Long-term auditory monitoring in children with Alport syndrome based on different degrees of renal injury / 临床耳鼻咽喉头颈外科杂志

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.

Necessity and methodological progress on the establishment of continuous reference intervals for pediatric age-dependent indicators / 中华检验医学杂志

Accurate and applicable reference intervals can provide important information for disease diagnosis, efficacy evaluation, and health monitoring. Age-dependent trends exist for many clinical laboratory indicators, and the interpretation of such indicators should consider the effect of age carefully. However, age-specific reference intervals have certain limitations in clinical application. Continuous reference intervals can not only help accurate interpretation of laboratory test results, but also provide a baseline value for dynamic monitoring of their changing trends. Based on the researches on pediatric reference intervals from our team, this paper introduces different algorithms for establishing continuous reference intervals, and their applicable conditions and implementation steps. The aim of this paper is to provide methodological guidance for the establishment of continuous reference intervals, so as to improve the quality of laboratory reports and the accuracy of clinical diagnosis in China.

Analysis of efficacy of ultrasound-guided percutaneous polidocanol sclerotherapy for thyroglossal duct cysts in children / 中华超声影像学杂志

Objective:To evaluate the effectiveness and safety of ultrasound-guided percutaneous polidocanol sclerotherapy of the thyroglossal duct cysts in children.Methods:A retrospective analysis of 26 children who were treated with ultrasound-guided percutaneous puncture sclerosis for thyroglossal duct cysts in Beijing Children′s Hospital, Capital Medical University from October 2019 to April 2022, the size of the cyst before treatment was recorded, and the cyst volume in accordance with V(ml) =1/6 πabc cyst volume was calculated. The follow-up time was the 1st, 3rd, 6th, and 12th months after sclerotherapy. And the cyst volume and volume reduction ratio at different time points was calculated. According to changes in the cystic volume, the treatment was considered effective if the cyst volume reduction rate was≥50%. Cosmetic grading scores (CGS) were performed pre-treatment and at the last follow-up after sclerotherapy using the WHO grading system. Complications were recorded during the follow-up period.Results:The cyst volume before treatment was 2.67 (3.78)ml, and the cyst volume at the 1st, 3rd, 6th and 12th month after treatment were 0.66(1.83), 0.45(0.87), 0.40(0.70), 0.38 (0.63)ml, respectively, there were significant differences between pre-treatment and each time point after-treatment (all P<0.001); the volume reduction rate at the last follow-up was (81.48±14.57)%. The reduction rate of cyst volume was more than 50% at the last follow-up in 22 children, the treatment efficiency was 84.62% (22/26). The cosmetic grading scores at the last follow-up after sclerotherapy was 1.74(1.50), and it decreased significantly compared with the pre-treatment score 3.85(0)( P<0.001). There was no skin pigmentation, pain in the injection area, local urticaria and blistering after sclerotherapy, no adverse reactions such as cyst bleeding and drunkenness-like reaction, and no serious complications occurred. There were 2 cases of cysts that presented infection with fever, and no serious complications occurred. Conclusions:Ultrasound-guided percutaneous sclerotherapy is a safe and effective minimally invasive treatment for thyroglossal duct cysts in children.

Genotype-phenotype correlations, surgical selections, and postoperative complications of 5α-reductase 2 deficiency in 69 children with hypospadias / 亚洲男科学杂志(英文版)

5α-reductase 2 deficiency prevents testosterone from being converted to dihydrotestosterone, which causes abnormal urogenital sinus development. The aim of this study was to analyze the relationship between genotype-phenotype, surgical selections, and postoperative complications of 5α-reductase 2-deficient patients with hypospadias. We retrospectively evaluated the medical records of patients who were diagnosed with 5α-reductase 2 deficiency after genetic testing in the Department of Endocrinology and underwent initial hypospadias surgery in the Department of Urology in Beijing Children's Hospital, Capital Medical University (Beijing, China), from April 2007 to December 2021. A total of 69 patients were included in this study; the mean age at surgery was 34.1 months, and the average follow-up time was 54.1 months. Sixty children were treated with preoperative hormone stimulation (PHS) to promote penile growth. The average penis length and glans width were increased by 1.46 cm and 0.62 cm, respectively. The most frequent mutations were p.R227Q (39.1%, 54/138), p.Q6* (15.2%, 21/138), p.G203S (12.3%, 17/138), and p.R246Q (11.6%, 16/138). In 64 patients who were followed up, 43 had a one-stage operation and 21 had a staged operation, and there were significant differences in external masculinization score (EMS) ( P = 0.008) and the average number of operation required to cure ( P < 0.001) between one-stage and staged operations. PHS had a positive effect ( P < 0.001) on penile development. The p.R227Q mutation was associated with higher EMS and less severe hypospadias. One-stage surgery can be selected if conditions permit. The growth and development of children are acceptable in the long term, but penis growth remains unsatisfactory. Long-term complications of hypospadias should be considered during puberty.

Clinical characteristics and serious complications of esophageal button battery ingestion in the pediatric on 83 cases / 中华耳鼻咽喉头颈外科杂志

Objective: To analyze the clinical characteristics and complications of esophageal foreign bodies of button battery ingestion in children. Methods: A retrospective descriptive study included 83 children who were hospitalized in our hospital on account of button battery ingestion from January 2011 to December 2021. There were 50 males (60.2%) and 33 females (39.8%). The age ranged from 7.6 months to one month off 10 years, with a median age of 18 months. The data of patient demographics and time from ingestion to admission, location, symptoms, management, complications, and follow-up outcome were recorded. SPSS17.0 software was used for statistical analysis. Results: Seventy-two children (86.7%) were younger than 3 years old. The time from ingestion to admission ranged from 1 h to 2 months, with a median time of 8 h. Among the 63 children who were first diagnosed in our hospital, the most common clinical symptoms were nausea and vomiting (32 cases, 50.8%), dysphagia (31 cases, 49.2%), salivation (11 cases, 17.5%) and fever (10 cases, 15.9%). Seventy-three of 83 cases had complete preoperative diagnostic tests, and 55 cases (75.3%) were diagnosed by X-ray. In 56 cases (76.7%), the foreign badies were impacted in the upper third of esophagus. In 72 cases (86.7%), the foreign badies were removed by rigid esophagoscopy. 23 (27.7%) had serious complications, including tracheoesophageal fistula in 15 cases(TEF;65.2%), vocal cord paralysis (VCP;34.8%) in 8 cases, esophageal perforation in 3 cases (EP;13.0%), hemorrhage in 3 cases(13.0%), mediastinitis in 3 cases (13%), and periesophageal abscess in 1 case (4.3%). There were significant differences in the exposure time of foreign bodies and unwitnessed ingestion by guardians in the complications group (P<0.05). 2 cases died (2.4%)respectively due to arterial esophageal fistula bleeding and respiratory failure caused by stent displacement during the treatment of tracheoesophageal fistula. Conclusion: Accidental button battery ingestion can be life-threatening. and it mostly happens in children under 3 years old. Serious complications may happen cause of non-specific clinical manifestations and unwitnessed ingestions. Anterior and lateral chest X-ray is the first examination choice. Tracheoesophageal fistula is the most common serious complication.

Trends of hospitalized cases of children′s hospitals in China before and after COVID-19 / 中华医院管理杂志

Objective:To analyze the impact of COVID-19 on the number of hospitalization and the disease spectrum of children′s hospitals in China, so as to provide scientific basis for improving the epidemic response policy of children′s hospitals and restoring hospital operations.Methods:Discharged patients from 27 children′s hospitals from January 1, 2019, to December 31, 2020, were selected from the FUTang Updating medical Records(FUTURE) Database. The ratio of discharge number in 2020 to that in 2019 was calculated. Age groups, disease types, and discharge months were further divided to calculate the ratio of discharge numbers under different conditions.Results:The total number of discharged patients in 2020 was 76.76% of the total number in 2019. In terms of discharge time, the ratio rose slowly from the lowest values in February and March, and reached about 90% of the same period of 2019 at the end of 2020. In terms of age, the biggest change in the discharge number was among children aged 1-3 years, which was 71.87% in 2019. In terms of disease classifications, respiratory diseases changed the most in the number of discharged patients in 2020, accounting for 56.03% of that in 2019. The top five hospitalized diseases of children did not change, while the other ranks changed slightly.Conclusions:COVID-19 has a huge and lasting impact on the number of hospitalizations in children′s hospitals, and the hospitals should develop multiple approaches such as online medical care to cope with the long-term negative impact of the pandemic.

Whole-Exome Sequencing Reveals Pediatric Rare Syndromic Hearing Loss / 罕见病研究

  Objective  To discuss the significance of genetic diagnosis of children with syndromic hearing loss by using whole-exome sequencing.  Methods  The clinical data of 34 children with sensorineural hearing loss were collected and the whole exons of genome of the children and their parents were sequenced and analyzed.  Results  Genetic causative gene and mutations have been identified in 19 children, including 4 genes (HARS2, USH2A, GATA3, MITF) related to rare syndromic hearing loss. Fifteen children were diagnosed with non-syndromic hearing loss related gene, including 8 cases with GJB2 mutation, 5 cases with SLC26A4 mutation and 2 cases with MYO15A mutation. Mutations of c.435_437del(p.K147del) and c.1403G > C (p.G468A) in gene HARS2, c.11389+1del in gene USH2A, c.1327delA(p.M443Wfs*33) in gene GATA3, c.627C > A(p.C209X) in gene MITF and c.8033_8057delinsG(p.N2678_D2686delinsS) in gene MYO15A were first reported.  Conclusions  Whole-exome sequencing helps the accurate diagnosis of causes of hearing loss, especially for the rare syndromic hearing loss with atypical clinical manifestations. Information from genetic testing may highlight further recommended exams of structure and functions of related organs.

The Diagnosis and Treatment of Rare Diseases in Children: Now and Future / 罕见病研究

Rare diseases refer to a group of diseases having very low incident rates in the population without unified definition up till now. Approximately 50% to 75% of rare diseases occur at birth or in childhood, incurring huge psychological and economic burden to families and society. With the rapid development of diagnostic technology and the continuous progress in treatment and the introduction of relevant policies, China has made great progress in the prevention and treatment of rare diseases. This article summarizes the latest progress in the diagnosis, clinical management, and research of pediatric rare diseases; and explores the prospects in the future.

Research advances of natural killer cells at the maternal-fetal interface / 生理学报

Natural killer (NK) cells are the main immune cells at the maternal-fetal interface and accumulate in the uterine decidua in early pregnancy. Many studies have shown that NK cells at the maternal-fetal interface have unique phenotypes and play critical roles in various processes, including immune tolerance during pregnancy, decidualization, invasion of trophoblasts, remodeling of the uterine spiral artery, formation of the placenta and growth of embryo. However, specific functions of NK cells and their mechanism remain to be fully elucidated. This review summarizes the research progress of NK cells at the maternal-fetal interface and their roles in the pregnancy-related disorders in recent years. The aims of this review are to gain deep insight of the function of NK cells at the maternal-fetal interface and provide new ideas for intervention of pregnancy-related diseases.

A multi-stage dynamic prevention and control study on hospital workplace violence based on crisis management theory / 中华医院管理杂志

Objective:To construct a multi-stage dynamic prevention and control model, establish a system of intervention points and prevention and control measures for the prevention and control of workplace violence in hospitals, so as to provide guidance for hospitals and medical staffs to effectively prevent and respond to such incidents.Methods:Based on the crisis management theory, a model for the prevention and control of workplace violence in hospitals was constructed, the intervention points and prevention and control measures were screened by the Delphi method.Results:A multi-stage dynamic prevention and control model of workplace violence in hospitals was constructed, and a system of intervention points and prevention and control measures for workplace violence in hospitals were established according to the model. The system was divided into three stages: the pre-event stage contained 10 intervention points and 48 countermeasures, the in-event stage contained 6 intervention points and 17 countermeasures, and the post-event stage contained 3 intervention points and 12 countermeasures.Conclusions:It is an effective way to avoid violence and reduce the damage degree of violent incidents by selecting different countermeasures for different intervention points and carrying out multi-stage dynamic prevention and control of workplace violence in hospitals.

Optimization for technical resources of children′s gastroscopy based on diagnosis-related groups / 中华医院管理杂志

Objective:To analyze the gastroscopy treatment technology in a Children′s Medical Center based on the diagnosis-related groups(DRG) and put forward suggestions for resource optimization.Methods:The data of the front pages of medical records of 22 medical institutions in a Children′s Medical Center in 2018 were divided into DRG groups. The patients in gastroscope treatment operation group(GK3)were selected, and the disease diagnosis, operation and payment methods of the patients in this group were analyzed.Results:Of the 22 medical institutions, 16 had GK3 group cases, and the number of cases was significantly different, ranging from 2 to 917. Among them, the institution with code M was characterized by multiple endoscopic treatment of esophageal stricture, but most other institutions rarely carried out the treatment of esophageal stricture. In GK3 group, the main payment method of children in Institution M with the highest constituent ratio was at one′s own expense, followed by non-local medical insurance. The main payment type of O and P institutions with the second and third constituent ratio was local medical insurance.Conclusions:The gap of the technology of gastroscopy in the treatment of esophageal stricture is large in all institutions. The high-quality medical resources can be sunk through the construction of pediatric medical alliance, and the gap between the regional medical technology can be continuously leveled.

Practice of collaborative development model of pediatrics′s medical practice, education and research at Beijing municipal hospitals / 中华医院管理杂志

Collaborative development among medical practice, education and research is a strategic decision of the country in disciplinary development guided by the innovation-driven strategy. In October 2017, Beijing Hospitals Authority organized 18 tertiary hospitals with pediatrics discipline and founded a collaborative development center for pediatrics. This center operated in a model featuring both leadership of due authorities and autonomous administration. Two of the specialized pediatrics hospitals work as leading units, and existing high quality pediatrics resources of the member hospitals were pooled to establish an academics committee and an executive committee. A development system was established with disciplinary construction as the focus, collaborative development as the goal and horizontal collaboration as the means. It was designed to explore a new model featuring overall planning and standardized management of the discipline, building of a shared platform for clinical capacity development, joint development of continued medical education and talent cultivation, as well as diversified and multi-centered research and platform resources sharing. This model can effectively promote the overall development level of pediatrics in Beijing municipal hospitals.

Exploration on the management model of international scientific and technological cooperation based on the combination of " project-talent-base" in hospital / 中华医学科研管理杂志

Objective:Explore the " project-talent-base" combining international science and technology cooperation management model and mechanism through summarizing the practical experience of a tertiary pediatric hospital in Beijing in recent years, to provide reference for strengthening the hospital′s international science and technology cooperation.Methods:Main problems existing in the management of international science and technology cooperation in domestic medical institutions are analyzed, and management methods of international science and technology cooperation adopted by the hospital are classified through policy sorting, literature research, summarization and classification.Results:The hospital has established an efficient and high-quality international science and technology cooperation management model mainly through four aspects: improving the international cooperation management system, enriching international cooperation and exchange projects, building different types of international science and technology cooperation platforms, and strengthening personnel training.Conclusions:Under the background of the globalization of science and technology and the accelerating pace of scientific research in China, improving the hospital′s international science and technology cooperation management model will effectively promote the introduction of international advanced technologies and concepts, and enhance the overall science and technology innovation capabilities and discipline construction.

Research on the mode of hospital-institute integration based on project cooperation / 中华医学科研管理杂志

Objective:To explore the hospital-institute integration mode based on project cooperation.Methods:Focusing on the management innovation of clinical and basic scientific research cooperation mode, the method of hospital-institute integration mode based on project cooperation and the key points in practice were discussed, to develop better understandings of the construction and development such mode.Results:Several key points of developing hospital-institute integration mode based on project cooperation were proposed. Main measurements adopted including the integration of scientific research and clinic service, comprehensive construction of management concept, scientific research capacity, teaching organization and service concept, cultivation of high-level research talents, construction of research departments, according to which formed the main structure of the hospital. Furthermore, it is important to change the development mode in time to promote the healthy development of the hospital.Conclusions:It is a new direction of the clinical and basic research cooperation innovation to encourage the hospital-institute integration mode based on project cooperation. It is also an effective strategy to promote the improvement of diagnosis and treatment level under the new medical reform situation.

Huayu Jiedu Prescription Alleviates Gut Microbiota and Fecal Metabolites in Mice with Endometriosis / 中国实验方剂学杂志

Objective:To investigate the effect of Huayu Jiedu prescription （HYJDP） on gut microbiota and fecal metabolites in mice with endometriosis. Method:Normal female C57BL/6J mice were divided into normal control group （CO）， endometriosis group （EM） and Chinese medicine Huayu Jiedu decocotion group （CM）. CO and EM groups received normal saline and CM group received HYJDP by intragastric administration. Untargeted metabolomics method was used to detect metabolites in fecal supernatant of mice， and receiver operating characteristic （ROC） analysis was used to screen the differential metabolites， 16S rRNA high-throughput sequencing was used to detect the gut microbiota， and Spearman correlation coefficient was used to represent the degree of correlation between differential metabolites and intestinal flora. Lipopolysaccharides （LPSs） in intestinal wall tissue， serum and peritoneal lavage fluid were detected by enzyme-linked immunosorbent assay （ELISA）. The expression of Vimentin and E-cadherin in ectopic lesions was detected by immunohistochemistry. Result:HYJDP alleviated the disorders of fecal metabolites and gut microbiota in EMS mice， especially with the recovered levels of homoveratric acid， melilotoside C and physapubescin in fecal supernatant. In the comparison of these three factors between EM group and CO group as well as between EM group and CM group， the variable important in projection （VIP） value was both above 2， and AUC in ROC analysis was both >0.9. As compared with EM group， HYJDP restored the abundance of species such as <italic>Lachnospiraceae_NK4A136_group</italic>， <italic>Lactobacillus</italic> and <italic>Blautia </italic>（<italic>P</italic><0.05）. In addition， the level of LPS in peritoneal fluid supernatant of EM group was significantly higher than that of CO group （<italic>P</italic><0.05） and CM group （<italic>P</italic><0.05）. The protein expression of vimentin and E-cadherin in endometriosis decreased significantly （<italic>P</italic><0.05）. Conclusion:HYJDP which can improve the intestinal environment and reduce the level of LPS in mice with endometriosis， is an effective drug for the treatment of endometriosis.

Clinical diagnosis and treatments of type Ⅱ-Ⅳ congenital laryngotracheoesophageal cleft in 8 children / 中华耳鼻咽喉头颈外科杂志

Objective: To investigate the clinical diagnosis and treatment of congenital laryngotracheoesophageal cleft (LTEC) in children. Methods: The clinical data of 8 children (including 7 males and 1 female)with congenital laryngotracheoesophageal cleft from January 2016 to June 2020 were retrospectively analyzed. The median diagnosing age was 3.75 months (5 days to 12 months). According to the modified Benjamin Inglis classification proposed by Sandu in 2006,there were 3 cases of type Ⅱ, 3 cases of type Ⅲa, 1 case of type Ⅲb and 1 case of type Ⅳa. All children were followed up regularly. Results: Six patients were treated for recurrent bronchopneumonia and aspiration during feeding. The patients were first treated in the pneumology departmentt or intensive care unit. Six patients combined with other malformations. Endoscopic repair operations were performed in 6 cases (3 cases of type Ⅱ, 3 cases of type Ⅲ a), 1 case of LTEC was operated through cervical approach, and 1 case of type IVa LTEC associated with VACTERL was repaired under thoracoscope combined with suspension laryngoscope. Seven patients underwent tracheotomy before or during the repair operations. Gastrostomy was performed in 2 children. The operations were successfully performed in all cases. Three children with type Ⅱ LTEC recovered well and decannulated. One case of type Ⅲa was followed up for 5 months with occasionally choking while feeding. Two cases of type Ⅲa, 1 case of type Ⅲb and 1 case of type Ⅳa died due to severe reflux, tracheomalacia or respiratory failure. Conclusions: Congenital LTEC is a rare congenital malformation which is difficult to diagnose for the poor specificity of clinical manifestations. LTEC needs to be classified by endoscopy examination under general anesthesia. Severe cases of LTEC have poorer outcomes than the mild cases, and the perioperative managements need multi-disciplinary cooperation to reduce the mortality.

Study on the feature of cortical auditory evoked potential under different auditory tasks in cochlear implant children / 中华耳鼻咽喉头颈外科杂志

Objective: To investigate the variation regularity and influencing factors of cortical auditory evoked potential (CAEP) evoked by pure tone, syllable and tone stimuli in cochlear implant (CI) children. Methods: Cortical auditory evoked potential (CAEP) responses were collected from 46 CI children in the sound field. Pure tones with frequencies of 1 kHz and 2 kHz were used as the standard and the deviant respectively in the pure tone stimulation condition. The Chinese Mandarin tokens/ba/-/pa/and/ba1/-/ba4/pairs were used as the stimuli respectively in the syllable and tone stimulation condition. The latency, amplitude and presence rate of P1 and mismatch negative(MMN) were obtained and the correlation between the difficulty of auditory task, the age of hearing month, the duration of severe-profound hearing loss, the wearing history of hearing aid before CI, the hearing threshold of the better ear before CI and the latency and amplitude of P1 and MMN were analyzed. All statistical analyses and figures were conducted using SPSS 25.0. Results: The P1 presence rate of pure tone, syllable and tone group was 100% (17/17), 100% (13/13) and 75.0% (12/16), respectively, with significant difference (χ²=8.214, P=0.016). There was significant difference between pure tone group and tone group (χ²=4.836, P=0.028), but no significant difference between pure tone group and syllable group, syllable group and tone group. The MMN presence rate of pure tone, syllable and tone group was 94.1% (16/17), 84.6% (11/13) and 62.5% (10/16), respectively, but no significant difference among the three groups with different auditory tasks(χ²=0.066, P=0.066). No significant difference was observed among the three groups of different auditory tasks in the latency and amplitude of P1 and MMN. Multiple linear regression analysis showed that the latency of P1 was positively correlated with the difficulty of auditory task and the hearing threshold of the better ear before CI, and negatively correlated with hearing age and the history of hearing aid before CI. The latency of MMN was positively correlated with the difficulty of auditory task, and negatively correlated with hearing age. Conclusions: The P1 presence rate of pure tone auditory task is significantly higher than that of tone auditory task. The difficulty of auditory task, hearing age, the history of hearing aid before CI, and the hearing threshold of the better ear before CI has significant effects on the P1 latency. The difficulty of auditory task and hearing age has significant effects on the MMN latency.

Construction of transgenic mice with specific Cre recombinase expression in the zona fasciculata in adrenal cortex / 生理学报

The adrenal gland is an important endocrine organ of human body. CYP11B1 gene was specifically expressed in the zona fasciculata in adrenal cortex. In order to better study the function of genes specifically expressed in the zona fasciculata in adrenal cortex, the mice with Cre recombinase specifically expressed in the zona fasciculata in adrenal cortex were constructed. It was then confirmed that CYP11B1 was specifically expressed in adrenal glands. Then, using CRISPR/Cas9 technique, CYP11B1-2A-GfpCre recombinant vector was constructed and subsequently injected into the fertilized eggs of mice. It was confirmed that the Cre gene was mainly expressed in the zona fasciculata in adrenal cortex of CYP11B1Cre mice by using mTmG and LacZ staining. The CYP11B1Cre mice were then mated with cystathionine γ-lyase (CTH) mice, thereby generating CTH/CYP11B1Cre mice. It was also confirmed that CTH gene in the zona fasciculata in adrenal cortex was specifically knocked out in these mice. These results suggest that transgenic mice with specific Cre recombinase expression in the zona fasciculata in adrenal cortex were constructed successfully. This animal model can be a powerful tool for the study of the function of genes expressed in the zona fasciculata in adrenal cortex.

Endothelial dysfunction in children with obstructive sleep apnea syndrome / 中华儿科杂志

Objective@#To evaluate the endothelial function in obstructive sleep apnea syndrome(OSAS) children and to identify related factors of endothelial dysfunction.@*Methods@#This was a cross-sectional study. Children with habitual snoring (snoring ≥3 nights per week) admitted to the ward of otolaryngology, head and neck surgery, Beijing Children′s Hospital were recruited to this study between 1st June 2015 and 1st March 2016. All children aged 3 to 11 years and of them 245 were boys and 110 were girls. All subjects underwent an overnight polysomnography (PSG), as well as endothelial function testing. All subjects were grouped into primary snoring (PS) and OSAS group according to the obstructive apnea hypopnea index (OAHI). T test or Wilcoxon test were used to compare the differences in PSG results between the two groups, and univariate and multivariate correlation analyses were used to explore the relevant factors affecting the endothelial function.@*Results@#A total of 355 subjects were enrolled and 248 had OSAS, and 107 had PS. There were no significant differences in age, gender and body mass index (BMI) Z-score between the two groups (all P>0.05). OSAS group had higher OAHI, oxgen desaturation index and respiratory related arousal index (5.2 (2.2, 13.2) vs. 0.4 (0.1, 0.7), 4.1 (2.0, 13.1) vs. 0.5 (0.1, 1.0), 2.5 (1.0, 4.8) vs. 0.4 (0.1, 0.9), Z=-14.957, -11.790, -10.378, all P<0.01), and lower minimum oxygen saturation and reactive hyperemia index (RHI) than those of PS (0.89 (0.85, 0.92) vs. 0.94 (0.91, 0.95), 1.2±0.2 vs. 1.1±0.1, Z=-9.337, t=5.354, P<0.01). Univariate regression analysis showed that RHI was linearly correlated with age (parameter estimate=0.017, P<0.01), gender (parameter estimate=0.065, P<0.01), OAHI (parameter estimate=-0.023, P<0.01), oxygen desaturation index (parameter estimate=-0.019, P<0.01), respiratory related arousal index (parameter estimate=-0.031, P<0.01), and oxygen saturation nadir (parameter estimate=0.067, P=0.045). The relationship between BMI Z-score and RHI was quadratic. Multivariate regression analysis showed that age (parameter estimate=0.015, P<0.01), BMI Z-score (parameter estimate=0.040, P<0.01), BMI Z-score quadratic form (parameter estimate=-0.010, P<0.01), respiratory related arousal index (parameter estimate=-0.020, P<0.01) were independently correlated with RHI.@*Conclusions@#Children with OSAS have significant endothelial dysfunction compared with PS. Frequent arousals due to obstructive respiratory events during sleep may be a candidate risk factor for endothelial dysfunction in children with OSAS.

Prevalence Of Respiratory Symptoms And Pulmonary Function Status Of Restaurant Workers

@#The cooking process may emit toxic compounds and airway irritants from both the fuel combustion and cooking fumes which is harmful to the respiratory health among the restaurant workers. A cross-sectional study of 243 restaurant workers from the selected restaurants in Sungai Long, Malaysia was conducted. The standardized British Medical Research Council questionnaire on Respiratory Symptoms (1986) was used during the interview to access the symptoms and the spirometry test was performed to evaluate the pulmonary functions of the participants. The data of socio-demography and occupational characteristics were also collected. The most complaint respiratory symptoms by the restaurant workers were breathlessness, which accounted for 33.7%, followed by wheezing (14%). The mean values of all pulmonary function tests (PFT) of the restaurant workers were within the normal range (>80%), except for the Peak Expiratory Flow (PEF) (79.09%). The results of the bivariate statistical analysis, Chi-square, ANOVA and t-test, showed the determining factors of the respiratory health among the workers were workers’ age and gender in addition to the working duration and the ethnicity. The restaurant workers in Sungai Long were at risk of developing respiratory symptoms and lower pulmonary function values due to prolonged exposure to cooking fumes. Emphasis should be given to the safety and health of restaurant workers and health education should be provided to the restaurant workers and owners. Strategies to increase notification of such occurrences among these workers should be looked into by related agencies in the country.