Objective:To investigate the clinical features and prognosis of male with anti-melanoma differentiation-associated gene 5 (MDA5) autoantibody.Methods:The clinical data of 246 patients with DM and anti-MDA5 autoantibodies hospitalized by Jiangsu Myositis Cooperation Group from 2017 to 2020 were collected and retrospectively analyzed. Chi-square test was performed to compared between counting data groups; Quantitative data were expressed by M ( Q1, Q3), and rank sum test was used for comparison between groups; Single factor survival analysis was performed by Kaplan-Meier method and Log rank test; Cox regression analysis were used for multivariate survival analysis. Results:①The male group had a higher proportion of rash at the sun exposure area [67.1%(47/70) vs 52.8%(93/176), χ2=4.18, P=0.041] and V-sign [50.0%(35/70) vs 30.7%(54/176), χ2=8.09, P=0.004] than the female group. The male group had higher levels of creatine kinase [112(18, 981)U/L vs 57 (13.6, 1 433)U/L, Z=-3.50, P<0.001] and ferritin [1 500 (166, 32 716)ng/ml vs 569 (18, 14 839)ng/ml, Z=-5.85, P<0.001] than the female group. The proportion of ILD [40.0%(28/70) vs 59.7%(105/176), χ2=7.82, P=0.020] patients and the red blood cell sedimentation rate[31.0(4.0, 101.5)mm/1 h vs 43.4(5.0, 126.5)mm/1 h, Z=-2.22, P=0.026] in the male group was lower than that of the female group, but the proportion of rapidly progressive interstitial lung disease (PR-ILD) [47.1%(33/70) vs 31.3%(55/176), χ2=5.51, P=0.019] was higher than that of the female group. ②In male patients with positive anti-MDA5 antibodies,the death group had a shorter course of disease[1.0(1.0, 3.0) month vs 2.5(0.5,84) month, Z=-3.07, P=0.002], the incidence of arthritis [16.7%(4/24) vs 42.2%(19/45), χ2=4.60, P=0.032] were low than those in survival group,while aspartate aminotransferase (AST)[64(22.1, 565)U/L vs 51(14,601)U/L, Z=-2.42, P=0.016], lactate dehydrogenase (LDH) [485(24,1 464)U/L vs 352(170, 1 213)U/L, Z=-3.38, P=0.001], C-reactive protein (CRP) [11.6(2.9, 61.7) mg/L vs 4.95(0.6, 86.4) mg/L, Z=-1.96, P=0.050], and ferritin levels [2 000(681, 7 676) vs 1 125 (166, 32 716)ng/ml, Z=-3.18, P=0.001] were higher than those in the survival group, and RP-ILD [95.8%(23/24) vs 22.2%(10/45), χ2=33.99, P<0.001] occurred at a significantly higher rate. ③Cox regression analysis indicated that the course of disease LDH level, and RP-ILD were related factors for the prognosis of male anti-MDA5 antibodies [ HR (95% CI)=0.203(0.077, 0.534), P=0.001; HR (95% CI)=1.002(1.001, 1.004), P=0.003; HR (95% CI)=95.674 (10.872, 841.904), P<0.001]. Conclusion:The clinical manifestations of male anti-MDA5 antibody-positive patients are different from those of female. The incidence of ILD is low, but the proportion of PR-ILD is high. The course of disease, serum LDH level, and RP-ILD are prognostic factors of male anti-MDA5 antibody-positive patients.

Objective:To investigate the application of extracorporeal membrane oxygenation(ECMO)bridging heart transplantation in critically ill children.Methods:The clinical data of two cases of critical infants with venous-arterial ECMO(VA-ECMO)bridging heart transplantation and literature review were retrospectively analyzed.Results:Two cases received orthotopic heart allograft with VA-ECMO support, and were discharged uneventfully without significant postoperative complications.On the 13th day of ECMO assistance, the first child was treated with orthotopic heart transplantation in a hospital qualified for heart transplantation, and the ECMO was evacuated during the operation.After 21 days of the heart transplantation, the patient was discharged from the hospital.The patient was followed up to be healthy after heart transplantation, and had the same development as children of the same age, and had been taking anti-rejection drugs for a long time.On the 10th day of VA-ECMO treatment, the second case received awake ECMO after cardiac function improved.On the 12th day of VA-ECMO treatment, the patient was successfully evacuated from VA-ECMO and waited for heart transplantation.Cardiac orthotopic transplantation was performed after the 17 days after VA-ECMO evacuation.The patient was transferred to the general ward after 6 days of hospitalization in the intensive care unit, and was discharged 23 days after transplantation with conventional anti-rejection therapy.Discharge follow-up in good health, normal school life.Conclusion:When VA-ECMO cannot be withdrawn from the heart of the critically ill children and the end-stage heart, VA-ECMO bridging heart transplantation should be selected at the right time for the children who meet the indications for heart transplantation to create survival opportunity for the previously hopeless children, save the life of the end-stage children, and improve the quality of life.

The article reports a rare case of isolated persistent left superior vena cava (PLSVC) found during the catheterization of a dialysis catheter in an end-stage renal disease patient and the occurrence of guidewire entrapment in Chiari net. The patient was scheduled to have emergency dialysis due to end-stage renal disease and acute left heart failure. And a tunnel-cuffed catheter (TCC) for dialysis insertion was planned. Isolated PLSVC was found firstly by the imaging process. Then the guidewire was entrapped at the right atrium in the further operation. During the operation of the guidewire, the guidewire loosened and withdrawn with a fine fiber, which was considered a possibility of Chiari net. In the follow-up after one week, the TCC worked well, and the heart function improved after dialysis.

Renal replacement therapy and perioperative management have difficulties in hemophilia patients with end-stage renal disease. The paper summarized the diagnosis and treatment experience of six hemophilia patients complicated with end-stage renal disease from January 1, 2000 to March 31, 2023 in Peking Union Medical College Hospital. Among 6 patients treated with peritoneal dialysis, 3 were treated with hemodialysis or continuous venous-venous hemodialysis. Altogether 11 dialysis access procedures were conducted successfully, and no serious bleeding or thrombotic events. In further conjunction with literature review, the paper summarized the key points of dialysis access appliance relevant to such patients, to provide reference for renal replacement treatment paths.

Humans ; Dermatomyositis/pathology* ; Interferon-Induced Helicase, IFIH1/genetics* ; Lung Diseases, Interstitial ; Autoantibodies ; Retrospective Studies

Objective:To compare the short-term efficacy of orthopedic robot-assisted and traditional fluoroscopy-guided sustentaculum talus screw fixation of Sanders type II and III calcaneal fracture with.Methods:A retrospective cohort study was conducted to analyze the clinical data of 54 patients with Sanders type II and III calcaneal fracture admitted to People′s Hospital of Nanchang County of Jiangxi Province from October 2020 to October 2021, including 30 males and 24 females; aged 29-57 years [(39.5±7.4)years]. Type of fracture was Sanders type II in 38 feet and Sanders type III in 22 feet. Sustentaculum talus screw fixation was performed using orthopedic robot-assisted technique in robot group (26 patients, 28 feet) and using intraoperative fluoroscopy-guided technique in freehand group (28 patients, 32 feet). The intraoperative fluoroscopy frequency, incision length, intraoperative blood loss and operation time were recorded. Calcaneus width, B?hler angle and Gissane angle were measured preoperatively and at 3-, 6-month postoperatively. Foot function was assessed using the Maryland scale at 6 months after operation. Postoperative complications were observed.Results:All patients were followed up for 6-8 months [(6.9±0.7)months]. The intraoperative fluoroscopy frequency and operation time were (2.8±0.7)times and (46.9±2.4)minutes in robot group, compared to (10.1±2.0)times and (75.6±3.1)minutes in freehand group (all P<0.01). There was no significant difference in the incision length and intraoperative blood loss between the two groups (all P>0.05). The calcaneus width, B?hler angle and Gissane angle in the two groups were improved at postoperative 3, 6 months when compared with preoperative ones (all P<0.05), and the improvement was much better in robot group than that in freehand group ( P<0.05 or 0.01). The excellent and good rate of Maryland score was 100% (28/28) (21 excellent, 7 good) in robot group at postoperative 6 months, compared to 78.1% (25/32) (19 excellent, 6 good, 7 fair) in freehand group ( P<0.05). The postoperative complication rate was 0% (0/28) in robot group and was 6.3% (2/32) in freehand group ( P>0.05). Conclusion:For Sanders type II and III calcaneal fracture, orthopedic robot-assisted sustentaculum talus screw fixation is superior to traditional fluoroscopy-guided internal fixation since it can significantly reduce intraoperative fluoroscopy frequency, shorten operation time, improve calcaneus width, B?hler angle and Gissane angle, and achieve better foot function.

Objective:To investigate the attitude of neonatologists toward the treatment of extremely preterm infants (EPIs) in China.Methods:A cross-sectional survey was conducted using a questionnaire designed and posted on Wenjuanxing, a web-based survey platform, from June to July 2021. The respondents were neonatal physicians in various provinces and cities in China. The questionnaire covered the basic information, treatment experience and attitude towards EPIs, and opinions on the current definition of the preterm infant in China. The results were described or analyzed using the Chi-square test.Results:A total of 1 066 valid replies were collected. The respondents included 322 males and 744 females, among whom 78.1% (832/1 066) were assistant director physicians or director physicians, 82.8% (882/1 066) were from tertiary hospitals, and 83.0% (885/1 066) had the experiences of treating EPIs. In terms of the attitude toward the treatment of EPIs, 63.0% (672/1 066) of the respondents suggested that the lower limit of gestational age for EPIs requiring active resuscitation should be defined at 25 gestational weeks or less. Moreover, 57.1% (609/1 066) considered that the current domestic definition of preterm infants as 28 gestational age or above was inappropriate, and 75.2% (458/609) considered that the lower limit defined as preterm infants should be 25 gestational weeks or less. Concerning the treatment experience in EPIs, 54.3% (579/1 066) of the respondents suggested that in their hospital, withdrawing treatment in EPIs was common or very common, and 83.3% (888/1 066) considered that the main reason for withdrawing treatment was family members' concerns about the prognosis. Those who hesitated about treating the EPIs accounted for 71.6% (763/1 066), and 83.9% (640/763) hesitated due to the poor prognosis and possible medical disputes. Moreover, 32.7% (349/1 066) of the respondents or their colleagues had been involved in medical disputes about whether to treat EPIs, and 74.8% (797/1 066) believed that the patients should be the decision-maker on whether to treat EPIs or not.Conclusion:Most neonatal physicians in this survey hold a positive attitude toward the treatment of EPIs and believe that the lower limit of gestational age for preterm infants should be lowered. However, a hesitating attitude to the care of EPIs is still common, and uncertainty about the prognosis of EPIs remains a concern.

Objective:Analyze the influence of blood transfusion therapy under the guidance of thromboelastography on the blood potassium and coagulation indicators and prognosis of patients with post-traumatic hemorrhage.Methods:The clinical data of 73 patients with post-traumatic hemorrhage in Liyang People′s Hospital from March 2018 to February 2021 were retrospectively analyzed. Among them, TEG blood transfusion guidance group (group A) and conventional coagulation indicator guidance group (group B) were divided according to whether TEG test was performed. Repeated measurement analysis of variance and post LSD- t test were used to observe serum potassium, coagulation indexes and prognosis of 2 groups at each time point. Results:The amount of various drugs used in group A was significantly lower than that in group B ( P<0.05). Comparison of serum potassium levels between the two groups before transfusion: (3.94 ± 0.85) mmol/L vs. (3.98 ± 0.71) mmol/L; and on the first day after transfusion: (4.33 ± 0.48) mmol/L vs. (4.57 ± 0.73) mmol/L, there were statistically significant ( P<0.05); on day one. day two and day three after blood transfusion, the indexes of PT and APTT in group A were significantly lower than those in group B: PT: (14.30 ± 1.43) s vs. (16.25 ± 1.74) s, (14.41 ± 1.55) s vs. (16.27 ± 1.48) s, (14.73 ± 1.50) s vs. (16.30 ± 1.45) s; APTT: (32.3 ± 3.6) s vs. (36.7 ± 3.5) s, (32.6 ± 3.4) s vs. (36.8 ± 3.6) s, (32.2 ± 3.2) s vs. (36.3 ± 3.3) s; the above indexes of the two groups before treatment were significantly higher than those at each time point after treatment ( P<0.05). The total effective rate of hemostasis in group A was higher than that in group B: 97.37%(37/38) vs. 80.00%(28/35), P<0.05. Conclusions:Patients with post-traumatic hemorrhage can be recovered by blood transfusion under the guidance of thromboelastography, and the disorder of blood coagulation indicators can be restored, and at the same time, it has a better hemostatic effect.

Objective:To analyze the clinical characteristics of adult Chinese patients with syncope.Methods:This is a cross-sectional survey study. Patients with preliminary diagnosis of syncope in the Emergency Department, Geriatrics and Cardiology Outpatient Department, or Syncope Unit of 37 hospitals in 19 provinces, autonomous regions and the Hong Kong Special Administrative Region from June 2018 to March 2021 were included in this study. The clinical features of these patients with syncope were analyzed.Results:A total of 4 950 consecutive patients with syncope were included in this study. The age was (56.3±16.8)years, and 2 604 cases (52.6%) were male. The most common type of syncope was neurally mediated syncope (2 345 (47.4%)), followed by cardiac syncope (1 085 (21.9%)), orthostatic hypotensive syncope (311 (6.3%)), and unexplained syncope accounted for nearly one third (1 155 (23.3%)). Predisposing syncope was more common in patients under 65 years of age(2 066(72.4%) vs. 786(27.6%),χ 2=136.5, P<0.001). Presyncope was more common in patients with neurally mediated syncope (1 972(79.0%) vs.1 908(73.9%), χ 2=17.756, P<0.001). Premonitory symptoms were more common in women(1 837(80.0%) vs. 1 863(73.0%),χ 2=33.432, P<0.001). Presyncope syndrome was more common in patients under 65 years of age (2 482(77.8%) vs. 1 218(73.4%),χ 2=17.523, P=0.001). Cyanosis was more common in ≥65 years old patients (271(18.2%) vs. 369(12.7%), χ 2=23.235, P<0.001). Urinary incontinence was more common in old patients aged ≥65 years(252(15.2%) vs. 345(10.8%), χ 2=19.313, P<0.001). Family history was more common in patients with cardiogenic syncope compared with other types of syncope (264(24.3%) vs. 754(19.5%), χ 2=11.899, P=0.001). Hypertention(1 480(30.5%)), coronary heart disease(1 057(21.4%)), atrial flutter and atrial fibrillation(359(7.2%)), second degree atrioventricular block(236(4.8%)) were common complications of syncope. The proportion of patients with coronary heart disease was significantly higher in cardiac syncope than that of other types of syncope(417(38.4%) vs. 640(16.6%), χ 2=241.376, P<0.001). Other common complications included cerebrovascular diseases (551 (11.1%)) and diabetes mellitus (632(12.8%)). Conclusions:Neurally mediated syncope is the most common syncope in adult Chinese population. Patients with predisposing conditions and premonitory conditions are younger. Presyncope is more common in women. The proportion of family history and coronary heart disease is higher in patients with cardiogenic syncope.

Objective:To explore the risk factors for mortality in pediatric acute respiratory distress syndrome (PARDS) requiring extracorporeal membrane oxygenation (ECMO) support.Methods:Clinical data of 109 patients with severe PARDS supported by ECMO, who were hospitalized in 6 ECMO centers in China from September 2012 to February 2020, were retrospectively analyzed. They were divided into survival group and death group according to the prognosis. Chi-square test and rank sum test were used to compare the variables between the two groups, including the demographic data, laboratory examination results, clinical data before and after ECMO, and other supportive treatment. Univariate and multivariate Logistic regression models were used to analyze the prognostic risk factors.Results:In these 109 cases, 54 died and 55 survived. Compared with the survival group, the death group had higher incidences of acute kidney injury (AKI) (48.1% (26/54) vs. 21.8% (12/55) , χ2=8.318, P=0.004) and coagulation dysfunction (22.2% (12/54) vs. 7.3% (4/55) , χ2=4.862, P=0.027), and higher rate of renal replacement therapy (48.1% (26/54) vs. 21.8% (12/55) , χ2=9.694, P=0.008) during ECMO support. Logistic regression analysis showed that continuous renal replacement therapy (CRRT) and AKI were independent risk factors for death in patients with severe PARDS requiring ECMO support ( HR=3.88,95% CI 1.04-14.52, HR=4.84,95% CI 1.21-19.46, both P<0.05). Conclusion:AKI and CRRT are independent risk factors for predicting mortality in patients with severe PARDS requiring ECMO support.