ObjectiveTo analyze the clinical treatment plan and traditional Chinese medicine （TCM） medication rule of children with primary nephrotic syndrome （PNS） in the First Affiliated Hospital of Henan University of Chinese Medicine. MethodsThe gender and age of children firstly diagnosed with nephrotic syndrome in the pediatric nephrology department of the First Affiliated Hospital of Henan University of Chinese Medicine from November 2019 to December 2022 were collected， and the use of immunosuppressive agents and related frequencies were counted. According to the inclusion and exclusion criteria， an independent TCM prescription database for children with nephrotic syndrome was established. Excel was used to analyze the relevant information of the literature. The frequency counting， association rule analysis， and cluster analysis were carried out on TCM in the prescription， and the high-frequent drugs were analyzed. Results（1） General information： A total of 711 children were included， consisting of 522 males （73.42%） and 189 females （26.58%）. The ratio of male to female was about 2.76∶1. The disease mainly occurred in infants and preschool age， and the average age of onset was （4.74 ± 3.48） years old. （2） Clinical treatment plan and use of immunosuppressive agents： Of the 711 children with PNS， 237 were treated with hormone alone （32.33%）， and 474 （66.67%） received immunosuppressive agents combined with hormones. In the initial treatment， hormone combined with Tacrolimus （TAC） was the preferred treatment （32.91%）. For children with refractory PNS who exhibited poor clinical efficacy， Rituximab （RTX） was mostly used for treatment， with a ratio of up to 23.63%. （3） TCM syndrome and medication rule： In PNS syndrome differentiation， Qi and Yin deficiency was identified as the main syndrome. This involved a total of 477 cases， accounting for 67.09%. Yang deficiency of spleen and kidney was observed in 118 cases， accounting for 16.60%. A total of 711 children were included， of which 706 children were treated with TCM. This involved a total of 706 prescriptions， 226 TCM， and 9 793 frequencies. There were 30 herbs used more than 95 times. The top five TCM were Radix et Rhizoma Glycyrrhizae （81.16%）， Radix Astragali （71.81%）， Poria （68.84%）， Rhizoma Atractylodis Macrocephalae （63.60%）， and Fructus Corni （57.37%）. The drug association rules and network diagram showed that the combination of ''Radix Astragali-Rhizoma Atractylodis Macrocephalae-Poria'' was the closest， and five types of combinations were obtained by cluster analysis. ConclusionIn the diagnosis and treatment of PNS in children， TAC combined with hormones shows good clinical efficacy and high safety. For children with refractory PNS， RTX combined with hormones can be used. TCM medication for PNS should follow the basic principles of strengthening the body and vital Qi and make good use of drugs such as Radix Astragali， Poria， Rhizoma Atractylodis Macrocephalae， and cornus to regulate the Yin and Yang balance and achieve better clinical efficacy.

ObjectiveTo analyze the clinical treatment plan and traditional Chinese medicine （TCM） medication rule of children with primary nephrotic syndrome （PNS） in the First Affiliated Hospital of Henan University of Chinese Medicine. MethodsThe gender and age of children firstly diagnosed with nephrotic syndrome in the pediatric nephrology department of the First Affiliated Hospital of Henan University of Chinese Medicine from November 2019 to December 2022 were collected， and the use of immunosuppressive agents and related frequencies were counted. According to the inclusion and exclusion criteria， an independent TCM prescription database for children with nephrotic syndrome was established. Excel was used to analyze the relevant information of the literature. The frequency counting， association rule analysis， and cluster analysis were carried out on TCM in the prescription， and the high-frequent drugs were analyzed. Results（1） General information： A total of 711 children were included， consisting of 522 males （73.42%） and 189 females （26.58%）. The ratio of male to female was about 2.76∶1. The disease mainly occurred in infants and preschool age， and the average age of onset was （4.74 ± 3.48） years old. （2） Clinical treatment plan and use of immunosuppressive agents： Of the 711 children with PNS， 237 were treated with hormone alone （32.33%）， and 474 （66.67%） received immunosuppressive agents combined with hormones. In the initial treatment， hormone combined with Tacrolimus （TAC） was the preferred treatment （32.91%）. For children with refractory PNS who exhibited poor clinical efficacy， Rituximab （RTX） was mostly used for treatment， with a ratio of up to 23.63%. （3） TCM syndrome and medication rule： In PNS syndrome differentiation， Qi and Yin deficiency was identified as the main syndrome. This involved a total of 477 cases， accounting for 67.09%. Yang deficiency of spleen and kidney was observed in 118 cases， accounting for 16.60%. A total of 711 children were included， of which 706 children were treated with TCM. This involved a total of 706 prescriptions， 226 TCM， and 9 793 frequencies. There were 30 herbs used more than 95 times. The top five TCM were Radix et Rhizoma Glycyrrhizae （81.16%）， Radix Astragali （71.81%）， Poria （68.84%）， Rhizoma Atractylodis Macrocephalae （63.60%）， and Fructus Corni （57.37%）. The drug association rules and network diagram showed that the combination of ''Radix Astragali-Rhizoma Atractylodis Macrocephalae-Poria'' was the closest， and five types of combinations were obtained by cluster analysis. ConclusionIn the diagnosis and treatment of PNS in children， TAC combined with hormones shows good clinical efficacy and high safety. For children with refractory PNS， RTX combined with hormones can be used. TCM medication for PNS should follow the basic principles of strengthening the body and vital Qi and make good use of drugs such as Radix Astragali， Poria， Rhizoma Atractylodis Macrocephalae， and cornus to regulate the Yin and Yang balance and achieve better clinical efficacy.

Objective:To explore the pregnancy outcome and postpartum clinical phenotype of LCR22B/C~D central 22q11.2 deletion syndrome.Methods:For fetuses diagnosed with central 22q11.2 deletion by chromosomal microarray analysis (CMA) at the Prenatal Diagnosis Center of the Third Affiliated Hospital of Zhengzhou University from January 2019 to April 2022, their prenatal imaging, parental CMA verification, pregnancy outcomes and postpartum clinical phenotype were analyzed.Results:Eight cases of central 22q11.2 deletion syndrome were included, including six cases with LCR22B~D 22q11.2 deletions and two with LCR22C~D 22q11.2 deletions. Among the six cases with LCR22B~D type 22q11.2 deletions, three had shown cardiovascular malformations (right aortic arch, ventricular septal defect, mild tricuspid regurgitation), one had shown urinary defect (right kidney heterotopia). Two cases with LCR22C~D 22q11.2 deletions showed nonspecific ultrasonographic findings, including oligohydramnios with growth restriction and nuchal skin thickening. The CMA verification showed that six cases were inherited from their parents, and five couples had chosen to continue with the pregnancy. Postpartum follow-up showed that the physical and intellectual development of all children were normal. One couple had opted to terminate the pregnancy considering the ectopic fetal right kidney. Two remaining cases had decided to terminate their pregnancies without parental verification.Conclusion:The central 22q11.2 deletion syndrome of the LCR22B/C~D type is different from the classical types. Its genetic information mainly comes from parents. Prenatal imaging has mainly shown cardiovascular and urinary abnormalities. Postnatal growth and intellectual development have been normal. Therefore, the couples should be provided with suffice prenatal genetic counseling.

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.

Galactinol synthase (GolS) genes play important roles in plant response to abiotic stress. In this research, the plant expression vector of soybean GmGolS2-2 gene was constructed and transformed into tobacco to study the drought tolerance of transgenic tobacco. A GmGolS2-2 gene with 975 bp coding sequence was cloned from soybean leaves by reverse transcription-polymerase chain reaction (RT-PCR). GmGolS2-2 was linked to the plant expression vector pRI101 by restriction enzyme sites Nde Ⅰ and EcoR Ⅰ, and transformed into tobacco by leaf disc method. Genomic DNA PCR and real-time PCR showed that three GmGolS2-2 transgenic tobacco plants were obtained. The growth status of GmGolS2-2 transgenic tobacco under drought stress was better than that of wild-type tobacco. After drought stress treatment, the electrolyte leakage and malondialdehyde content of transgenic tobacco were lower than those of wild-type tobacco, but the proline content and soluble sugar content were higher than those of wild-type tobacco. The results of real-time PCR showed that the heterologous expression of GmGolS2-2 increased the expression of stress-related genes NtERD10C and NtAQP1 in transgenic tobacco. The above results indicated that GmGolS2-2 improved drought resistance of transgenic tobacco.
Drought Resistance ; Tobacco/genetics* ; Soybeans/genetics* ; Plant Proteins/metabolism* ; Plants, Genetically Modified/genetics* ; Stress, Physiological/genetics* ; Droughts ; Gene Expression Regulation, Plant

OBJECTIVE: To explore the clinical phenotypes and genetic diagnosis of 2 sporadic cases for cleidocranial dysplasia. METHODS: The clinical data of two cases of CCD admitted to the Third Affiliated Hospital of Zhengzhou University on December 16, 2021 and December 9, 2021 were analyzed retrospectively, and the whole exome sequencing (WES), chromosome microarray analysis and copy number variation sequencing were performed. RESULTS: The main ultrasonographic findings of the fetus had included poorly calcified skull bones, budging of parieto-occipital area, compression and deformation of skull, and loss of nasal bone. The infant's clinical phenotypes included delayed closure of anterior fontanelle, recurrent respiratory tract infection, growth retardation, and clavicular hypoplasia. By WES analysis, the fetus was found to harbor a heterozygous c.911_914delinsTTT variant of the RUNX2 gene, whilst the infant was found to harbor a heterozygous c.1008delT variant of the RUNX2 gene. Both variants were verified by Sanger sequencing to have occurred de novo. CONCLUSION For sporadic cases featuring cleidocranial dysplasia, prenatal ultrasonography is particularly important. Hypoplastic clavicle, skull calcification and nasal bone absence are the main features. Diagnosis should also be suspected for infants featuring growth retardation, recurrent respiratory tract infections and clavicular dysplasia. The identification of the c.911_914delinsTTT and c.1008delT variants of the RUNX2 gene has facilitated genetic counseling and prenatal diagnosis, and also expanded the mutational spectrum of the RUNX2 gene.
Female ; Humans ; Pregnancy ; Cleidocranial Dysplasia/genetics* ; Core Binding Factor Alpha 1 Subunit ; DNA Copy Number Variations ; Growth Disorders ; Retrospective Studies

Objective:To investigate the feasibility, safety and effectiveness of Da Vinci robotic surgical system in the reoperation of recurrent or residual thyroid cancer.Methods:Retrospective analysis was performed on the clinical data of 9 patients with Da Vinci robot-assisted reoperation for thyroid cancer in the 960th Hospital of the People′s Liberation Army of China from September 2018 to January 2022, the operation time, number of lymph nodes dissected, intraoperative blood loss, length of hospital stay, total postoperative drainage volume, incidence of complications, satisfaction with postoperative aesthetic effect, visual analyogue scale (VAS) score at the 24 h after surgery and number of recurrence during follow-up were counted.Results:The surgery time of 9 cases was (186.67±44.44) min, the number of lymph nodes cleared were (15.77±13.59), intraoperative blood loss was (21.11±16.91) mL, hospital stay were (10.67±3.32) days, total postoperative drainage was (286.94±90.85) mL. There was no complications, and all patients were satisfied with the postoperative cosmetic effect whose VAS score was (8.22±1.09), and VAS score was 0 to 3 (2.44±0.73) points, no recurrence during the follow-up period from 6 to 46 months.Conclusion:With adequate preoperative evaluation and an experienced surgeon team, the use of robots in recurrent or residual thyroid cancer resurgery is feasible, safe and effective.

Objective:To explore the model of first aid skills training based on competition.Method:The second-year residents who participated in the competition in 2021 ( n = 142) were selected. According to whether they attended BLS training in the first year, they were divided into group A ( n = 88) who attended BLS training and group B ( n = 54) who did not. Chi-square test, T test, Wilcoxon rank sum test and Logistic regression were used in our study. Results:There was no significant difference in gender and specialty between both groups (female, 62.5% vs. 68.5%, P= 0.466; TCM, 50% vs. 53.7%, P= 0.668), but the real resuscitation experience of group A was more than that of group B (40.9% vs. 9.3%, P= 0.000). The qualified rate of CPR and the compression score in group A was higher than that in group B [(81.8% vs. 61.1%, P = 0.006; (30±5) vs. (25 ±10), P= 0.001], including compression frequency, depth, rebound and compression/respiration rate (73.9% vs. 55.6%, P= 0.024; 88.6% vs. 70.4%, P= 0.006; 96.6% vs. 87%, P= 0.031). In the theoretical examination, the correct rates of electrocardiogram [(53.63±2.9)% vs. (50.44±2.57)%] and first aid medication [(57.38±3.55)%, P = 0.001] in the two groups were significantly lower than the qualified rate. After adjusting other factors, Logistic regression analysis showed that the CPR qualification rate in group A was 2.769 times higher than that in group B ( P= 0.015, 95% CI 1.215~6.311) Conclusions:The first aid skills training mode based on competition can objectively reflect first aid skills level of residents. We found that the quality of CPR skills was not related to gender, specialty and real CPR experiences but experience of BLS training was an independent influencing factor. And how to identify and manage arrhythmias and how to choose different emergency drugs are urgent emergency skills for residents to improve.

OBJECTIVE: To explore the clinical phenotype and genetic variants of a fetus with Glutaracidemia type II C (GA II C). METHODS: Clinical data of a 32-year-old pregnant woman and her fetus with GA II C diagnosed at the Third Affiliated Hospital of Zhengzhou University in December 2021 due to the enlargement and enhanced echo of the kidneys and oligohydramnios fluid at 17 weeks were analyzed retrospectively. Amniotic fluid sample of the fetus and peripheral blood samples of the couple were collected for whole exome sequencing (WES). Candidate variants were verified by Sanger sequencing. Copy number variation (CNV) was detected by using low-coverage whole genome sequencing (CNV-seq). RESULTS: At 18 weeks' gestation, ultrasound revealed that the fetus had enlargement and enhanced echo of the kidneys along with no echo of renal parenchymal tubular fissure and oligohydramnios. MRI at 22 weeks' gestation confirmed that both kidneys were enlarged with uniformly increased abnormal T2 signal and decreased DWI signal. The volume of both lungs was small, with slightly higher T2 signal. No CNV was detected in the fetus. WES revealed that the fetus has harbored compound heterozygous variants of the ETFDH gene, namely c.1285+1G＞A and c.343_344delTC, which were inherited from its father and mother, respectively. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), both variants were classified as pathogenic (PVS1+PM2_Supporting+PS3_Supporting; PVS1+PM2_Supporting+PM3). CONCLUSION The c.1285+1G＞A and c.343_344delTC compound heterozygous variants of the ETFDH gene probably underlay the disease in this fetus. Type II C glutaric acidemia may manifest as bilateral kidney enlargement with enhanced echo and oligohydramnios. Discovery of the c.343_344delTC has enriched the spectrum of ETFDH gene variants.
Pregnancy ; Humans ; Female ; Mutation ; DNA Copy Number Variations ; Oligohydramnios/genetics* ; Retrospective Studies ; Phenotype ; Fetus/diagnostic imaging*