BACKGROUND: Congenital heart disease (CHD) is a leading cause of birth defect-related mortality. However, more recent CHD mortality data for China are lacking. Additionally, limited studies have evaluated sex, rural-urban, and region-specific disparities of CHD mortality in China. METHODS: We designed a population-based study using data from the Dataset of National Mortality Surveillance in China between 2008 and 2021. We calculated age-adjusted CHD mortality using the sixth census data of China in 2010 as the standard population. We assessed the temporal trends in CHD mortality by age, sex, area, and region from 2008 to 2021 using the joinpoint regression model. RESULTS: From 2008 to 2021, 33,534 deaths were attributed to CHD. The period witnessed a two-fold decrease in the age-adjusted CHD mortality from 1.61 to 0.76 per 100,000 persons (average annual percent change [AAPC] = -5.90%). Females tended to have lower age-adjusted CHD mortality than males, but with a similar decline rate from 2008 to 2021 (females: AAPC = -6.15%; males: AAPC = -5.84%). Similar AAPC values were observed among people living in urban (AAPC = -6.64%) and rural (AAPC = -6.12%) areas. Eastern regions experienced a more pronounced decrease in the age-adjusted CHD mortality (AAPC = -7.86%) than central (AAPC = -5.83%) and western regions (AAPC = -3.71%) between 2008 and 2021. Approximately half of the deaths (46.19%) due to CHD occurred during infancy. The CHD mortality rates in 2021 were lower than those in 2008 for people aged 0-39 years, with the largest decrease observed among children aged 1-4 years (AAPC = -8.26%), followed by infants (AAPC = -7.01%). CONCLUSIONS CHD mortality in China has dramatically decreased from 2008 to 2021. The slower decrease in CHD mortality in the central and western regions than in the eastern regions suggested that public health policymakers should pay more attention to health resources and health education for central and western regions.
Humans ; Heart Defects, Congenital/mortality* ; Male ; Female ; China/epidemiology* ; Infant ; Child, Preschool ; Adult ; Child ; Adolescent ; Infant, Newborn ; Middle Aged ; Young Adult ; Aged ; Rural Population

Metachromatic leukodystrophy (MLD) is an inherited disease caused by a deficiency of the enzyme arylsulfatase A (ARSA). Lentivirus-modified autologous hematopoietic stem cell gene therapy (HSCGT) has recently been approved for clinical use in pre and early symptomatic children with MLD to increase ARSA activity. Unfortunately, this advanced therapy is not available for most patients with MLD who have progressed to more advanced symptomatic stages at diagnosis. Patients with late-onset juvenile MLD typically present with a slower neurological progression of symptoms and represent a significant burden to the economy and healthcare system, whereas those with early onset infantile MLD die within a few years of symptom onset. We conducted a pilot study to determine the safety and benefit of HSCGT in patients with postsymptomatic juvenile MLD and report preliminary results. The safety profile of HSCGT was favorable in this long-term follow-up over 9 years. The most common adverse events (AEs) within 2 months of HSCGT were related to busulfan conditioning, and all AEs resolved. No HSCGT-related AEs and no evidence of distorted hematopoietic differentiation during long-term follow-up for up to 9.6 years. Importantly, to date, patients have maintained remarkably improved ARSA activity with a stable disease state, including increased Functional Independence Measure (FIM) score and decreased magnetic resonance imaging (MRI) lesion score. This long-term follow-up pilot study suggests that HSCGT is safe and provides clinical benefit to patients with postsymptomatic juvenile MLD.
Humans ; Leukodystrophy, Metachromatic/genetics* ; Pilot Projects ; Genetic Therapy/methods* ; Hematopoietic Stem Cell Transplantation ; Male ; Follow-Up Studies ; Female ; Lentivirus/genetics* ; Child ; Child, Preschool ; Hematopoietic Stem Cells/metabolism* ; Cerebroside-Sulfatase/metabolism* ; Adolescent

Objective:To construct a diagnostic prediction model for childhood asthma and conduct a preliminary evaluation based on the test results of specific IgE (sIgE) for airborne allergens and in combination with clinical data.Methods:This study is a case-control study. A total of 4 338 cases that completed the sIgE test for airborne allergens in the Allergy Department of Beijing Children′s Hospital Affiliated to Capital Medical University from January to December 2023 were selected as the research subjects. They were divided into the asthma group and the non-asthma group based on the diagnostic information. Age, gender, cough and wheezing symptoms, and the classification results of sIgE concentrations of 15 airborne allergens were collected as the predictor variables of the asthma diagnostic prediction model. Differential analysis and LASSO regression were employed for the screening of predictor variables. The multivariate logistic regression method was applied to construct the nomogram prediction model. The data set was randomly split at a ratio of 7∶3 into a training set (3 036 cases) for constructing the prediction model and a validation set (1 302 cases) for testing the predictive efficacy of the model. The area under the receiver operating characteristic (ROC) curve (AUC), the Hosmer-Lemeshow calibration curve were utilized to assess the discrimination and goodness of fit of the model, and the clinical decision curve (DCA) was adopted to evaluate the clinical application value of the model.Results:Among 4 338 pediatric cases, children aged 0 to <3 years accounted for 10.17% (441 cases), those aged 3 to <6 years accounted for 36.49% (1 583 cases), those aged 6 to <12 years accounted for 46.98% (2 038 cases), and those aged 12 to 18 years accounted for 6.36% (276 cases). Males constituted 65.17% (2 827 cases), and females 34.83% (1 511 cases). The proportion of children without wheezing symptoms was 41.47% (1 799 cases), while those with wheezing symptoms was 58.53% (2 539 cases). The asthma group accounted for 41.77% (1 812 cases), and the non-asthma group for 58.23% (2 526 cases). Statistically significant differences were observed between the asthma group and the non-asthma group in 18 predictive variables including age, gender, wheezing symptoms, d1, d2, e1, e5, g2, g6, m6, t11, t3, t6, w1, w22, w6, wx5, and m3 ( P<0.05). LASSO regression analysis identified six predictor variables: age (calculated in months), cough and wheezing symptoms, and sIgE of four airborne allergens, namely, Dermatophagoides pteronyssinus (d1), Canis familiaris dander (e5), Aspergillus fumigatus (m3), and Artemisia vulgaris pollen (w6).Multifactorial regression analysis revealed that the contribution degrees of the above-mentioned predictor variables to the asthma diagnosis prediction model were ranked as follows: cough and wheezing symptoms ( OR=24.37, P<0.001), m3 ( OR=1.34, P<0.001), d1 ( OR=1.22, P<0.001), e5 ( OR=1.12, P=0.028), w6 ( OR=1.11, P<0.001), and age ( OR=1.01, P<0.001).The AUCs of the nomogram prediction model for the training set and the validation set were 0.853 (95% CI: 0.840-0.866) and 0.838 (95% CI: 0.817-0.860), respectively. The Hosmer-Lemeshow calibration curve indicated a good fit ( P=0.215 for the training set; P=0.352 for the validation set). The DCA of the validation set demonstrated that when the probability threshold for predicting the occurrence of childhood asthma was 8%-92%, the model had the best applicability. Conclusion:By combining age, cough and wheezing symptoms, and sIgE of the four airborne allergens (d1, e5, m3, and w6) selected from 15 airborne allergens, a childhood asthma diagnosis prediction model with good predictive performance and clinical practicability was constructed. It can serve as a simple and convenient tool for accurately identifying asthma and provides a practical basis for the application of artificial intelligence big data analysis models in the prevention, treatment, and management of childhood asthma.

Objective:To analyze the distribution of allergen components of dust mite in children with airway allergic diseases.Methods:This was a cross-sectional study. The clinical data of children with dust mite-induced allergic asthma (AA) complicated with allergic rhinitis (AR) or allergic rhinitis who were treated in Department of Allergy,Beijing Children′s Hospital from January 2019 to October 2022 were retrospectively analyzed. The spedific IgE (sIgE) levels to Der p1,Der p2,Der p5,Der p7,Der p10,Der p21,Der p23 and Der f1,Der f2 were detected by protein chip method. The distribution of dust mite sensitized components and the sIgE levels in children with different airway allergic diseases and different ages were compared.Results:Among 138 children with airway allergic diseases,there were 97 boys and 41 girls,age (6.86±2.61) years old,and there were 106 cases of AA combined AR (AAAR group) and 32 cases of AR alone (AR group). The sensitization rates of Der p2 was the highest (75.4%,104/138),followed by Der f2 (74.6%,103/138),Der f1 (73.9%,102/138),Der p1 (71.7%,99/138),Der p21 (19.6%,27/138),Der p5 (16.7%,23/138),Der p23 (14.5%,20/138),Der p7 (11.6%,16/138) and Der p10 (2.9%,4/138). The co-sensitization rate of Der p1,Der p2,Der f1 and Der f2 was the highest (31.2%,43/138). There was no significant difference in sensitization rate of dust mites components between AAAR group and AR group(all P>0.05). AAAR group had higher levels of sIgE to Der p23 than AR group [0.1 (0,0.1) IU/ml vs. 0 (0,0.1) IU/ml,Z=-2.819, P=0.005]. There were no significant differences in the positive rate of dust mite components and sIgE levels between children aged≤6 and>6 years old with airway allergic diseases(all P>0.05). Conclusions:Der p1,Der p2,Der f1 and Der f2 are the major components of dust mites sensitizing airway allergic diseases in children. Der p1,Der p2,Der f1 and Der f2 are the main co-sensitizing components in children with dust mite-induced airway allergic diseases. Compared with AR,the sIgE level to Der p23 in children with AAAR is higher.

Image 1.

OBJECTIVE: To observe the characteristics of changes in non-invasive hemodynamic parameters in neonates with septic shock so as to provide clinical reference for diagnosis and treatment. METHODS: A observational study was conducted. The neonates with sepsis complicated with septic shock or not admitted to neonatal intensive care unit (NICU) of the First Affiliated Hospital of Shantou University Medical College were enrolled as the study subjects, who were divided into preterm infant (< 37 weeks) and full-term infant (≥ 37 weeks) according to the gestational age. Healthy full-term infants and hemodynamically stable preterm infants transferring to NICU after birth were enrolled as controls. Electronic cardiometry (EC) was used to measure hemodynamic parameters, including heart rate (HR), mean arterial pressure (MAP), stroke volume (SV), stroke volume index (SVI), cardiac output (CO), cardiac index (CI), systemic vascular resistance (SVR) and systemic vascular resistance index (SVRI), before treatment in the septic shock group, at the time of diagnosis of sepsis in the sepsis without shock group, and before the discharge from the obstetric department or on the day of transferring to NICU in the control group. RESULTS: Finally, 113 neonates with complete data and parental consent for non-invasive hemodynamic monitoring were enrolled, including 32 cases in the septic shock group, 25 cases in the sepsis without shock group and 56 cases in the control group. In the septic shock group, there were 17 cases at the compensated stage and 15 cases at the decompensated stage. There were 21 full-term infants (20 cured or improved and 1 died) and 11 premature infants (7 cured or improved and 4 died), with the mortality of 15.62% (5/32). There were 18 full-term infants and 7 premature infants in the sepsis without shock group and all cured or improved without death. The control group included 28 full-term infants and 28 premature infants transferring to NICU after birth. Non-invasive hemodynamic parameter analysis showed that SV, SVI, CO and CI of full-term infants in the septic shock group were significantly lower than those in the sepsis without shock group and control group [SV (mL): 3.52±0.99 vs. 5.79±1.32, 5.22±1.02, SVI (mL/m²): 16.80 (15.05, 19.65) vs. 27.00 (22.00, 32.00), 27.00 (23.00, 29.75), CO (L/min): 0.52±0.17 vs. 0.80±0.14, 0.72±0.12, CI (mL×s^-1×m^-2): 40.00 (36.67, 49.18) vs. 62.51 (56.34, 70.85), 60.01 (53.34, 69.68), all P < 0.05], while SVR and SVRI were significantly higher than those in the sepsis without shock group and control group [SVR (kPa×s×L^-1): 773.46±291.96 vs. 524.17±84.76, 549.38±72.36, SVRI (kPa×s×L^-1×m^-2): 149.27±51.76 vs. 108.12±12.66, 107.81±11.87, all P < 0.05]. MAP, SV, SVI, CO and CI of preterm infants in the septic shock group were significantly lower than those in the control group [MAP (mmHg, 1 mmHg ≈ 0.133 kPa): 38.55±10.48 vs. 47.46±2.85, SV (mL): 2.45 (1.36, 3.58) vs. 3.96 (3.56, 4.49), SVI (mL/m²): 17.60 (14.20, 25.00) vs. 25.50 (24.00, 29.00), CO (L/min): 0.32 (0.24, 0.63) vs. 0.56 (0.49, 0.63), CI (mL×s^-1×m^-2): 40.01 (33.34, 53.34) vs. 61.68 (56.68, 63.35), all P < 0.05], while SVR and SVRI were similar to the control group [SVR (kPa×s×L^-1): 1 082.88±689.39 vs. 656.63±118.83, SVRI (kPa×s×L^-1×m^-2): 126.00±61.50 vs. 102.37±11.68, both P > 0.05]. Further analysis showed that SV, SVI and CI of neonates at the compensation stage in the septic shock group were significantly lower than those in the control group [SV (mL): 3.60±1.29 vs. 4.73±1.15, SVI (mL/m²): 19.20±8.33 vs. 26.34±3.91, CI (mL×s^-1×m^-2): 46.51±20.34 vs. 61.01±7.67, all P < 0.05], while MAP, SVR and SVRI were significantly higher than those in the control group [MAP (mmHg): 52.06±8.61 vs. 48.54±3.21, SVR (kPa×s×L^-1): 874.95±318.70 vs. 603.01±111.49, SVRI (kPa×s×L^-1×m^-2): 165.07±54.90 vs. 105.09±11.99, all P < 0.05]; MAP, SV, SVI, CO and CI of neonates at the decompensated stage in the septic shock group were significantly lower than those in the control group [MAP (mmHg): 35.13±6.08 vs. 48.54±3.21, SV (mL): 2.89±1.17 vs. 4.73±1.15, SVI (mL/m²): 18.50±4.99 vs. 26.34±3.91, CO (L/min): 0.41±0.19 vs. 0.65±0.15, CI (mL×s^-1×m^-2): 43.34±14.17 vs. 61.01±7.67, all P < 0.05], while SVR and SVRI were similar to the control group [SVR (kPa×s×L^-1): 885.49±628.04 vs. 603.01±111.49, SVRI (kPa×s×L^-1×m^-2): 114.29±43.54 vs. 105.09±11.99, both P > 0.05]. CONCLUSIONS Full-term infant with septic shock exhibit a low cardiac output, high vascular resistance hemodynamic pattern, while preterm infant with septic shock show low cardiac output and normal vascular resistance. At the compensated stage the hemodynamic change is low output and high resistance type, while at the decompensated stage it is low output and normal resistance type. Non-invasive hemodynamic monitoring can assist in the identification of neonatal septic shock and provide basis for clinical diagnosis and treatment.
Humans ; Shock, Septic/physiopathology* ; Infant, Newborn ; Hemodynamics ; Female ; Male ; Case-Control Studies ; Infant, Premature

Purpose To investigate the expression of bone sialoprotein(BSP)in the tissues and cells of endome-trial cancer(EC)and its effects on the proliferation and invasion of EC cells.Methods The expression of BSP was assessed by immunohistochemistry in 235 EC tissues and 88 normal endometrial tissues,and its correlation with clinico-pathological features was analyzed.Western blot was used to compare BSP levels between human endometrial carcinoma cell line(HHUA)and normal human endumetial epithelial cells(HEEC).BSP was knocked down in HHUA cells via transient transfection,and the cells were divided into blank control group and BSP-knockdown group.The effects of BSP knockdown on cell cycle,proliferation,migration,invasion,and apoptosis were evaluated using PI staining,CCK-8,scratch,Transwell,and Annexin V-FITC assays,respectively.Protein levels of TGF-β signaling pathway compo-nents were analyzed by Western blot.Results BSP expression was significantly higher in EC tissues than in normal endometrium(P＜0.001)and correlated with lymph node metastasis and advanced FIGO stage(P＜0.05).BSP pro-tein level was also significantly elevated in HHUA cells(2.455 8±0.008 9)compared to HEECs(1.571 2±0.005 4)(P＜0.01).After knockdown,compared with the control group,the proliferation index(74.4±3.33),migration rate(0.48±0.03),and invasion ability(0.36±0.11)of the cells were increased,and the apoptosis rate(25.97％)of the cells was increased(P＜0.05).Furthermore,the expression levels of TGF-β signaling pathway downstream proteins TGF-β1(0.290 4±0.002 3)、TGF-β2(0.292 9±0.001 6)、Smad2(0.469 3±0.001 1)、Smad3(0.247 0±0.001 7)、pAKT(0.382 1±0.001 9)、ATK(0.119 6±0.001 6)and MEK1(0.258 9±0.000 3)in the BSP-knockdown group of EC cells decreased(P＜0.01).Conclusion BSP is highly expressed in endometrial cancer and promotes cancer cell proliferation,invasion,and metastasis by activating the TGF-β signaling pathway.

Objective:To develop and validate a risk prediction model for hospital discharge readiness in patients undergoing surgery for Stanford type B aortic dissection (TBAD) .Methods:A total of 130 patients who underwent TBAD surgery at Taizhou Hospital of Zhejiang Province between January 2020 and September 2024 were recruited by convenience sampling and divided into a training set ( n=91) and a validation set ( n=39) at a 7∶3 ratio. Readiness for hospital discharge was assessed using the Chinese version of the Readiness for Hospital Discharge Scale, and patients were categorized into good and poor readiness groups. Univariate analysis was used to compare demographic and disease-related data as well as admission Aortic Dissection Detection Risk Score (ADD-RS) between groups. Logistic regression was employed to identify factors influencing discharge readiness. A nomogram was developed using R software, and its predictive performance was evaluated with receiver operating characteristic (ROC) curves. Calibration curves and decision curve analysis (DCA) were further used to assess the accuracy and clinical utility of the model. Results:Logistic regression identified feedback-based health education, number of hospitalizations, ADD-RS score, emergency surgery, and length of hospital stay as independent predictors of discharge readiness. ROC curve analysis showed that the area under the curve ( AUC) for predicting poor discharge readiness was 0.91 [95% CI (0.85, 0.97) ] in the training set and 0.84 [95% CI (0.80, 0.99) ] in the validation set. Calibration curves and the Hosmer-Lemeshow test confirmed good calibration in both sets ( P>0.05). DCA demonstrated significant net clinical benefit when the high-risk threshold exceeded 0.02, although the benefit decreased as the threshold approached 0.80. Conclusions:The risk prediction model developed in this study effectively predicts poor discharge readiness in patients after TBAD surgery, showing good discrimination and calibration. The identified risk factors provide targeted directions for clinical interventions, which may help improve patients' readiness for discharge.

Objective:To evaluate the preventive effect of an antimicrobial peptide spray on chemoradiotherapy-induced oral mu-cositis in patients with hematologic malignancies.Methods:From December 2021 to July 2023,a total of 191 newly diagnosed pa-tients with hematologic malignancies undergoing concurrent chemoradiotherapy at our hospital were included in the study.Patients were divided into a treatment group(n=124,received antimicrobial peptide spray)and a control group(n=67,received placebo spray).All patients underwent standardized chemoradiotherapy regimens and oral care.Outcomes compared between groups includ-ed the incidence and severity of oral mucositis,ulcer healing time,pain scores,antibiotic usage,inflammatory markers[C-reactive protein(CRP),procalcitonin(PCT)],duration of neutropenia,adverse events,and quality of life.Results:The incidence of oral mucositis in the treatment group was significantly lower than in the control group(12.90％vs.31.34％,P＜0.05),with a relative risk reduction(RRR)of 58.84％,absolute risk reduction(ARR)of 18.44％,and a number needed to treat(NNT)of 5.423.The treatment group showed shorter ulcer healing time,lower pain scores,reduced antibiotic usage and intensity,lower mean levels of CRP and PCT,and a shorter duration of neutropenia.The incidence of exacerbated local pain and drug-related adverse reactions was also significantly lower in the treatment group,compared to the control group(P＜0.05),with no evident systemic toxicity ob-served.Patients in the treatment group reported higher quality of life and satisfaction scores(both P＜0.05).Conclusion:The an-timicrobial peptide spray effectively reduces the incidence and severity of chemoradiotherapy-associated oral mucositis,mitigates in-flammation and infection risk,and improves quality of life.

Objective:To develop and validate a risk prediction model for hospital discharge readiness in patients undergoing surgery for Stanford type B aortic dissection (TBAD) .Methods:A total of 130 patients who underwent TBAD surgery at Taizhou Hospital of Zhejiang Province between January 2020 and September 2024 were recruited by convenience sampling and divided into a training set ( n=91) and a validation set ( n=39) at a 7∶3 ratio. Readiness for hospital discharge was assessed using the Chinese version of the Readiness for Hospital Discharge Scale, and patients were categorized into good and poor readiness groups. Univariate analysis was used to compare demographic and disease-related data as well as admission Aortic Dissection Detection Risk Score (ADD-RS) between groups. Logistic regression was employed to identify factors influencing discharge readiness. A nomogram was developed using R software, and its predictive performance was evaluated with receiver operating characteristic (ROC) curves. Calibration curves and decision curve analysis (DCA) were further used to assess the accuracy and clinical utility of the model. Results:Logistic regression identified feedback-based health education, number of hospitalizations, ADD-RS score, emergency surgery, and length of hospital stay as independent predictors of discharge readiness. ROC curve analysis showed that the area under the curve ( AUC) for predicting poor discharge readiness was 0.91 [95% CI (0.85, 0.97) ] in the training set and 0.84 [95% CI (0.80, 0.99) ] in the validation set. Calibration curves and the Hosmer-Lemeshow test confirmed good calibration in both sets ( P>0.05). DCA demonstrated significant net clinical benefit when the high-risk threshold exceeded 0.02, although the benefit decreased as the threshold approached 0.80. Conclusions:The risk prediction model developed in this study effectively predicts poor discharge readiness in patients after TBAD surgery, showing good discrimination and calibration. The identified risk factors provide targeted directions for clinical interventions, which may help improve patients' readiness for discharge.