Objective:To analyze the nutritional status of progeria, and to provide reference for scientific nutritional management of progeria.Methods:This cross-sectional study included 15 children with progeria who were treated at Children′s Hospital, Zhejiang University School of Medicine, between April 2022 and May 2023. Data of medical history, physical examination, laboratory tests, dietary survey and body composition were collected and analyzed.Results:Among 15 patients there were 7 males and 8 females, aged 7.8 (2.3, 10.8) years. Twelve of the 15 patients exhibited signs of malnutrition. A 24-hour dietary survey was carried out in 14 of them. The daily energy intake of 11 cases was below recommended levels. Carbohydrate intake was insufficient in 10 cases, protein intake was insufficient in 7 cases, and fat intake was insufficient in 12 cases. Deficiencies in calcium, magnesium, iron and zinc were noted in 13, 13, 9 and 10 cases, respectively. Body composition was determined by dual-energy X-ray absorptiometry in 8 cases, and the bone mineral density was below average in 5 of them.Conclusions:Malnutrition, characterized by reduced energy intake, micronutrient deficiencies, and alteration in body composition, is prevalent in children with progeria. Regular routine nutritional assessment and proper interventions may benefit their long-term health status.

Objective:To establish the prediction model of the ultimate risk of mechanical ventilation for patients undergoing nasal high-flow oxygen therapy in the intensive care unit (ICU), provide clinicians with a convenient and effective prediction method and accurate treatment timing, and improve the prognosis of ICU patients.Methods:Patients admitted to the ICU of our hospital from January 2019 to December 2021 were retrospectively enrolled. General clinical data of the patients were collected, including vital signs, biochemical indices of blood gas, inflammatory indices, acute comorbidities, APACHE Ⅱ score, length of stay in ICU and total length of stay, within 24 h after admission. Statistical analysis was performed on the above indicators and a chart was constructed.Results:Finally, 362 patients were enrolled in this study, and were divided into the transnasal high flow oxygen therapy group (HFNC group) and noninvasive positive pressure ventilation group (NIPPV group) according to whether mechanical ventilation was finally performed. The univariate and binary Logistic multivariate regression analysis showed that APACHE Ⅱ score ( OR=1.323, 95% CI: 1.818-1.483), ROX index ( OR=0.371, 95% CI: 0.226-0.609), total length of stay ( OR=1.097, 95% CI: 1.003-1.200) and complicating acute respiratory failure ( OR=2.456, 95% CI: 1.368-4.506) were independent influencing factors for determining whether patients underwent mechanical ventilation. Based on the above independent influencing factors, the lipopograms were constructed. The goodness of fit R2 and C-index of the model were 0.892 and 0.985, respectively through evaluation and verification model. The calibration curve of the model fitted well with the ideal curve, and the areas under the ROC curve of the rosettes and independent factors were 0.985, 0.959, 0.899, 0.656 and 0.576, respectively, indicating that the model was more effective than the independent index in predicting risk. Decision curve analysis also showed that the rosette had high clinical benefit. Conclusions:There are many related factors affecting whether patients undergo mechanical ventilation after nasal high-flow oxygen therapy. In this paper, after univariate and multivariate analysis, the most valuable indicators are combined to establish a line chart with better predictive performance to assess patients' risk, which can further provide clinicians with simple and effective prediction methods and improve the prognosis of patients.

Objective:To compare the effects of two pretreatment schemes on the efficacy, gonad and reproductive function of haploid hematopoietic stem cell transplantation recipients with severe aplastic anemia(SAA).Methods:The data of 73 patients with SAA who underwent haploid hematopoietic stem cell transplantation were analyzed retrospectively.The pretreatment scheme was divided into Fludarabine+ Cyclophosphamide+ Antithymocyte globulin group(FC lowATG group, 45 cases)and Busulfan+ Cyclophosphamide+ Antithymocyte globulin group(Bucy/ATG group, 28 cases). The changes of blood cell implantation time, follicle stimulating hormone(FSH), luteinizing hormone (LH), estradiol and testosterone were compared between the two groups. Results:there was no significant difference in blood cell implantation time between the two groups( P=0.096; P=0.133). The levels of FSH and LH in female recipients in Bucy/ATG group were higher than those in FC lowATG group, and the level of estradiol was lower than that in FC lowATG group.There were significant differences between the groups(all P<0.05). The pregnancy or fertility rate of female recipients in Bucy/ATG group was lower than that in FC lowATG group(all P<0.05). There was no significant difference in FSH, LH, testosterone and fertility between the two groups(all P>0.05). There was no significant difference in 2-year overall survival rate and failure free survival rate between the two groups( P=0.091; P=0.084). Conclusions:FC lowATG may be an effective pretreatment scheme for haploid hematopoietic stem cell transplantation in SAA with less damage to gonad and reproductive function.

Objective:To verify the application value of team-based learning (TBL) joint case-based discussion (CBD) method applied in the course teaching of Introduction to General Practice.Methods:Students from two clinical oriented classes in the same grade were divided into the experimental group ( n=35) and the control group ( n=35). The control group implemented the traditional teaching method, and the experimental group implemented the TBL joint CBD method. The teaching effect were compared between the two groups. SPSS 20.0 was used for frequency description and statistical test analysis. Non-normal distribution data were tested with Mann-Whitney U test. Results:After the teaching, the average rank of multiple-choice questions (35.28) and analytic questions (42.24) in the experimental group were significantly higher than those of the control group (32.68 and 25.52, respectively, P<0.05). Conclusion:The TBL joint CBD method applied in the course of general practice helps to improve students' test achievement, enhance their overall quality, and lay a foundation for training comprehensive competencies of general practitioners.

Objective: To analyze the clinical characteristics caused by acute poisoning by inhalation of hydrogen chloride (HCl) and to raise awareness and treatment level of the disease. Methods: The clinical manifestations, imaging features, diagnosis, treatment and prognosis of 5 patients with acute HCl poisoning were analyzed retrospectively. Results: Among the 5 cases of HCl poisoning, 2 cases were severe poisoning, 3 cases were moderate poisoning. All patients were treated with corticosteroids and symptomatic treatment, one of them was treated with venovenous extracorporeal membrane oxygenation (VV-ECMO) . All patients were recovered and discharged from hospital. Conclusion The lung damage of acute poisoning by inhalation of HCl is rapidly progressing, early detection and timely medical treatment can obtain a better prognosis.

Objective To investigate the efficacy and safety of 308-nm excimer laser compared to high-intensity ultraviolet radiation for the treatment of active localized vitiligo,and to observe changes in skin lesions before and after the treatment by confocal laser scanning microscopy.Methods Sixty patients with 203 skin lesions of active localized vitiligo and were enrolled into this study,and the vitiligo disease activity (VIDA) score of these patients ranged from 2 to 3.We selected more than 3 skin lesions from a same anatomical site of each patient,one of lesions served as a control and the other skin lesions (≥ 2) were randomly treated with 308-nm excimer laser (laser group) or high-intensity ultraviolet radiation (ultraviolet group).The treatment was conducted twice a week for 25 sessions,and a 3-month follow-up was performed.Results A total of 48 patients with 169 skin lesions completed the trial.The marked response rate was significantly higher in the laser group [66.15％ (43/65)] than in the ultraviolet group [44.64％ (25/56),x2 =8.28,P ＜ 0.01].The patients with a VIDA score of 2 showed a significantly higher marked response rate [67.69％(44/65)] compared with those with a VIDA score of 3 [44.64％(25/56),x2 =6.80,P ＜ 0.01].During the 3-month follow-up,no relapse was observed.Confocal laser scanning microscopy showed that the number of inflammatory cells increased at the dermal-epidermal junction of the intra-and extra-marginal lesional skin.After treatment,the number of inflammatory cells markedly decreased and returned to normal level in lesions.Conclusion Both 308-nm excimer laser and high-intensity ultraviolet radiation are effective in the treatment of active localized vitiligo,but the 308-nm excimer laser shows a more rapid onset of action and a better therapeutic effect.

A CALLG2008 protocol was developed by the Chinese Acute Lymphoblastic Leukemia Cooperative Group for adult acute lymphoblastic leukemia (ALL).We retrospectively analyzed 153 newly diagnosed adult patients with Philadelphia chromosome (Ph)-positive ALL enrolled into imatinib (400 mg/d) plus CALLG2008 regimen between 2009 and 2015.The median age was 40 years (range,18-68 years),with 81 (52.3％) males.The overall hematologic complete remission (CR) rate was 96.7％ after induction.With a median follow-up of 24.2 months,the estimated 3-year overall survival (OS) and event-free survival (EFS) rates were 49.5％ (95％ confidence interval (CI):38.5％-59.5％) and 49.2％ (95％ CI:38.3％-59.2％),respectively.Fifty-eight (36 with haploidentical donor) patients underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT) in first CR.Among the patients in CR1 after induction,both the 3-year OS and EFS were significantly better in the allo-HSCT group than in the without allo-HSCT group (73.2％,95％ CI:58.3％-83.5％ vs.22.2％,95％ CI:8.7％-39.6％ and 66.5％,95％ CI:50.7％-78.2％ vs.16.1％,95％ CI:5.1％-32.7％,respectively).Multivariate analysis showed that allo-HSCT and achievement of major molecular response were associated with favorable OS or EFS independently.Interestingly,in the allo-HSCT cohort,the donor type (haploidentical versus matched donors) had no significant impact on EFS or OS.All these results suggested that imatinib plus CALLG2008 was an effective protocol for Ph-positive ALL.Haploidentical donors can also be a reasonable alternative expedient donor pool.

Objective To investigate the expression of DNA methyltransferase (DNMT) in HL-60 cells induced by tetrandrine (Tet).Methods HL-60 cells were treated with different concentrations of Tet and decitabine (DAC) alone and in combination with both.Methyl thiazolyl tetrazolium (MTT) assay was used to assess cytotoxic effect.Flow cytometry (FCM) was used to determine apoptosis rate.Real-time quantitative polymerase chain reaction (PCR) assay was used to quantify mRNA levels of DNMT.Western blot was used to quantify the expression of DNMT protein.Results Tet inhibited the growth and proliferation of HL-60 cells in a time-and dose-dependent manners (both P ＜0.01).Tet treated HL-60 cells after 48 h at the concentration of 2 μmol/L,and 4 μmol/L,the levels of DNMT gene and protein in the drug administration group decreased compared to the control group.After incubation for 48 h with Tet 2 μmol/L combined with DAC 4 μmol/L,the combination group was significantly depressed.Conclusions Tet could potently inhibit the growth and proliferation of HL-60 cells,reduce the expression levels of DNMT mRNA and protein,and have a more obvious effect in the combination group.

Vertebrobasilar dolichoectasia(VBD ) can increase the risk of stroke. Significant expansion, elongation and tortuosity of the vertebrobasilar arteries are the main morphology manifestations of VBD. However, there is no consensus on the quantitative imaging assessment of VBD. Many studies showed that some quantitative parameters, such as basilar artery length and bending length, basilar angulation, vertebral tortuosity index can be used for the quantitative imaging assessment of VBD.

The clinical laboratory equipment on hospital ship was introduced, whose status and common faults were analyzed during Philippines humanitarian medical rescue mission and 3 times of Mission of Harmony. The experience was summarized for the maintenance of the clinical laboratory equipment. The problems and countermeasures were proposed for the clinical laboratory equipment.