Objective:To investigate long-term auditory changes and characteristics of Alport syndrome（AS） patients with different degrees of renal injury. Methods:Retrospectively analyzing clinical data of patients diagnosed AS from January 2007 to September 2022, including renal pathology, genetic detection and hearing examination. A long-term follow-up focusing on hearing and renal function was conducted. Results:This study included 70 AS patients, of which 33（25 males, 8 females, aged 3.4-27.8 years） were followed up, resulting in a loss rate of 52.9%.The follow-up period ranged from 1.1to 15.8 years, with 16 patients followed-up for over 10 years. During the follow-up, 10 patients presenting with hearing abnormalities at the time of diagnosis of AS had progressive hearing loss, and 3 patients with new hearing abnormalities were followed up, which appeared at 5-6 years of disease course. All of which were sensorineural deafness. While only 3 patients with hearing abnormalities among 13 patients received hearing aid intervention. Of these patients,7 developed end-stage renal disease（ESRD）, predominantly males （6/7）. The rate of long-term hearing loss was significantly different between ESRD group and non-ESRD group（P=0.013）. There was no correlation between the progression of renal disease and long-term hearing level（P>0.05）. kidney biopsies from 28 patients revealed varying degrees of podocyte lesion and uneven thickness of basement membrane. The severity of podocyte lesion was correlated with the rate of long-term hearing loss（P=0.048）, and there was no correlation with the severity of hearing loss（P>0.05）. Among 11 cases, theCOL4A5mutationwas most common （8 out of 11）, but there was no significant correlation between the mutation type and hearing phenotype（P>0.05）. Conclusion:AS patients exhibit progressive hearing loss with significant heterogeneity over the long-term.. THearing loss is more likely to occur 5-6 years into the disease course. Hearing abnormalities are closely related to renal disease status, kidney tissue pathology, and gene mutations, emphasizing the need for vigilant long-term hearing follow-up and early intervention.
Male ; Child ; Female ; Humans ; Nephritis, Hereditary/pathology* ; Retrospective Studies ; Kidney ; Deafness ; Hearing Loss/genetics* ; Kidney Failure, Chronic/pathology* ; Mutation

Objective:To analyze the factors affecting the quality of cardiopulmonary resuscitation (CPR) performed by medical staff in hospital and to explore the training methods to enhance their in-hospital emergency response capabilities.Methods:A cross-sectional study was conducted, involving medical staff of intensive care unit (ICU) and general internal medicine wards in China-Japan Friendship Hospital in December 2021. The American Heart Association (AHA) resuscitation quality improvement (RQI) model was used to evaluate the skills of the subjects in performing external chest compressions and bag-mask ventilation on adult and infant simulators. While ICU subjects were undergoing RQI model objective assessment, two instructors also provided subjective scoring for their operations. The study compared the differences in RQI model objective assessment scores between ICU and general internal medicine ward subjects, between doctors and nurses, in the RQI model objective scoring for adult and infant resuscitation, in the scoring differences of different positions for chest compressions, and the differences between traditional subjective scoring and RQI objective scoring when ICU subjects were assessed for compression and ventilation.Results:A total of 75 medical staffs were enrolled, consisting of 50 from the ICU (including 24 doctors and 26 nurses) and 25 from the general internal medicine wards (including 10 doctors and 15 nurses). The ICU medical staff's scores for adult resuscitation skills were significantly higher than those of the general internal medicine ward medical staff [adult compression score: 82.5 (66.0, 96.5) vs. 65.0 (52.5, 74.5), adult ventilation score: 82.0 (68.8, 98.0) vs. 61.0 (48.0, 82.0), both P < 0.01]. The nursing group's compression scores for both adult and infant were significantly higher than those of the doctor group [adult compression score: 77.0 (68.5, 89.5) vs. 63.0 (40.8, 90.3), infant compression score: 54.4±25.1 vs. 41.5±18.5, both P < 0.05]. The compression and ventilation scores for the infant were significantly lower than those for adult resuscitation [compression score: 48 (29, 65) vs. 76 (58, 90), ventilation score: 56 (42, 75) vs. 76 (60, 96), both P < 0.01]. When the rescuer was positioned on the right side of the model, the compression score for the adult significantly increased [79.0 (65.0, 92.0) vs. 65.0 (51.3, 77.0), P < 0.05]. The ICU medical staff's traditional subjective scores of compression and ventilation assessments for adult were significantly higher than the RQI model objective scores [adult compression score: 88.8 (79.4, 92.5) vs. 82.5 (66.0, 95.5), adult ventilation score: 95.0 (80.0, 98.1) vs. 82.0 (68.8, 98.0), both P < 0.01]. Conclusions:Rich experience in emergency rescue is related to the improvement of CPR skills, and performing chest compressions from the right side of the adult model is more effective. Objective scoring of resuscitation skills based on the RQI model may more accurately reflect the performance of the trainees.

Objective:To investigate the accuracy and reproducibility of diaphragmatic excursion (DE) measurements through hepato-renal/spleno-renal section as a novel method for assessing diaphragmatic function.Methods:Twelve healthy participants were recruited. Each participant underwent DE measurements performed by four operators with varying levels of experience using traditional methods (liver/spleen section) and novel methods (hepato-renal/spleno-renal section), respectively. Among them, two experienced operators were critical care clinicians, and diaphragmatic ultrasound was performed in more than 50 cases. The other two inexperienced operators were respiratory therapists, with less than 10 cases of diaphragmatic ultrasound operations, who received a 2-hour theoretical and operational training before the study. Operators initially used the conventional method with a 1.5-6.0 MHz convex probe in M-mode, placing the sampling line perpendicular to the diaphragm at the point of maximum excursion, and the liver/spleen section DE was determined during normal breathing of participant. Then, they used the novel method with a 1.6-4.5 MHz phased array probe to observe diaphragmatic movement cranio-caudally along the mid-axillary line, employing anatomic M-mode with the sampling line placed perpendicular to the diaphragm at the level of the renal midpoint, and the DE of the hepato-renal/spleno-renal section was measured during normal breathing. The liver and hepato-renal sections were used to assess the right diaphragm, and spleen and spleno-renal sections were used to assess the left diaphragm. Correlation analysis of DE measurements from different sections was conducted using the Deming method, while consistency was assessed using the Bland-Altman method. The consistency of clinical acceptability was defined as the absence of fixed and proportional bias, with a difference of two standard deviations less than 40% of the mean measurement value. Percentage consistency limit = two standard deviations of the differences between measurements/mean measurement value×100%.Results:Four operators performed image scans of DE in all four sections for each of the twelve subjects, with a high DE acquisition rate of 100% (48/48) for hepato-renal and spleno-renal sections, followed by the liver section [91.7% (44/48)] and the spleen section [66.7% (32/48)], particularly for the left diaphragm assessment, where the DE acquisition rate of spleno-renal section was significantly higher than that of traditional spleen section ( P < 0.01). The overall measurement results showed that no significant difference was found in DE determined via the hepato-renal and spleno-renal sections using the novel method (cm: 1.64±0.10 vs. 1.55±0.14, P > 0.05), and they were significantly higher than those determined via the conventional liver and spleen sections (cm: hepato-renal section vs. liver section was 1.64±0.10 vs. 1.44±0.09, spleno-renal section vs. spleen section was 1.55±0.14 vs. 1.09±0.14, both P < 0.01). Correlation analysis revealed good correlations of DE between hepato-renal section and spleno-renal section, between liver section and hepato-renal section, between liver section and spleno-renal section ( r values were 0.62, 0.59, and 0.42, all P < 0.01). Consistency analysis showed that the consistency in DE between hepato-renal section and spleno-renal section, as well as between liver section and hepato-renal section was good (both % consistency limits < 40%). However, the DE measured in the spleen section were not correlated with the other three sections, and there was no inconsistency (all % consistency limits > 40%). There was no statistically significant difference in DE measured by the four operators in the liver, spleen, hepato-renal, and spleno-renal sections (cm: 1.49±0.34, 1.44±0.37, 1.43±0.30, and 1.40±0.27 in liver section; 1.10±0.36, 1.05±0.18, 1.09±0.22, and 1.06±0.26 in spleen section; 1.67±0.43, 1.57±0.34, 1.63±0.32, and 1.66±0.36 in hepato-renal section; 1.45±0.33, 1.48±0.34, 1.50±0.24, and 1.65±0.26 in spleno-renal section; all P > 0.05). According to the clinically acceptable range of consistency limits, the DE measured by the four operators in all four sections showed good consistency (all % consistency limits < 40%). Conclusion:The novel method of measuring DE through hepato-renal/spleno-renal sections is accurate, highly reproducible, and has a high acquisition rate, serving as a viable alternative to the conventional method involving the liver/spleen section.

In order to explore the role of pre-basic course teaching in the standardized training of residents in clinical pathology, we have independently designed and constructed a pre-basic course teaching system mainly focusing on anatomy and histoembryology, consisting of two levels of theoretical teaching (small lectures by students and systematic lectures by instructors) and two dimensions of practical training (sample collection teaching and case teaching). This teaching model centering on participatory lecturing, practice, summarization, assessment, and feedback has been demonstrated effective. The results showed that the theoretical and practical assessment scores of the experimental group [(458.80±17.60) and (415.40±19.30), respectively] were significantly higher than those of the control group [(444.50±20.90) and (398.80±23.70), respectively]. Among 28 students of grades 2019 to 2021 surveyed for teaching effectiveness, 96.43% were satisfied with the teaching model. The established teaching model provides new ideas for the reform of teaching methods in the standardized training of residents in clinical pathology.

Objective To summarize the most robust evidence on application timing and management strategies for chronic wound oxygen therapy and to provide an evidence-based basis for standardizing clinical practice.Methods A systematic literature search of BMJ Best Practice,UpToDate,NICE,SIGN,GIN,NZGG,NGC,RNAO,WOCN,WUWHS,APWCA,IWII,WHS,WI,AWMA,WCET,ESVS,AAWC,Medlive,JBI,Cochrane Library,PubMed,Embase,Web of Science,CNKI,Wanfang and VIP was conducted to collect the literature including clinical decision resources,guidelines,expert consensuses,evidence summaries,systematic reviews,and meta-analyses related to the application timing and management strategies for chronic wound oxygen therapy.The search period spanned from October 13,2018,to October 13,2023.There were 2 researchers who assessed the quality of the included literature,extracted relevant data,and synthesized the evidence.Results In total,we included 28 pieces of literature,consisting of 6 guidelines,4 expert consensus papers,3 evidence summaries,and 15 systematic reviews.From these sources,we distilled 18 pieces of evidence including 7 aspects of indications,contraindications,application timing,assessment,management strategies,effectiveness,and adverse reactions of hyperbaric oxygen therapy;12 pieces of evidence from 6 aspects of indications,application time,assessment,management strategies,effectiveness,and adverse reactions of local oxygen therapy.Conclusion Oxygen therapy can be used as an adjunct therapy for chronic wound management.Clinical practitioners should fully evaluate the scope and timing of the application of oxygen therapy,consider the feasibility,suitability,clinical signific ance,and effectiveness of evidence based on the specific clinical situations,and apply evidence in combination with patient preferences to promote chronic wound healing.

Stimulator of interferon genes (STING) is a transmembrane protein located on the endoplasmic reticulum that plays an important role in innate immune regulation. After cerebral ischemia, STING exacerbates neuronal death by activating autophagy, triggering inflammation, and inducing oxidative stress. It can also destroy the integrity of the blood-brain barrier via neutrophil extracellular traps (NETs), thereby exacerbating ischemic brain injury. Therefore, elucidating the biological characteristics of STING and its role and mechanism in cerebral ischemia is of great significance for the development of therapeutic drugs for cerebral ischemia.

Objective:To elucidate the epidemiological characteristics and changing trends of liver failure in order to provide evidence-based strategies for prevention and treatment.Methods:The epidemiological information of inpatients with liver failure admitted and treated at Beijing You'an Hospital from 2012 to 2021 was retrospectively collected. The trend test was used to analyze age, gender, as well as the year-by-year changes in the underlying acute and chronic etiology of acute liver failure (ALF), sub-acute liver failure (SALF), acute-on-chronic liver failure (ACLF), and chronic liver failure (CLF).Results:During the study period, information on a total of 8512 inpatients, aged 51.3±13.5 years and mainly male (71.9%) with liver failure, was collected. The highest to lowest proportions of liver failure types were ACLF 4 023 (47.3%), CLF 3 571(42.0%), SALF 670 (7.9%), and ALF 248 (2.9%). The top five causes of liver failure in the overall population, accounting for 87.6% of the total, were hepatitis B 3 199 (37.58%), alcoholic liver disease 2 237 (26.28%), cryptogenic liver disease 906(10.61%), hepatitis B + alcoholic liver disease 603 (7.08%), drugs 488 (5.73%), The top three etiologies of patients with different types of liver failure were acute etiologies for acute liver failure (ALF), followed by drugs 107 (43.1%), hepatitis B 47(19.0%), and unknown etiology 36 (14.5%); sub-acute liver failure (SALF), followed by drugs 381(56.9%), unknown etiology 106 (15.8%), and sepsis 56 (8.4%); and acute-on-chronic liver failure (ACLF), followed by drugs 2 092(52.0%), alcoholic liver disease 813(20.2%), and cryptogenic liver disease 398(9.9%); and chronic etiologies for chronic liver failure (CLF), followed by alcoholic liver disease 1 410(39.5%), hepatitis B 1 028(28.8%), and cryptogenic liver disease 364(10.2%). Longitudinal analysis showed that the average age of patients with liver failure increased year by year, but the sex ratio trend did not change significantly, with male patients predominating throughout. The proportion of drug-induced liver failure in patients with ALF and SALF increased year by year, and the difference in the trend test was statistically significant ( P < 0.05). The proportion of patients with chronic etiologies of ACLF and CLF decreased year by year among hepatitis B, while the proportion of alcoholic liver disease, autoimmune liver disease, and cryptogenic liver disease increased year by year (the difference was statistically significant, P < 0.05). Conclusion:The etiological spectrum of liver failure is changing in our country. Although hepatitis B is still the main cause of liver failure, its proportion shows a decreasing trend year by year, with the exception of ACLF, which is no longer the primary etiology of other types of liver failure, while drug-induced liver disease, alcoholic liver disease, autoimmune liver disease, and cryptogenic liver disease are increasing year by year and will become the focus of liver disease prevention and treatment in the future.

Objective:To summarize initial experience of applying nanopore third-generation sequencing detection method (nanopore sequencing) for genetic diagnosis of non-classical 21 hydroxylase deficiency (NC 21-OHD), and to explore its performance and application prospects.Methods:Clinical data of the two NC 21-OHD patients, who were hospitalized at the First Affiliated Hospital of Zhengzhou University in May 2019, were collected. Peripheral venous blood was collected and genome DNA extracted. Genetic variants was detected by nanopore sequencing and underwent bioinformatic analysis. Pathogenetic mutations in CYP21A2 gene were validated with PCR-sanger sequencing in the two patients and their parents.Results:The average reads length and sequence depth in the patient one was 12, 792 bp and 27.19×. The average reads length and sequence depth in the patient two was 13, 123 bp and 21.34×. Compound variants of c.293-13C>G/c.844G>T (p.Val282Leu) and c.332_339delGAGACTAC (p.Gly111Valfs)/c.844G>T (p.Val282Leu) were detected in these two patients, which were consistent with clinical phenotype of NC 21-OHD. Further analysis showed that c.293-13C>G mutation was inherited from her father and c.844G>T (p.Val282Leu) mutation was inherited from her mother for the patient one. The c.844G>T (p.Val282Leu) mutation was inherited from her father and c.332_339delGAGACTAC (p.Gly111Valfs) mutation from her mother.Conclusions:The heterozygous mutations in CYP21A2 gene are the cause of NC 21-OHD in these two patients. Nanopore sequencing technique is a reliable new detection method for patients with NC 21-OHD.

Objective:To explore the effect of emergency rescue ability training of operating room nurses based on video tracking method, and provide reference for improving emergency ability of operating room nurses.Methods:This study was a quasi-experimental study. In March 2021, 85 nurses working in the Department of Anesthesiology and Surgery of the Provincial Hospital Affiliated to Shandong First Medical University were selected by cluster sampling method. We implemented a training program of rescue emergency ability based on video tracking and adopted the inspection list of rescue emergency ability assessment and doctors′ satisfaction questionnaire of operating room nurses to evaluate the scores of each item in the nurses' rescue emergency ability list and doctors' satisfaction of rescue cooperation of operating room nurses.Results:The scores of the dimensions of recognition of fatal arrhythmias, fatal arrhythmia, several situations that can directly call help, writing of emergency state nursing records and handover of medical records, use of defibrillator, correct use and maintenance of negative pressure attraction, use and management of rescue vehicle and situation disposal in the inspection list of rescue ability after training were 9.80 ± 1.61, 26.06 ± 2.20, 17.34 ± 1.29, 13.00 ± 1.57, 7.35 ± 0.74, 10.24 ± 1.14, 33.89 ± 2.73, which were higher than before training 9.24 ± 1.18, 24.92 ± 2.15, 15.69 ± 1.92, 12.21 ± 1.66, 6.55 ± 0.92, 8.94 ± 1.32, 32.94 ± 2.20. The differences were statistically significant ( t values were -6.83 to -2.51, all P<0.05); after the training, in the questionnaire of doctors' satisfaction with nurses, surgical materials and instruments preparation, first aid skill operating level, attention to surgical progress, active and correct delivery, orderly and busy, coordination and communication ability, professional knowledge, evaluation ability and foresight, ability to deal with emergencies, clear division of labor and good cooperation, and responsibility scores were 4.22 ± 0.58, 4.52 ± 0.54, 4.53 ± 0.47, 4.43 ± 0.58, 4.44 ± 0.44, 4.37 ± 0. 59, 4.45 ± 0.51, 4.51 ± 0.53, 4.51 ± 0.57, 4.17 ± 0.63, which were higher than the pre-training 4.05 ± 0.58, 4.38 ± 0.56, 4.26 ± 0.76, 4.04 ± 0.67, 4.25 ± 0.62, 4.19 ± 0.74, 4.25 ± 0.74, 4.34 ± 0.67, 4.21 ± 0.84 and 3.56 ± 0.58. All differences were statistically significant ( t values were -8.22 to -2.10, all P<0.05). Conclusions:The training method based on video tracking method improved the emergency rescue ability of operating room nurses and the doctor's satisfaction with rescue cooperation, and provided a reference for the training of operating room nurses.

Objective:To observe the expression changes of microRNA(miR)-122 in liver tissue of rats infected with Clonorchis sinensis and its correlation with expression level of inflammatory cytokines. Methods:Totally 24 SPF grade Wistar male rats were selected and randomly divided into a control group (100 μl physiological saline gavage), a 4-week infection group (100 Clonorchis sinensis metacercariae gavage), and an 8-week infection group (100 Clonorchis sinensis metacercariae gavage) based on body weight (100-120 g) using a random number table method, with 8 rats in each group. Starting from the third week of infection, rat feces were collected and directly smeared with physiological saline for identification of Wistar rat animal models infected with Clonorchis sinensis. After 4 and 8 weeks of infection, the rats in the 4- and 8-week infection groups were euthanized, while 4 rats in the control group were euthanized, respectively. The heart blood and left lobe liver tissue and serum samples were collected from each group of rats. Using hematoxylin-eosin (HE) staining to observe liver pathological damage under the light microscope, real-time fluorescence quantitative PCR to detect the expression level of miR-122 in liver tissue, and Luminex 200 liquid suspension chip to detect the expression levels of serum inflammatory cytokines [tumor necrosis factor-α(TNF-α), interleukin-1β(IL-1β), interleukin-6 (IL-6)]. The correlation between miR-122 and inflammatory cytokines was analyzed using Pearson correlation. Results:Under the light microscope, the morphology of hepatocytes in control group was normal, and no inflammatory cell infiltration was observed. There was inflammatory cells such as lymphocyte, eosinophil and other inflammatory cell infiltration around the portal area in the 4-week infection group. The hepatocytes of the 8-week infected rats were arranged in a disordered manner, with varying degrees of swelling, loose and lightly stained cytoplasm, and some hepatocytes showed watery degeneration; additionally, bile duct dilation and thickening of the bile duct wall were observed in the liver tissue. There were statistically significant differences of liver miR-122 (1.00 ± 0.32, 2.57 ± 0.60, 3.63 ± 1.63), serum TNF-α [(0.14 ± 0.06), (0.43 ± 0.09), (0.61 ± 0.10) ng/ml], and IL-6 expression levels [(0.03 ± 0.01), (1.06 ± 0.24), (1.48 ± 0.33) ng/ml] in control group, 4- and 8-week infection groups ( F = 13.36, 69.99, 82.23, P < 0.001). There was no statistically significant difference in expression level of IL-1β between different groups ( F = 2.15, P = 0.141). The Pearson correlation analysis showed that the expression level of miR-122 was positively correlated with the expression levels of inflammatory cytokines TNF-α and IL-6 ( r = 0.67, 0.80, P < 0.001). Conclusion:Clonorchis sinensis infection can increase the expression of miR-122 in the host liver tissue, and the miR-122 is closely related to the expression levels of inflammatory cytokines TNF-α and IL-6.