscle layer suture internal drainage. The duration of surgery, wound recovery, anal function, anal motility, degree of pain, and recurrence rate were compared.Results:The surgical time of the study group was longer than that of the control group: (34.86 ± 4.17) min vs. (25.21 ± 3.95) min ( P<0.05), the wound healing time was shorter than that of the control group: (15.72 ± 3.26) d vs. (18.94 ± 4.42) d ( P<0.05), and the wound area was smaller than that of the control group: (5.76 ± 1.72) cm 2 vs. (7.78 ± 1.89) cm 2 ( P<0.05). At 4 weeks after surgery, the Wexner scores in both groups were lower than those at 1 d after surgery ( P<0.05), and the study group was lower than the control group: (3.39 ± 1.08) scores vs. (4.15 ± 1.21) scores ( P<0.05). After surgery, the high pressure zone length, anal rest pressure and anal maximum contraction pressure in the control group were lower than before surgery ( P<0.05), and the study group were higher than the control group: (4.92 ± 1.11) cm vs. (3.64 ± 1.02) cm, (11.93 ± 1.22) kPa vs. (9.45 ± 1.08) kPa, (13.34 ± 3.42) kPa vs. (10.82 ± 2.75) kPa ( P<0.05). At 48 h after surgery, the visual analogue scale (VAS) scores of both groups were lower than those at 12 h after surgery ( P<0.05), and the study group was lower than the control group: (1.63 ± 0.21) scores vs. (2.25 ± 0.34) scores ( P<0.05). After 6 months of follow-up, neither group experienced any recurrence. Conclusions:Anal fistula incision, muscle layer suture and internal drainage surgery for low anal fistula can shorten the wound healing time, reduce the wound area, improve anal function and anal motility, alleviate pain, and avoid recurrence. However, the surgical time is longer.

Objective:To further investigate the safety and efficacy of Belimumab in the treatment of patients with systemic lupus erythematosus (SLE).Methods:All SLE patients treated with Belimumab from May 1, 2020 to February 1, 2022 in Peking Union Medical College Hospital were retrospectively collected and analyzed. The clinical manifestations, the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2000) score, and laboratory test such as levels of anti-dsDNA antibody, the medication before and after Belimumab treatment, adverse events were collected. Normally distributed data were tested using the t-test, otherwise the Wilcoxon paired signed rank test was used. Results:A total of 81 patients were enrolled in this study. The use of belimumab could significantly decrease the SLEDAI-2000 score [10.00(7.75, 12.00) vs. 4.00(3.75, 6.00), Z=-5.38, P<0.001], ESR of SLE patients [19.50(12.75, 32.25) mm/1 h vs. 14.00(7.75, 20.25) mm/1 h, Z=-3.71, P=0.003], anti-dsDNA titer detected by CLIFT [300.00 (117.00, 864.00) vs. 183.00(100.00, 471.00), Z=-4.15, P=0.001], meanwhile, increase the complement C3 [0.78 (0.62, 0.97)g/L vs. 0.69 (0.55, 0.84)g/L, Z=-4.68, P<0.001], and the complement C4 [0.12 (0.08, 0.19)g/L vs. 0.10 (0.05, 0.14)g/L, Z=-4.78, P<0.001]. We also observed that with the use of Belimumab, the dosage of Glucocorticoids decreased significantly, which were [10.00(7.50, 22.50) mg vs. 7.50(5.00, 10.00) mg, Z=-4.90, P<0.001]. In addition, the antibody of IgG, IgA and IgM decreased significantly. Only one patient stopped the administration of Belimumab due to the low level of immunoglobulin. Conclusion:Belimumab can alleviate disease activity of patients with SLE and help in safely tapering the daily dose of glucocorticoid with good safety.

Mitochondrial and peroxisome fission deficiency-related encephalopathy caused by DNM1L gene mutation is a rare and fatal epileptic encephalopathy, with clinical phenotype and genetic heterogeneity. The acute stage is drug-resistant epilepsy with poor prognosis and serious neurological sequelae. A case of genetically confirmed encephalopathy related to mitochondrial and peroxisome fission defects is reported, the clinical data, treatment process are summarized, and the previous literature is reviewed to improve the understanding of the rare disease.

Objective:To investigate the relationship between intra-abdominal pressure (IAP) and respiratory function in mechanically ventilated children with severe pneumonia and its prognostic value.Methods:A case-series study.Ninety-two children with severe pneumonia who were treated with mechanical ventilation in the Intensive Care Unit of Children′s Hospital Affiliated to Zhengzhou University from May 2019 to May 2021 were taken as the study subjects.The IAP and respiratory function-related indexes of the children were monitored.Those with IAP ≥12 mmHg(1 mmHg=0.133 kPa) were divided into the study group, and those with IAP <12 mmHg were divided into the control group.The difference in respiratory function related indexes between the 2 groups was compared, and the relationship between IAP and respiratory function indexes was analyzed by Pearson correlation.The children were divided into a good prognosis group and a poor prognosis group according to their prognosis.The relevant clinical data of the children in the 2 groups were collected for univariate analysis, and multivariate Logistic regression was used to analyze the risk factors affecting the prognosis of children with mechanical ventilation for severe pneumonia.A receiver operating characteristic (ROC) curve was drawn to analyze the predictive value of IAP on the prognosis of mechanically ventilated children with severe pneumonia.Results:The arterial partial pressure of oxygen (PaO 2) [(80.15±8.23) mmHg], arterial blood oxygen saturation (SaO 2) [(91.32±2.13)%], and oxygenation index (PaO 2/FiO 2) [(198.29±20.25) mmHg] in the study group were lower than those in the control group [(85.74±8.42) mmHg, (97.05±2.47)%, and (234.84±25.24) mmHg], while the respiratory rate (RR) [(56.23±2.16) breaths/min vs. (50.41±2.24) breaths/min], partial pressure of carbon dioxide (PaCO 2) [(36.48±3.72) mmHg vs.(33.29±3.46) mmHg], fraction of inspired oxygen (FiO 2) [(40.42±4.25)% vs.(36.51±3.72)%], mean arterial pressure (MAP) [(55.98±5.69) mmHg vs.(52.79±5.43) mmHg], and positive end expiratory pressure (PEEP) [(5.03±0.52) cmH 2O vs.(4.52±0.47) cmH 2O, 1 cmH 2O=0.098 kPa] were higher than those in the control group, and the differences were all statistically significant (all P<0.05).The IAP of the children in the study group was (14.25±1.83) mmHg, and that in the control group was (10.38±1.14) mmHg.Pearson correlation analysis showed that IAP was negatively correlated with PaO 2, SaO 2, and PaO 2/FiO 2 ( r=-0.615, -0.587, and -0.647, all P<0.05), and was positively correlated with RR, PaCO 2, FiO 2, MAP, and PEEP ( r=0.618, 0.634, 0.579, 0.578, 0.593, all P<0.05).IAP [(14.76±1.58) mmHg], PaCO 2 [(39.95±4.21) mmHg], FiO 2 [(50.29±5.12)%], alanine aminotransferase[(42.08±4.15) U/L], and total bilirubin [(17.92±1.87) μmol/L] in the poor prognosis group were greater than those [(10.75±1.19) mmHg, (35.37±3.64) mmHg, (45.38±4.47)%, (39.87±4.06) U/L, and (17.09±1.75) μmol/L] in the good prognosis group, while PaO 2 [(65.42±7.86) mmHg vs.(76.42±7.51) mmHg], SaO 2 [(90.65±9.26)% vs.(96.21±2.19)%], and PaO 2/FiO 2 [(130.09±15.15) mmHg vs.(168.40±20.17) mmHg] were smaller than those in the good prognosis group, and the differences were statistically significant (all P<0.05).Logistic regression analysis showed that IAP, PaO 2, PaCO 2, and SaO 2 were risk factors affecting the prognosis of mechanically ventilated children with severe pneumonia (all P<0.05).The optimal cut-off point of IAP for predicting a poor prognosis in mechanically ventilated children with severe pneumonia was 14.55 mmHg, and the area under the ROC curve was 0.873, with a sensitivity of 88.10% and a specificity of 76.00%. Conclusions:IAP has a certain relationship with the respiratory function of mechanically ventilated children with severe pneumonia, and has a good predictive value for the prognosis of the children.

Objective To investigate the MRI characteristics of subventricular zone(SVZ)-associated isocitrate dehydrogenase(IDH)wild-type glioblastoma(GBM)and their correlations with Ki-67 expression and O6-methylguanine-DNA methyltransferase(MGMT)promoter methylation status.Methods A retrospective analysis was conducted on data of 78 patients with IDH wild-type GBM who underwent surgery and received pathological confirmation.Preoperative MRI contrast-enhanced T1 WI sequences were used to assess SVZ involvement,and postoperative molecular testing of tumor markers,including Ki-67 expression and MGMT methylation status,was utilized to categorize the patients accordingly.Results The SVZ involved(+)group(P＜0.001)and the MGMT(+)group(P=0.036)exhibited significantly larger tumor volumes.There were no significant differences between the groups in terms of gender,age,left/right hemispheric lateralization,or specific brain lobe distribution.There was no significant association between Ki-67 expression levels,MGMT methylation status,and SVZ involvement,respectively.Conclusion The SVZ(+)group and the MGMT(+)group demonstrates a wider range of tumor invasion.

Objective:To identify the genetic variation in a mucopolysaccharidosis type Ⅱ(MPS Ⅱ)family, and conduct a functional study of iduronate-2-sulfatase(IDS): c.323A>C.Methods:A five-generation MPS Ⅱ family of 83 individuals including 4 patients from northern China was collected. Urine mucopolysaccharide and Alder-Reilly body were tested to assist the clinical diagnosis of MPS Ⅱ. IDS enzyme activity was detected on core family members. By the whole exome sequencing of a MPS Ⅱ patient in this family and bioinformatics analysis, the variant was screened and further identified by PCR-Sanger sequencing. Finally, to validate the function of the variant in vitro, the wild-type IDS overexpression plasmid(pCMV-hIDS-WT)and the IDS overexpression plasmid carrying the mutation site(pCMV-hIDS-c.323A>C)were transfected into COS-7 cells and the IDS activity was detected. Results:The proband(Ⅳ3)and Ⅳ4 were diagnosed as MPS Ⅱ by urine mucopolysaccharide, Alder-Reilly body, and IDS enzyme activity tests. Ⅳ3, Ⅳ4, Ⅲ19, and Ⅲ32 were determined to carry IDS: c.323A>C missense variant through the whole-exome sequencing, and diagnosed as MPS Ⅱ. Meanwhile, Ⅱ2, Ⅱ4, Ⅱ8, Ⅱ12, Ⅱ14, Ⅲ5, Ⅲ7, Ⅳ14 in the MPS Ⅱ family carried IDS: c.323A>C missense variant, and were excluded as MPS Ⅱ. The in vitro experiment in COS-7 cells showed that the missense mutation led to a significant decrease in IDS enzyme activity. Conclusion:The variant IDS: c.323A>C: p.Y108S significantly decreases the activity of IDS enzyme in vivo and in vitro, and it is identified as a pathogenic variant for MPS Ⅱ.

OBJECTIVE: To analyze the characteristics of genetic variants among children with refractory epilepsy (RE). METHODS: One hundred and seventeen children with RE who had presented at the Affiliated Jinhua Hospital of Zhejiang University School of Medicine from January 1, 2018 to November 21, 2019 were selected as the study subjects. The children were divided into four groups according to their ages of onset: < 1 year old, 1 ~ 3 years old, 3 ~ 12 years old, and >= 12 years old. Clinical data and results of trio-whole exome sequencing were retrospectively analyzed. RESULTS: In total 67 males and 50 females were included. The age of onset had ranged from 4 days to 14 years old. Among the 117 patients, 33 (28.21%) had carried pathogenic or likely pathogenic variants. The detection rates for the < 1 year old, 1 ~ 3 years old and >= 3 years old groups were 53.85% (21/39), 12.00% (3/25) and 16.98% (9/53), respectively, with a significant difference among the groups (χ² = 19.202, P < 0.001). The detection rates for patients with and without comorbidities were 33.33% (12/36) and 25.93% (21/81), respectively (χ² = 0.359, P = 0.549). Among the 33 patients carrying genetic variants, 27 were single nucleotide polymorphisms (SNPs) or insertion/deletions (InDels), and 6 were copy number variations (CNVs). The most common mutant genes were PRRT2 (15.15%, 5/33) and SCN1A (12.12%, 4/33). Among children carrying genetic variants, 72.73% (8/11) had attained clinical remission after adjusting the medication according to the references. CONCLUSION 28.21% of RE patients have harbored pathogenic or likely pathogenic variants or CNVs. The detection rate is higher in those with younger age of onset. PRRT2 and SCN1A genes are more commonly involved. Adjusting medication based on the types of affected genes may facilitate improvement of the remission rate.
Infant ; Female ; Male ; Humans ; Child ; Infant, Newborn ; Child, Preschool ; DNA Copy Number Variations ; Drug Resistant Epilepsy/genetics* ; Retrospective Studies ; Polymorphism, Single Nucleotide