Objective To explore the application effect of prone position ventilation combined with veno-venous extracorporeal membrane oxygenation (VV-ECMO) in the treatment of severe primary graft dysfunction (PGD) after lung transplantation. Methods The clinical data of 75 lung transplant recipients who developed severe PGD after lung transplantation and were treated with VV-ECMO from January 2021 to June 2024 at Wuxi People's Hospital Affiliated to Nanjing Medical University were collected. The patients with severe graft dysfunction after lung transplantation were divided into VV-ECMO group (control group, 45 cases) and prone position ventilation combined with VV-ECMO group (treatment group, 30 cases). The general data of the two groups of patients were compared, including the donors' clinical data (age, gender and oxygenation index, etc) and the recipients' clinical data [gender, age and body mass index (BMI), etc]. Cox regression analysis was used to analyze the influencing factors of the recipients' 30-day, 90-day and 180-day survival after surgery. The survival curves of the two groups of recipients were drawn using Kaplan-Meier method and compared using the log-rank test. Results The intensive care unit (ICU) stay time, ECMO application time and ventilator use time of control group were longer than those of treatment group. The proportion of male recipients and the BMI of control group were lower than those of treatment group. The 30-day, 90-day and 180-day survival of control group was worse than that of treatment group, and the differences were statistically significant (all P<0.05). The univariate Cox regression analysis of the recipients' 30-day survival after surgery showed that the recipients' BMI, history of diabetes, enlargement of the right atrium and right ventricle, intraoperative blood transfusion volume and intraoperative red blood cell transfusion volume were statistically significant (all P<0.05). The multivariate Cox regression analysis showed that the history of diabetes and enlargement of the right atrium and right ventricle were risk factors affecting the 30-day survival of lung transplant recipients (all P<0.05). The univariate Cox regression analysis of the recipients' 90-day survival after surgery showed that the recipients' BMI, history of diabetes, enlargement of the right atrium and right ventricle, intraoperative blood transfusion volume, intraoperative red blood cell transfusion volume and group variable were statistically significant (all P<0.05). The multivariate Cox regression analysis showed that the history of diabetes, enlargement of the right atrium and right ventricle and group variable were risk factors affecting the 90-day survival of lung transplant recipients (all P<0.05). The univariate Cox regression analysis of the recipients' 180-day survival after surgery showed that the recipients' BMI, history of diabetes, right atrium and right ventricle enlargement, intraoperative blood transfusion volume, intraoperative red blood cell transfusion volume and group variable were statistically significant (all P<0.05). The multivariate Cox regression analysis showed that the history of diabetes, enlargement of the right atrium and right ventricle and group variable were risk factors affecting the 180-day survival of lung transplant recipients (all P<0.05). The 30-day, 90-day and 180-day survival rates of control group were lower, and the differences between the two groups were statistically significant (all P<0.05), with a median survival time of 100 days in control group. Conclusions In the clinical treatment of severe PGD after lung transplantation, prone position ventilation combined with VV-ECMO may shorten ECMO application time, invasive ventilation time and ICU stay time, and improve the short-term prognosis of lung transplantation.

BACKGROUND: The available evidence regarding the benefits of percutaneous coronary intervention (PCI) on patients receiving dialysis with coronary artery disease (CAD) is limited and inconsistent. This study aimed to evaluate the association between PCI and clinical outcomes as compared with medical therapy alone in patients undergoing dialysis with CAD in China. METHODS: This multicenter, retrospective study was conducted in 30 tertiary medical centers across 12 provinces in China from January 2015 to June 2021 to include patients on dialysis with CAD. The primary outcome was major adverse cardiovascular events (MACE), defined as a composite of cardiovascular death, non-fatal myocardial infarction, and non-fatal stroke. Secondary outcomes included all-cause death, the individual components of MACE, and Bleeding Academic Research Consortium criteria types 2, 3, or 5 bleeding. Multivariable Cox proportional hazard models were used to assess the association between PCI and outcomes. Inverse probability of treatment weighting (IPTW) and propensity score matching (PSM) were performed to account for potential between-group differences. RESULTS: Of the 1146 patients on dialysis with significant CAD, 821 (71.6%) underwent PCI. After a median follow-up of 23.0 months, PCI was associated with a 43.0% significantly lower risk for MACE (33.9% [ n  = 278] vs . 43.7% [ n  = 142]; adjusted hazards ratio 0.57, 95% confidence interval 0.45-0.71), along with a slightly increased risk for bleeding outcomes that did not reach statistical significance (11.1% vs . 8.3%; adjusted hazards ratio 1.31, 95% confidence interval, 0.82-2.11). Furthermore, PCI was associated with a significant reduction in all-cause and cardiovascular mortalities. Subgroup analysis did not modify the association of PCI with patient outcomes. These primary findings were consistent across IPTW, PSM, and competing risk analyses. CONCLUSION This study indicated that PCI in patients on dialysis with CAD was significantly associated with lower MACE and mortality when comparing with those with medical therapy alone, albeit with a slightly increased risk for bleeding events that did not reach statistical significance.
Humans ; Percutaneous Coronary Intervention/methods* ; Male ; Female ; Coronary Artery Disease/drug therapy* ; Retrospective Studies ; Renal Dialysis/methods* ; Middle Aged ; Aged ; China ; Proportional Hazards Models ; Treatment Outcome

AIM: To evaluate the clinical efficacy of corneal stromal lenticule-combined accelerated transepithelial corneal collagen cross-linking(SC-A-TE-CXL)in the treatment of severe keratoconus.METHODS: Prospective before-after self-control study. A total of 10 cases(14 eyes)of severe keratoconus with the thinnest corneal thickness(including epithelium)less than 400 μm were collected from March 2019 to July 2022 at the ophthalmology department of Affiliated Eye Hospital of Nanjing Medical University. Among them, 8 males(12 eyes)and 2 females(2 eyes)were treated with SC-A-TE-CXL. Corneal curvature, uncorrected visual acuity(UCVA), best corrected visual acuity(BCVA), the thinnest corneal thickness(TCT), central corneal thickness(CCT), non-contact intraocular pressure, endothelial cell density(ECD)and anterior or posterior elevations at the thinnest point before surgery and at 1, 3, 6 and 12 mo postoperatively were observed and recorded, as well as corneal cross-linking depth at 1 mo postoperatively.RESULTS: UCVA and BCVA at 1, 3, 6, and 12 mo after SC-A-TE-CXL were higher than those preoperatively, but there were no differences(F=0.793, P=0.535; F=0.783, P=0.542). K1, K2, Km and Kmax decreased at each time point postoperatively compared with those preoperatively, but there were no differences(F=0.627, P=0.574; F=1.264, P=0.296; F=0.727, P=0.520; F=1.115, P=0.359). Anterior and posterior elevations at the thinnest point both decreased compared with those preoperatively, but the differences were not statistically significant(F=1.046, P=0.359; F=1.164, P=0.337). The non-contact intraocular pressure at each time point postoperatively was higher than that preoperatively, but the differences were not statistically significant(F=0.814, P=0.522). There were no differences in CCT and TCT at any time points of the follow-ups compared with those preoperatively(F=0.931, P=0.453; F=0.782, P=0.542). There was no difference in ECD at 12 mo postoperatively versus preoperative value(t=1.266, P=0.228). At 1 mo postoperatively, anterior segment optical coherence tomography(AS-OCT)exhibited an increase of density in the anterior stroma, and there was a demarcation line with an average depth of 124.07±25.13 μm.CONCLUSION: SC-A-TE-CXL can be considered as a surgical treatment for severe keratoconus, which can delay the progression of severe keratoconus with high safety. However, the long-term efficacy of this treatment requires further observation.

Here, we report a previously unrecognized syndromic neurodevelopmental disorder associated with biallelic loss-of-function variants in the RBM42 gene. The patient is a 2-year-old female with severe central nervous system (CNS) abnormalities, hypotonia, hearing loss, congenital heart defects, and dysmorphic facial features. Familial whole-exome sequencing (WES) reveals that the patient has two compound heterozygous variants, c.304C>T (p.R102*) and c.1312G>A (p.A438T), in the RBM42 gene which encodes an integral component of splicing complex in the RNA-binding motif protein family. The p.A438T variant is in the RRM domain which impairs RBM42 protein stability in vivo. Additionally, p.A438T disrupts the interaction of RBM42 with hnRNP K, which is the causative gene for Au-Kline syndrome with overlapping disease characteristics seen in the index patient. The human R102* or A438T mutant protein failed to fully rescue the growth defects of RBM42 ortholog knockout ΔFgRbp1 in Fusarium while it was rescued by the wild-type (WT) human RBM42. A mouse model carrying Rbm42 compound heterozygous variants, c.280C>T (p.Q94*) and c.1306_1308delinsACA (p.A436T), demonstrated gross fetal developmental defects and most of the double mutant animals died by E13.5. RNA-seq data confirmed that Rbm42 was involved in neurological and myocardial functions with an essential role in alternative splicing (AS). Overall, we present clinical, genetic, and functional data to demonstrate that defects in RBM42 constitute the underlying etiology of a new neurodevelopmental disease which links the dysregulation of global AS to abnormal embryonic development.
Female ; Animals ; Mice ; Humans ; Child, Preschool ; Intellectual Disability/genetics* ; Heart Defects, Congenital/genetics* ; Facies ; Cleft Palate ; Muscle Hypotonia

ObjectiveTo explore the exposure-lag-response relationship between temperature and risk of varicella incidence，and to provide a scientific evidence for early warning and precise prevention and control of varicella epidemic. MethodsDaily varicella cases and daily meteorological data were collected in Minhang District， Shanghai from 2010 to 2022. A distributed lag nonlinear model was used to determine the exposure-lag-response relationship between temperature and risk of varicella incidence. Furthermore， effect of temperature on the incidence risk was determined across different age groups. ResultsIn 2010‒2022， the total number of notified varicella cases was 26 207 in Minhang District， with the highest incidence in the group aged 3‒14 years （50.35%）. The seasonal pattern of daily varicella cases showed a double peak. The large peak was found in November and December， followed by a smaller peak in May and June. Moreover， the distributed lag nonlinear model showed a unimodal curve in the relationship between temperature and varicella incidence. The RR value reached its maximum peak of 1.90 （95%CI： 1.25‒2.87） at 7 ℃. A reverse U-shape was found in the lag-response curves between temperature and varicella incidence. Furthermore， the effect of temperature on the varicella incidence showed a unimodal pattern in the varicella cases aged 3‒14 years. The RR value reached its peak at 11 ℃ （RR=2.89， 95%CI： 1.33‒6.24）. In contrast， the effect of temperature on the varicella incidence in the cases aged 15 years and above showed a unimodal pattern， with RR value reaching the peak at 5 ℃ （RR=2.14， 95%CI： 1.33‒3.44）. ConclusionThe unimodal curve is found in the relationship between temperature and varicella incidence. Low temperature is associated with increased risk of varicella incidence. Children aged 3‒14 years are more susceptible to the effect of temperature on the varicella incidence.

Objective:To compare the clinical efficacy between a single palmar lateral transverse approach and the modified Henry approach in the locking plate fixation for distal radius fractures.Methods:A retrospective study was conducted to analyze the data of 82 patients with distal radius fracture who had undergone locking plate fixation through either a single palmar lateral approach or the modified Henry approach between January 2016 and December 2022 at Department of Traumatic Orthopaedics, Taihe Hospital, Affiliated to Hubei University of Medicine. There were 25 males and 57 females, with an age of (53.8±12.7) years. Based on the difference in surgical approach, the patients were divided into a single transverse approach group ( n=42) and a modified Henry approach group ( n=40). The 2 groups were compared in terms of injury cause, injury location, time from injury to surgery, AO fracture classification, tourniquet time during surgery, incision length, fracture reduction, Patient and Observer Scar Assessment Scale (PSAS & OSAS) and Disability of the Arm, Shoulder, and Hand (DASH) score at the last follow-up, and complications after surgery. Results:There were no statistically significant differences in the baseline characteristics between the 2 groups, indicating comparability ( P>0.05). All patients were followed up for (8.2±3.5) months. Follow-ups revealed one case of injury to the palmar cutaneous branch of the median nerve but no other complications like infection, non-union, internal fixation failure, or tendon injury in the single transverse approach group. The single transverse approach group was significantly superior over the modified Henry approach group in incision length [(2.4±0.9) cm versus (5.3±1.6) cm], OSAS (8.1±4.2 versus 10.3±5.7), and PSAS (10.1±5.8 versus 14.7±6.4) ( P<0.05). There were no significant differences between the 2 groups in tourniquet time, fracture reduction quality, and DASH score at the last follow-up ( P>0.05). Conclusion:In locking plate fixation for distal radius fractures, in comparison with the modified Henry approach, a single palmar lateral approach is more minimally invasive so that more aesthetically pleasing outcomes can be achieved to facilitate patients' rapid return to work and society.

17q12 microdeletion syndrome is a rare genetic disease,commonly characterized by newly occurring mutations,which can cause abnormalities of the urinary and reproductive tract,diabetes mellitus,neurological and psychiatric disorders and mild deformities.This article reports a case of 17q12 microdeletion syndrome with CRYBB2 gene missense mutation,combined with menstrual abnormalities,multiple cysts in both kidneys,hypomagnesemia,hyperuricemia,small pancreatic morphology and low pancreatic enzyme levels.

Objective:To determine incidence of seasonal influenza virus infection in the community and to analyze the factors influencing seasonal influenza virus infection.Methods:This study recruited residents aged 6-59 years to build a cohort in 15 villages/streets in Macheng city in November 2019. Meanwhile, a cross-sectional baseline survey was conducted immediately to collect sera, information on demographics and child protection knowledge, behaviors, as well as attitudes using a questionnaire from the participants enrolled in the cohort (i.e., before the influenza epidemic season). In July 2020, a cross-sectional follow-up survey was conducted to collect sera once again (i.e., after the influenza season). Paired sera from the two cross-sectional surveys were tested for influenza virus-specific antibodies by hemagglutination inhibition (HI) test or micro-neutralization (MN) test using a circulating representative strain of each subtype/lineage of influenza virus as the test antigen. The infections with influenza virus subtype/lineage was confirmed if there was a four-fold or more increase in titers of antibodies against circulating representative strain of the subtype/lineage of influenza virus. Factors influencing infection with influenza A (H3N2) and B/Victoria viruses were analyzed using univariable and multivariable logistic regression.Results:In November 2019, 800 study participants were enrolled in the cohort, including 340 children aged 6-17 years and 460 adults aged 18-59 years; 605 study participants (including 224 children and 381 adults) were followed up in July 2020 and their paired sera were obtained before and after the influenza season. 25.3% (153/605) of the participants were confirmed to be infected with at least one subtype/lineage of seasonal influenza virus by HI and MN tests. The overall incidence of influenza viruses of all subtypes/lineages in children was 44.2% (95% CI: 37.6%-50.8%) which was significantly higher than the incidence of 14.1% in adults (95% CI: 10.7%-17.7%). Children had the highest incidence of influenza A (H3N2) virus infection, followed by B/Victoria. MN or HI antibody titers in A (H3N2)[ OR=0.88 (95% CI: 0.84-0.93)] and B/Victoria[ OR=0.97 (95% CI: 0.95-0.99)] before the influenza season were significantly associated with whether children were infected with that subtype/lineage of influenza virus. Conclusions:The residents aged 6-59 years in Macheng city had a substantial incidence of seasonal influenza virus infection during the influenza season from winter 2019 to spring 2020. Notably, almost half of children aged 6-17 years have been infected with seasonal influenza virus. Higher titers of HI/MN antibodies against seasonal influenza virus before the influenza season would be likely to reduce the risk of infection with influenza A (H3N2) and B/Victoria.

Background::Carotid intima-media thickness (IMT) and diameter, stiffness, and wave reflections, are independent and important clinical biomarkers and risk predictors for cardiovascular diseases. The purpose of the present study was to establish nationwide reference values of carotid properties for healthy Chinese adults and to explore potential clinical determinants.Methods::A total of 3053 healthy Han Chinese adults (1922 women) aged 18-79 years were enrolled at 28 collaborating tertiary centers throughout China between April 2021 and July 2022. The real-time tracking of common carotid artery walls was achieved by the radio frequency (RF) ultrasound system. The IMT, diameter, compliance coefficient, β stiffness, local pulse wave velocity (PWV), local systolic blood pressure, augmented pressure (AP), and augmentation index (AIx) were then automatically measured and reported. Data were stratified by age groups and sex. The relationships between age and carotid property parameters were analyzed by Jonckheere-Terpstra test and simple linear regressions. The major clinical determinants of carotid properties were identified by Pearson’s correlation, multiple linear regression, and analyses of covariance.Results::All the parameters of carotid properties demonstrated significantly age-related trajectories. Women showed thinner IMT, smaller carotid diameter, larger AP, and AIx than men. The β stiffness and PWV were significantly higher in men than women before forties, but the differences reversed after that. The increase rate of carotid IMT (5.5 μm/year in women and 5.8 μm/year in men) and diameter (0.03 mm/year in both men and women) were similar between men and women. For the stiffness and wave reflections, women showed significantly larger age-related variations than men as demonstrated by steeper regression slopes (all P for age by sex interaction <0.05). The blood pressures, body mass index (BMI), and triglyceride levels were identified as major clinical determinants of carotid properties with adjustment of age and sex. Conclusions::The age- and sex-specific reference values of carotid properties measured by RF ultrasound for healthy Chinese adults were established. The blood pressures, BMI, and triglyceride levels should be considered for clinical application of corresponding reference values.

Objective:To explore the influence of age on the passive stiffness of women′s quadriceps muscles and the relationship between stiffness and muscle functioning.Methods:Twenty healthy elderly women formed the senior-aged group, while 20 young, healthy women were selected as the young group. The passive stiffness of the subjects′ vastus medialis, rectus femoris and vastus lateralis muscles was measured using ultrasound shear wave imaging with the knee flexed to 30°, 60°, 90° and 105°. Both groups completed the 6-minute walk test (6MWT) and the five times sit-to-stand test (FTSST). Maximum voluntary isometric contraction (MIVC) strength during knee extension was also measured.Results:The passive muscle stiffness of the rectus femoris and vastus lateralis muscles was significantly greater in the senior-aged group when the knee was flexed at 60°, 90° or 105°. The 6MWT and FTSST times of the young group were of course significantly better, and their MIVC at 60° and 90° of knee flexion was also significantly stronger. Rectus femoris stiffness among the senior-aged group was negatively correlated with MIVC when the knee was flexed at 60°.Conclusions:The passive stiffness of the muscles studied increases with age, especially in stretching. When the knee joint is flexed at 60° there tends to be a negative correlation between the MIVC of the quadriceps femoris and the stiffness of the rectus femoris among elderly females.