This article reported the diagnosis and management of a case of a child with Kabuki syndrome. Kabuki syndrome is characterized by distinct facial features, skeletal anomalies, abnormal skin texture, and intellectual disabilities, and is primarily caused by heterozygous mutations in the lysine methyltransferase 2D(KMT2D) gene. The onset in this patient was insidious, presenting with short stature and intellectual impairment. Genetic testing identified a pathogenic heterozygous variant in the KMT2D gene. This article focuses on analyzing the necessity and appropriateness of growth hormone therapy in children with Kabuki syndrome, and includes a review of relevant literature to improve clinicians′ understanding of this rare condition.

Body weight abnormalities, including overweight, obesity, and underweight, have become a dual public health challenge in Chinese adults: overweight and obesity lead to a variety of chronic complications, while underweight increases the risks of malnutrition, sarcopenia, and organ dysfunction. To systematically address these issues, multidisciplinary experts in endocrinology, sports science, nutrition, and psychiatry from various regions have held multiple weight management seminars. Based on the latest epidemiological data and clinical evidence, they expanded the guideline to include assessment and intervention strategies for underweight, in addition to the core content of obesity management. This guideline outlines the etiological mechanisms, evaluation methods, and multidimensional management strategies for overweight and obesity, covering key areas such as diagnosis and assessment, medical nutrition therapy, exercise prescription, pharmacological intervention, and psychological support. It is intended to provide a scientific and standardized approach to weight management across the adult population, aiming to curb the rising prevalence of obesity, mitigate complications associated with abnormal body weight, and improve nutritional status and overall quality of life.

Objective:To investigate the correlation of psychological resilience with family functioning in adolescents.Methods:This study was a cross-sectional study. A total of 1 300 students in the pre-junior to senior grades of three middle schools with medical-educational integration project in Shanghai Minhang District were selected by stratified cluster random sampling from November 2023 to April 2024. Psychological resilience levels and family functioning were assessed using the Resilience Scale for Children and Adolescents (RSCA) and the Family Assessment Device (FAD) among adolescents, respectively. The relationship between psychological resilience levels and family function, and the related influencing factors were examined by Pearson correlation analysis and multiple linear regression analysis.Results:A total of 1 217 valid questionnaires were obtained with an effective rate of 93.6%. There were 600 boys and 617 girls, with an mean age of (14.04±2.19) years. The average score of RSCA for was (87.07±15.96) points. Univariate analysis showed that genders, ethnicities, family structures, parents′ marital status, main caregivers, parenting styles, and having negative life events were significantly associated with psychological resilience levels of adolescents (all P<0.05). Multiple linear regression showed that problem-solving ( β=6.902, P<0.001) and total family function ( β=16.764, P<0.001) were positively correlated with adolescents′ psychological resilience, while emotional response ( β=-3.869, P=0.001), emotional involvement ( β=-4.948, P=0.001), and behavioral control ( β=-6.801, P=0.002) were negatively correlated with adolescents′ psychological resilience. The final model explained 31.7% of the influencing factors of psychological resilience in adolescents. Conclusion:The level of psychological resilience of adolescents in Minhang District of Shanghai is above the medium level, and family functioning has a significant impact on their mental toughness.

Objective:To investigate the correlation of psychological resilience with family functioning in adolescents.Methods:This study was a cross-sectional study. A total of 1 300 students in the pre-junior to senior grades of three middle schools with medical-educational integration project in Shanghai Minhang District were selected by stratified cluster random sampling from November 2023 to April 2024. Psychological resilience levels and family functioning were assessed using the Resilience Scale for Children and Adolescents (RSCA) and the Family Assessment Device (FAD) among adolescents, respectively. The relationship between psychological resilience levels and family function, and the related influencing factors were examined by Pearson correlation analysis and multiple linear regression analysis.Results:A total of 1 217 valid questionnaires were obtained with an effective rate of 93.6%. There were 600 boys and 617 girls, with an mean age of (14.04±2.19) years. The average score of RSCA for was (87.07±15.96) points. Univariate analysis showed that genders, ethnicities, family structures, parents′ marital status, main caregivers, parenting styles, and having negative life events were significantly associated with psychological resilience levels of adolescents (all P<0.05). Multiple linear regression showed that problem-solving ( β=6.902, P<0.001) and total family function ( β=16.764, P<0.001) were positively correlated with adolescents′ psychological resilience, while emotional response ( β=-3.869, P=0.001), emotional involvement ( β=-4.948, P=0.001), and behavioral control ( β=-6.801, P=0.002) were negatively correlated with adolescents′ psychological resilience. The final model explained 31.7% of the influencing factors of psychological resilience in adolescents. Conclusion:The level of psychological resilience of adolescents in Minhang District of Shanghai is above the medium level, and family functioning has a significant impact on their mental toughness.

This article reported the diagnosis and management of a case of a child with Kabuki syndrome. Kabuki syndrome is characterized by distinct facial features, skeletal anomalies, abnormal skin texture, and intellectual disabilities, and is primarily caused by heterozygous mutations in the lysine methyltransferase 2D(KMT2D) gene. The onset in this patient was insidious, presenting with short stature and intellectual impairment. Genetic testing identified a pathogenic heterozygous variant in the KMT2D gene. This article focuses on analyzing the necessity and appropriateness of growth hormone therapy in children with Kabuki syndrome, and includes a review of relevant literature to improve clinicians′ understanding of this rare condition.

Body weight abnormalities, including overweight, obesity, and underweight, have become a dual public health challenge in Chinese adults: overweight and obesity lead to a variety of chronic complications, while underweight increases the risks of malnutrition, sarcopenia, and organ dysfunction. To systematically address these issues, multidisciplinary experts in endocrinology, sports science, nutrition, and psychiatry from various regions have held multiple weight management seminars. Based on the latest epidemiological data and clinical evidence, they expanded the guideline to include assessment and intervention strategies for underweight, in addition to the core content of obesity management. This guideline outlines the etiological mechanisms, evaluation methods, and multidimensional management strategies for overweight and obesity, covering key areas such as diagnosis and assessment, medical nutrition therapy, exercise prescription, pharmacological intervention, and psychological support. It is intended to provide a scientific and standardized approach to weight management across the adult population, aiming to curb the rising prevalence of obesity, mitigate complications associated with abnormal body weight, and improve nutritional status and overall quality of life.

Objective:To investigate the effect of pulmonary vascular dysfunction in the prognosis of patients with acute lung injury (ALI).Methods:Patients with ALI who underwent pulmonary artery catheterization in the department of critical care medicine of Wuhan NO.1 Hospital from June 2017 to June 2019 were enrolled. The general information, clinical and hemodynamic indexes [central venous pressure (CVP), pulmonary artery wedge pressure (PAWP), pulmonary artery systolic pressure (sPAP), pulmonary artery diastolic pressure (dPAP), mean pulmonary artery pressure (mPAP), cardiac index (CI)], acute physiology and chronic health evaluation Ⅱ (APACHEⅡ) score, arterial blood gas parameters [pH, partial pressure of oxygen (PO 2), partial pressure of carbon dioxide (PCO 2), oxygenation index (PaO 2/FiO 2)], whether there was shock or not; ventilator parameters [platform pressure (Plat), positive end-expiratory pressure (PEEP)], etc. were recorded. Pulmonary artery oxygen saturation, pulmonary vascular function indexes [transpulmonary potential gradient (TPG) and pulmonary vascular resistance index (PVRi)] were calculated. The relationship between TPG, PVRi and mechanical ventilation time, the length of intensive care unit (ICU) stay, cardiovascular days and 60-day mortality were analyzed in patients with different prognosis of 60-day and whether the TPG increased (≥12 mmHg was defined as elevated TPG, 1 mmHg = 0.133 kPa). Results:A total of 65 patients were included in the study, including 30 males and 35 females; aged (48.9±15.2) years old. Forty-eight cases survived in 60-days, 17 died, and the 60-day mortality was 26.2%. At the baseline, there were no significant differences in cardiopulmonary function measurements, such as CVP, sPAP, dPAP, PAWP, CI, etc. between the two groups of patients with different prognosis. The APACHEⅡ score, shock ratio, TPG and PVRi of the death group were significant higher than those of the survival group [APACHEⅡ: 34±9 vs. 28±11, shock: 52.9% vs. 25.0%, TPG (mmHg): 16.2±1.9 vs. 14.6±2.1, PVRi (kPa·s·L -1): 31.8±4.2 vs. 29.7±3.5, all P < 0.05]. The 60-day mortality of 47 patients with TPG ≥ 12 mmHg was significantly higher than that of 18 patients with TPG < 12 mmHg (34.0% vs. 5.6%), and the mechanical ventilation time and the length of ICU stay were also significantly longer (days: 17±9 vs. 11±8, 16±5 vs. 12±5), and the cardiovascular days also increased significantly (days: 23±7 vs. 18±6), and the differences were statistically significant (all P < 0.05). Pearson correlation analysis showed that PVRi was significantly correlated with mechanical ventilation time, the length of ICU stay and cardiovascular days ( r1 = 0.317, P1 = 0.030; r2 = 0.277, P2 = 0.005; r3 = 0.285, P3 = 0.002). In the individual multivariate Logistic regression model, the highest PVRi was an independent risk factor for the 60-day mortality [odds ratio ( OR) = 30.5, 95% confidence interval was 20.4-43.1, P = 0.023]. Conclusion:Pulmonary vascular dysfunction is common in ALI patients and is independently associated with adverse outcomes.

Objective: To describe the distribution and drug resistance of pathogens at hematology department of Jiangsu Province from 2014 to 2015 to provide reference for empirical anti-infection treatment. Methods: Pathogens were from hematology department of 26 tertiary hospitals in Jiangsu Province from 2014 to 2015. Antimicrobial susceptibility testing was carried out according to a unified protocol using Kirby-Bauer method or agar dilution method. Collection of drug susceptibility results and corresponding patient data were analyzed. Results: The separated pathogens amounted to 4 306. Gram-negative bacteria accounted for 64.26%, while the proportions of gram-positive bacteria and funguses were 26.99% and 8.75% respectively. Common gram-negative bacteria were Escherichia coli (20.48%) , Klebsiella pneumonia (15.40%) , Pseudomonas aeruginosa (8.50%) , Acinetobacter baumannii (5.04%) and Stenotropho-monas maltophilia (3.41%) respectively. CRE amounted to 123 (6.68%) . Common gram-positive bacteria were Staphylococcus aureus (4.92%) , Staphylococcus hominis (4.88%) and Staphylococcus epidermidis (4.71%) respectively. Candida albicans were the main fungus which accounted for 5.43%. The rates of Escherichia coli and Klebsiella pneumonia resistant to carbapenems were 3.5%-6.1% and 5.0%-6.3% respectively. The rates of Pseudomonas aeruginosa resistant to tobramycin and amikacin were 3.2% and 3.3% respectively. The resistant rates of Acinetobacter baumannii towards tobramycin and cefoperazone/sulbactam were both 19.2%. The rates of Stenotrophomonas maltophilia resistant to minocycline and sulfamethoxazole were 3.5% and 9.3% respectively. The rates of Staphylococcus aureus, Enterococcus faecium and Enterococcus faecalis resistant wards vancomycin were 0, 6.4% and 1.4% respectively; also, the rates of them resistant to linezolid were 1.2%, 0 and 1.6% respectively; in addition, the rates of them resistant to teicoplanin were 2.8%, 14.3% and 8.0% respectively. Furthermore, MRSA accounted for 39.15% (83/212) . Conclusions Pathogens were mainly gram-negative bacteria. CRE accounted for 6.68%. The rates of Escherichia coli and Klebsiella pneumonia resistant to carbapenems were lower compared with other antibacterial agents. The rates of gram-positive bacteria resistant to vancomycin, linezolid and teicoplanin were still low. MRSA accounted for 39.15%.