Objective:To investigate the intervention effect of decision aid on the fear of falling in elderly patients after total hip arthroplasty.Methods:This study was a quasi experimental research. From June 2021 to November 2021, 84 patients after total hip arthroplasty who were admitted to the Department of Orthopedics of Zhengzhou Central Hospital Affiliated to Zhengzhou University were selected as the research objects. According to the order of admission, they were divided into the control group (42 cases, 2 cases fell off ) and the observation group (42 cases). The control group was given routine nursing care and guidance for fear of falling, while the observation group was given a decision-making aid intervention program on the basis of the control group. The time to first getting out of bed, the scores of Modified Falls Efficacy Scale (MFES), Generalized Anxiety Disorder (GAD-7) and Harris Hip Score (HHS) were compared between two groups.Results:The time to get out of bed for the first time in the observation group was (42.71 ± 6.41) h, lower than that in the control group (49.95 ± 5.73) h, and the difference was statistically significant ( t=5.38, P<0.05). Twelve weeks after discharge, the GAD-7 score in the observation group was (4.64 ± 1.43), which was lower than that of the control group (6.85 ± 1.83), and the difference was statistically significant ( t=6.10, P<0.05). The score of the MFES in the observation group was [8.50(8.00,10.00)], which was higher than that in the control group [7.50(7.00,8.00)], and the difference was statistically significant ( Z=-6.26, P<0.05). The hip joint function score of the observation group was (81.74 ± 4.24), which was higher than that of the control group (74.30 ± 4.51), and the difference was statistically significant ( t=7.69, P<0.05). Conclusions:Decision support can advance the time of downward movement of elderly patients after total hip arthroplasty, reduce their anxiety and fear of falling, and improve hip function.

Objective:To explore the clinical manifestations and genetic characteristics of Wolf-Hirschhorn syndrome (WHS) to improve the ability of diagnosis and differential diagnosis of the disease.Methods:The clinical features and auxiliary examinations and treatment of a proband with WHS caused by microdeletion of 4p16.3 segment who admitted to the Third Affiliated Hospital of Zhengzhou University in December 2021 were recorded, and whole exome sequencing (WES) of the family was performed. The prognosis was followed up.Results:The female proband, 11 months old, presented with convulsions at the age of 8 months, with the characteristics of heat sensitivity and cluster seizures, and her identical twin sister had a similar medical history. Physical examination found malnutrition, retarded development, special face, prominent forehead, wide nasal bridge, small jaw, precordial murmur and grade 3/6 murmur in the whole period, hyperactivity of P2, and low limb muscle tone. The whole exon and copy number variation (CNV) test of the family revealed that the proband had a 1.99 Mb heterozygous deletion in the chromosome 4p16.3 segment, including WHSC1 (NSD2), WHSC2 (NEFLA) and other genes. Copy number variation sequencing (CNV-Seq) of the proband and her sister showed 1.97 and 1.92 Mb heterozygous deletion of chromosome 4p16.3, respectively. Genealogical analysis by quantitative polymerase chain reaction revealed that the CNV was de novo, and it was determined to be a pathogenic variant according to the American College of Medical Genetics and Genomics guidelines. The proband took sodium valproate orally, and her sister took oral sodium valproate, zonisamide, and levetiracetam successively, and at the same time they received family rehabilitation training. The age at the last follow-up was 1 year and 8 months. Neither of them had convulsions again in the past 3 months, but the developmental delay was obvious. Conclusion:WHS patients may present with growth retardation, epilepsy, Greek warrior helmet-like special face, and congenital heart disease, and may have microdeletions in the chromosome 4p16.3 segment.

Objective: To investigate the long-term efficacy and safety of left cardiac sympathetic denervation(LCSD) for long QT syndrome(LQTS) patients with either recurrence on drug therapy intolerance/refusal. Methods: This study was a retrospective cohort study. The cases selected from 193 patients with LQTS who were enrolled in the Chinese Channelopathy Registry Study from November 1999 to November 2012. This study selected 28 LQTS patients with either recurrence on drug therapy intolerance/refusal and underwent LCSD surgery in the Peking University People's Hospital or Beijing Tongren Hospital. The patients were allocated into 3 groups: high-risk group(n=13, baseline QTc ≥550 ms or symptomatic in the first year of life or highly malignant genetics); intermediate-risk group(n=10, 500 ms≤baseline QTc<550 ms, symptomatic after the first year and without highly malignant genetics); low-risk group(n=5, baseline QTc<500 ms, symptomatic after the first year and without highly malignant genetics). LCSD was performed with the traditional supraclavicular approach or video assisted thoracoscopic surgery (VATS). Patients were regularly followed up until 20 years after the surgery. Data were collected before and 1 year after surgery and at the last follow-up. Patients' electrocardiograph(ECG), cardiac events and surgery-related complications were recorded. Kaplan-Meier survival analysis was used to determine the cardiac event-free survival based on different risk stratification and genotypes. Results: A total of 28 LQTS patients, aged 20.5 (15.0, 37.5) and underwent LCSD surgery, were enrolled in this study, including 23(82.1%) women. There were 11(39.3%) patients treated with traditional approach while 17(60.7%) with VATS-LCSD. There were 19(67.9%) patients had positive genetic test results, including 4 LQT1, 12 LQT2, 1 LQT1/LQT2 mixed type, and 2 Jervell-Lange-Nielsen (JLN) syndrome. The median follow-up period was 189.3(138.7, 204.9) months. The dropout rate was 10.7%(3/28) while 3 patients in the intermediate-risk group were lost to follow-up. Horner syndrome occurred in 1 patient (in the high-risk group). Sudden cardiac deaths were observed in 3 (12.0%) patients (all in the high-risk group), and 12 patients (48.0%) had syncope recurrences (2 in low-risk group, 3 in intermediate-risk group and 7 in high-risk group). A significant reduction in the mean yearly episodes of cardiac events was observed, from (3.5±3.3) before LCSD to(0.2±0.1) at one year after LCSD and (0.5±0.8) at last follow up(P<0.001). The mean QTc was shortened from (545.7±51.2)ms before the surgery to (489.0±40.1)ms at the last follow-up (P<0.001). Among the 20 patients with basic QTc ≥500 ms and completing the follow-up, the QTc intervals of 11(55.0%) patients were shortened to below 500 ms. The event free survival rates for any cardiac events after LCSD decreased sequentially in the low-, intermediate- and high-risk groups, and the difference was statistically significant (χ²=7.24, log-rank P=0.026). No difference was found in the event free survival rates among LQT1, LQT2 and undefined gene patients (χ²=5.20, log-rank P>0.05). Conclusions: LCSD surgery can reduce the incidence of cardiac events and shorten the QTc interval in patients with LQTS after the long-term follow-up. LCSD surgery is effective and safe for patients with LQTS ineffective or intolerant to drug therapy. However, high-risk patients are still at a high risk of sudden death after surgery and should be actively monitored and protected by combined therapies.
Electrocardiography ; Female ; Heart ; Humans ; Long QT Syndrome ; Male ; Retrospective Studies ; Sympathectomy/methods*

[摘 要] 目的：探讨抗PD-1单抗联合化疗及抗血管生成药物治疗晚期黑色素瘤的疗效和安全性。方法：收集2020年4月至2021年6月在北京大学肿瘤医院接受抗PD-1单抗联合化疗药物替莫唑胺±顺铂、白蛋白结合型紫杉醇及抗血管生成药物贝伐珠单抗治疗的14例（男6、女8例）不可切除的晚期黑色素瘤患者的临床资料。主要研究终点为无进展生存期（PFS），次要终点为客观有效率（ORR）、疾病控制率（DCR）、总生存期（OS）及安全性数据（CTCAE 5.0标准）。结果：14例晚期黑色素瘤患者均纳入生存分析，中位随访时间为5.50个月（95% CI: 0~13.12个月），中位PFS为7.43个月（95% CI: 3.07~11.79个月），中位OS为13.50个月（95% CI: 5.19~21.81个月），中位起效时间为1.5个月；ORR为28.6%（4例均为部分缓解），DCR为85.7%；不良反应多为1~2级。结论：抗PD-1单抗联合化疗及抗血管生成药物治疗在晚期黑色素患者中显示出初步的有效性及良好的安全性，此可能为晚期黑色素瘤的联合治疗策略提供了新思路。

Objective: To compare the clinical characteristics and plasma inflammatory markers levels in different endotypes of chronic rhinosinusitis with nasal polyps (CRSwNP), and to explore the plasma biomarkers associated with endotypes of CRSwNP. Methods: A total of 74 CRSwNP patients (male/female: 41/33; average age: 40 years) and 40 control subjects underwent septoplasty in Tongji Hospital from January 2015 to December 2017 were enrolled in this study. The demographic and clinical features of all subjects including age, gender, past history, visual analogue scale (VAS) and CT scores were recorded. Patients with CRSwNP were divided into Eos^highNeu^high, Eos^highNeu^low, Eos^lowNeu^high and Eos^lowNeu^low four endotypes according to the eosinophil (Eos) percentage and neutrophil (Neu) count of nasal polyps tissue. Preoperative blood routine was performed and the levels of 27 biomarkers in plasma were measured by Bio-Plex suspension chip method. The clinical characteristics and the level of serum biomarkers of patients with different endotypes were compared. SPSS 18.0 software was used for statistical analysis. Results: There was no difference in the clinical features including gender ratio, age, course of disease, VAS score, endoscopy and CT score among Eos^highNeu^high, Eos^highNeu^low, Eos^lowNeu^high and Eos^lowNeu^low CRSwNP patients. Compared with Eos^lowNeu^high and Eos^lowNeu^low CRSwNP patients, patients with Eos^highNeu^high and Eos^highNeu^low endotype demonstrated a higher prevalence of atopy, allergic rhinitis and asthma comorbidity, and increased peripheral blood eosinophil absolute count and percentage (all P<0.05). However, there was no significant difference between Eos^highNeu^high and Eos^highNeu^low CRSwNP. Plasma levels of all 27 mediators including type 1 cytokines (IL-12 and IFN-γ), type 2 cytokines (IL-4, IL-5 and IL-13), type 3 cytokines (IL-17A), pro-inflammatory cytokines (IL-6 and TNF-α) and tissue remodeling-related markers (bFGF, VEGF and PDGF-BB) demonstrated no significant difference among all endotypes of CRSwNP (all P>0.05). Conclusions: Eos^high and Eos^low CRSwNP patients display significant differences regarding the prevalence of atopy, allergic rhinitis and asthma comorbidity, peripheral blood eosinophil absolute count and percentage, but the clinical characteristics, blood cellular and biological markers can not effectively distinguish four endotypes of CRSwNP. Further studies are warranted to dig out the potential objective, convenient and reliable markers associated with endotypes in patients with CRSwNP.
Adult ; Chronic Disease ; Eosinophils ; Female ; Humans ; Inflammation Mediators ; Male ; Nasal Polyps/pathology* ; Rhinitis/pathology* ; Sinusitis/complications*

Chronic Disease ; Humans ; Nasal Polyps ; Prognosis ; Rhinitis ; Sinusitis

Objective:To investigate whether the existence of thoracic artery blood supply in peripheral pulmonary lesions is the key factor affecting the accuracy of contrast-enhanced ultrasound in differentiating benign and malignant lesions.Methods:From June 2020 to December 2021, a total of 170 patients with peripheral pulmonary lesions were consecutively enrolled in Xi′an Chest Hospital, and all patients underwent conventional ultrasound and contrast-enhanced ultrasound(CEUS). Taking ΔAT(lesion-lung arrival time difference ) of 2.5 seconds as the cut-off point for differentiating benign and malignant lesions(ΔAT<2.5 s for benign, ΔAT≥2.5 s for malignant), and the final pathological results as the gold standard, these patiens were divided into correct classification group and wrong classification group, and the main influencing factors of wrong classification were analyzed.Results:Compared with the correct classification group, the proportion of thoracic artery blood supply in the wrong classification group was significantly higher ( P<0.001). After adjusting the dendritic venous reflux, thoracic artery blood supply was an independent influencing factor for CEUS misclassification ( OR=3.531, 95% CI=1.805-6.908, P<0.001). In the patients with thoracic artery blood supply, the sensitivity of the absence of dendritic venous reflux in judging malignant peripulmonary lesions was 75.0%, the specificity was 91.3%, and the area under the ROC curve (AUC) was 0.832 (95% CI=0.715-0.915, P<0.001), while the sensitivity, specificity and AUC of CEUS in judging malignant peripulmonary lesions were 68.7%, 67.4% and 0.659 (95% CI=0.528-0.775, P=0.006), and there was significant statistical difference in the AUC between them( P<0.001). In the group of patients without thoracic arterial blood supply, the sensitivity of the absence of dendritic venous reflux in judging malignant peripulmonary lesions was 82.8%, the specificity was 63.3%, and the AUC was 0.730 (95% CI=0.636-0.811, P<0.001), while the sensitivity, specificity and AUC of CEUS in judging malignant peripulmonary lesions were 62.1%, 81.0% and 0.684 (95% CI=0.587-0.770, P=0.003), and there was no significant difference in the AUC between them ( P=0.425). Conclusions:The presence of thoracic artery blood supply significantly decrease the diagnostic efficiency of CEUS in differentiating benign and malignant of peripheral pulmonary lesions. For peripheral pulmonary lesions with thoracic arterial blood supply, the diagnostic efficiency of dendritic venous reflux is better than CEUS, while for lesions without thoracic artery supply, the diagnostic efficiency of dendritic venous reflux is equivalent to CEUS.

Objective:To investigate changes in circadian gene cryptochrome 2 (CRY2) expression in mouse models of psoriasis and HaCaT cells, and to explore underlying mechanisms.Methods:Imiquimod-induced mouse model experiment: 12 C57BL/6 female mice were randomly and equally divided into imiquimod group receiving topical imiquimod treatment for 5 consecutive days and control group receiving no treatment; these mice were sacrificed on day 6, skin tissues were resected from the back of mice, and immunofluorescence staining was performed to determine the CRY2 expression in the epidermis. HaCaT cell transfection experiment: HaCaT cells with small interfering RNA (siRNA) -mediated knockdown of CRY2 served as siRNA-CRY2 group, and siRNA-NC group as control group; 5-ethynyl-2′-deoxyuridine (EdU) staining was performed to evaluate the proliferative activity of the HaCaT cells, real-time fluorescence-based quantitative PCR (qPCR) to determine the mRNA expression of chemokines in the HaCaT cells, and Western blot analysis to determine phosphorylation levels of extracellular signal-regulated kinase 1/2 (ERK1/2) . Tumor necrosis factor-α (TNF-α) -stimulated animal and cell experiments: 12 C57BL/6 female mice were randomly and equally divided into TNF-α group subcutaneously injected with TNF-α solution in the ear for 6 days, and phosphate buffered saline (PBS) group subcutaneously injected with the same amount of PBS; the mice were sacrificed on day 7, skin tissues were resected from the ear of mice, and immunofluorescence staining was conducted to determine the CRY2 expression in the epidermis; CRY2-knockdown HaCaT cells stimulated with 50 ng/ml TNF-α for 12 hours served as siRNA-CRY2 + TNF-α group, and siRNA-NC + TNF-α group as control group; qPCR was performed to determine the mRNA expression of chemokines in HaCaT cells in the above groups. Statistical analysis was carried out by using two-independent-sample t test. Results:Immunofluorescence staining showed that the CRY2 protein expression was significantly lower in the mouse dorsal epidermis in the imiquimod group (0.94 ± 0.23) than in the control group (2.30 ± 0.25, t = 3.99, P = 0.016) . Compared with the siRNA-NC group, the siRNA-CRY2 group showed significantly increased proportions of EdU-positive cells (48.13% ± 10.97% vs. 38.23% ± 0.81%, t = 5.00, P = 0.007) , mRNA expression levels of chemokines CXCL1 and CXCL8, as well as significantly increased phosphorylated (p) -ERK1/2 protein expression levels (all P < 0.05) , while there were no significant differences in the CCL20 mRNA expression or ERK1/2 protein expression between the two groups (both P > 0.05) . Immunofluorescence staining showed significantly decreased CRY2 protein expression level in the mouse ear epidermis in the TNF-α group (0.37 ± 0.34) compared with the PBS group (2.04 ± 0.17, t = 4.38, P = 0.012) ; the relative mRNA expression levels of chemokines CXCL1, CXCL8, and CCL20 in HaCaT cells were significantly higher in the siRNA-CRY2 + TNF-α group than in the siRNA-NC + TNF-α group (all P < 0.05) . Conclusion:CRY2 was markedly underexpressed in psoriasis, which might promote the proliferation of keratinocytes and expression of chemokines CXCL1, CXCL8 and CCL20, and TNF-α might be an upstream cytokine that could downregulate CRY2 expression.

[摘 要] 目的：本研究旨在评估白蛋白紫杉醇+卡铂联合抗血管生成药物（nab-paclitaxel, carboplatin, antiangiogenic drug, NCA）方案用于既往治疗失败的晚期黑色素瘤患者的疗效和安全性。方法：收集2012年4月1日至2019年5月31日在北京大学肿瘤医院肾癌黑色素瘤科住院的黑色素瘤患者，回顾性分析NCA方案在既往治疗失败后的不可切除Ⅲ c期和Ⅳ期黑色素瘤患者中的疗效和安全性。主要终点指标为无进展生存期（PFS），次要指标为客观缓解率（ORR）、总生存期（OS）、疾病控制率（DCR）和不良反应。根据使用的抗血管药物分为恩度治疗组（n=73）和贝伐珠单抗治疗组（n=103），采用倾向性评分匹配以均衡不同抗血管生成药物组间基线变量的差异。结果：共计176例患者被纳入本项分析中。所有患者中位年龄51岁（范围为18~78岁）。Ⅳ期患者占97%，50%的患者LDH水平高于正常值，28%的患者存在肝转移。既往治疗线数占比分别为1线57%、2线33%、3~4线10%。所有患者的中位PFS为3.8个月（95%CI：3.0~4.6），中位OS为10.5个月（95%CI： 8.9~12.1）。2例患者获得完全缓解，9例患者获得部分缓解，全组的ORR为6%，DCR达70%。恩度治疗组和贝伐珠单抗治疗组的中位PFS分别为4.7个月（95%CI：3.5~5.9）和3.4个月（95%CI：3.0~4.6），两组中位OS分别为12.2个月（95% CI：11.1~13.2）和9.1个月（95%CI： 7.8~10.4）。对所有患者的年龄、性别、既往治疗线数和LDH水平进行倾向性评分匹配，贝伐珠单抗和恩度治疗组间PFS和OS差异无统计学意义。常见的不良反应包括脱发、周围神经病变、中性粒细胞减少、疲劳和恶心。26名（15%）患者由于不良反应停止了治疗。结论：白蛋白紫杉醇+卡铂联合抗血管生成药物对既往治疗失败的晚期黑色素瘤患者具有一定的疗效，不良反应可耐受。

Asians ; Biological Assay ; Breast Neoplasms/surgery* ; China ; Female ; Humans ; Mastectomy