Budd-Chiari syndrome with hepatopulmonary syndrome: a case report and literature review / 中华儿科杂志

Objective: To summarize the clinical features and prognosis of Budd-Chiari syndrome with hepatopulmonary syndrome (HPS) in children. Methods: The clinical data of a child who had Budd-Chiari syndrome with HPS treated at the Department of Pediatrics of the First Affiliated Hospital of Zhengzhou University in December 2016 was analyzed retrospectively. Taking "Budd-Chiari syndrome" and "hepatopulmonary syndrome" in Chinese or English as the keywords, literature was searched at CNKI, Wanfang, China Biomedical Literature Database and PubMed up to July 2023. Combined with this case, the clinical characteristics, diagnosis, treatment and prognosis of Budd-Chiari syndrome with HPS in children under the age of 18 were summarized. Results: A 13-year-old boy, presented with cyanosis and chest tightness after activities for 6 months, and yellow staining of the skin for 1 week. Physical examination at admission not only found mild yellow staining of the skin and sclera, but also found cyanosis of the lips, periocular skin, and extremities. Laboratory examination showed abnormal liver function with total bilirubin 53 μmol/L, direct bilirubin 14 μmol/L, and indirect bilirubin 39 μmol/L, and abnormal blood gas analysis with the partial pressure of oxygen of 54 mmHg (1 mmHg=0.133 kPa), the partial pressure of carbon dioxide of 31 mmHg, and the alveolar-arterial oxygen gradient of 57 mmHg. Hepatic vein-type Budd-Chiari syndrome, cirrhosis, and portal hypertension were indicated by abdominal CT venography. Contrast-enhanced transthoracic echocardiography (CE-TTE) was positive. After symptomatic and supportive treatment, this patient was discharged and received oxygen therapy outside the hospital. At follow-up until March 2023, there was no significant improvement in hypoxemia, accompanied by limited daily activities. Based on the literature, there were 3 reports in English while none in Chinese, 3 cases were reported. Among a total of 4 children, the chief complaints were dyspnea, cyanosis, or hypoxemia in 3 cases, and unknown in 1 case. There were 2 cases diagnosed with Budd-Chiari syndrome with HPS at the same time due to respiratory symptoms, and 2 cases developed HPS 1.5 years and 8.0 years after the diagnosis of Budd-Chiari syndrome respectively. CE-TTE was positive in 2 cases and pulmonary perfusion imaging was positive in 2 cases. Liver transplantation was performed in 2 cases and their respiratory function recovered well; 1 case received oxygen therapy, with no improvement in hypoxemia; 1 case was waiting for liver transplantation. Conclusions: The onset of Budd-Chiari syndrome with HPS is insidious. The most common clinical manifestations are dyspnea and cyanosis. It can reduce misdiagnosis to confirm intrapulmonary vascular dilatations with CE-TTE at an early stage. Liver transplantation is helpful in improving the prognosis.

The Uptake and Distribution Evidence of Nano- and Microplastics in vivo after a Single High Dose of Oral Exposure / 生物医学与环境科学（英文）

OBJECTIVE@#Tissue uptake and distribution of nano-/microplastics was studied at a single high dose by gavage in vivo.@*METHODS@#Fluorescent microspheres (100 nm, 3 μm, and 10 μm) were given once at a dose of 200 mg/(kg∙body weight). The fluorescence intensity (FI) in observed organs was measured using the IVIS Spectrum at 0.5, 1, 2, and 4 h after administration. Histopathology was performed to corroborate these findings.@*RESULTS@#In the 100 nm group, the FI of the stomach and small intestine were highest at 0.5 h, and the FI of the large intestine, excrement, lung, kidney, liver, and skeletal muscles were highest at 4 h compared with the control group ( P < 0.05). In the 3 μm group, the FI only increased in the lung at 2 h ( P < 0.05). In the 10 μm group, the FI increased in the large intestine and excrement at 2 h, and in the kidney at 4 h ( P < 0.05). The presence of nano-/microplastics in tissues was further verified by histopathology. The peak time of nanoplastic absorption in blood was confirmed.@*CONCLUSION@#Nanoplastics translocated rapidly to observed organs/tissues through blood circulation; however, only small amounts of MPs could penetrate the organs.

Effects of R-loops on Genome Stability / 中国生物化学与分子生物学报

The R-loop is a three-stranded nucleic acid structure, which consists of a RNA: DNA hybrid and a DNA single strand. R-loop can be divided into two types: physiological and pathological. The physiological R-loop is involved in many physiological processes such as DNA replication, transcription, and gene expression regulation, while the pathological R-loop induces DNA damage and genome rearrangement. There are many factors that affect the formation of R-loops. Unregulated R-loops destroy genomic stability by interfering with DNA replication and double-strand DNA break repair, and can cause cancer. Therefore, the regulation of R-loops is very important. RNA/ DNA helicase Senataxin (SETX), DEAD-box helicase 5 (DDX5), ribonuclease H (RNase H) and DNA topoisomerase I (topo) play an important role in regulating the balance of R-loops in vivo. Among them, SETX is one of the most characteristic R-loop decomposing enzymes, which can dissolve the R-loops produced during transcriptional termination sites, replication-transcriptional conflicts and DNA damage repair. Senataxin mutations will lead to ataxia with eye movement apraxia type 2 (AOA2) and amyotrophic lateral sclerosis type 4 (ALS4). Currently there are still many unsolved issues, although many in-depth studies of R-loops have been carried out. Therefore, the structure and function of physiological and pathological R-loops still need to be further explored. This review mainly focuses on the definition and classification of R-loops, the factors that affect the formation of R-loops, the influence of R-loops on genomic stability and R-loop-related diseases, and explores the possibility of using R-loops as a therapeutic target in the future.

Follow-up and prognostic study of infants with positional plagiocephaly / 中国当代儿科杂志

OBJECTIVES@#To study the effects of infantile positional plagiocephaly on the growth and neural development.@*METHODS@#A retrospective study was conducted on the medical data of 467 children who underwent craniographic examination and were followed up to 3 years of age in Peking University Third Hospital from June 2018 to May 2022. They were divided into four groups: mild positional plagiocephaly (n=108), moderate positional plagiocephaly (n=49), severe positional plagiocephaly (n=12), and normal cranial shape (n=298). The general information of the four groups and the weight, length, head circumference, visual acuity screening results, hearing test results, and the scores of Pediatric Neuropsychological Developmental Scales/Gesell Developmental Schedules of the four groups from 6 to 36 months old were compared.@*RESULTS@#The rates of adverse perinatal factors, congenital muscular torticollis, and supine fixed sleeping posture in the mild, moderate, and severe positional plagiocephaly groups were higher than the normal cranial group (P<0.05). There was no significant difference in weight, length, and head circumference among the four groups at 6, 12, 24 and 36 months of age (P>0.05). The incidence rate of abnormal vision in the severe positional plagiocephaly group was higher than that in the mild positional plagiocephaly, moderate positional plagiocephaly and normal cranial shape groups at 24 and 36 months of age (P<0.05). The scores of the Pediatric Neuropsychological Developmental Scales at 12 and 24 months of age and the scores of the Gesell Developmental Schedules at 36 months of age in the severe positional plagiocephaly group were lower than those in the mild positional plagiocephaly, moderate positional plagiocephaly and normal cranial shape groups, but the difference was not statistically significant (P>0.05).@*CONCLUSIONS@#Adverse perinatal factors, congenital muscular torticollis, and supine fixed sleeping position may be associated with infantile positional plagiocephaly. Mild or moderate positional plagiocephaly has no significant impact on the growth and neural development of children. Severe positional plagiocephaly have adverse effects on the visual acuity. However, it is not considered that severe positional plagiocephaly can affect the neurological development.

Systematic review and Meta-analysis of Gusongbao preparation in treatment of primary osteoporosis / 中国中药杂志

This study aims to provide evidence for clinical practice by systematically reviewing the efficacy and safety of Gusongbao preparation in the treatment of primary osteoporosis(POP). The relevant papers were retrieved from four Chinese academic journal databases and four English academic journal databases(from inception to May 31, 2022). The randomized controlled trial(RCT) of Gusongbao preparation in the treatment of POP was included after screening according to the inclusion and exclusion criteria. The quality of articles was evaluated using risk assessment tools, and the extracted data were subjected to Meta-analysis in RevMan 5.3. A total of 657 articles were retrieved, in which 15 articles were included in this study, which involved 16 RCTs. A total of 3 292 patients(1 071 in the observation group and 2 221 in the control group) were included in this study. In the treatment of POP, Gusongbao preparation+conventional treatment was superior to conventional treatment alone in terms of increasing lumbar spine(L2-L4) bone mineral density(MD=0.03, 95%CI[0.02, 0.04], P<0.000 01) and femoral neck bone mineral density, reducing low back pain(MD=-1.69, 95%CI[-2.46,-0.92], P<0.000 1) and improving clinical efficacy(RR=1.36, 95%CI[1.21, 1.53], P<0.000 01). Gusongbao preparation was comparable to similar Chinese patent medicines in terms of improving clinical efficacy(RR=0.95, 95%CI[0.86, 1.04], P=0.23). Gusongbao preparation was inferior to similar Chinese patent medicines in reducing traditional Chinese medicine syndrome scores(MD=1.08, 95%CI[0.44, 1.71], P=0.000 9) and improving Chinese medicine syndrome efficacy(RR=0.89, 95%CI[0.83, 0.95], P=0.000 4). The incidence of adverse reactions of Gusongbao preparation alone or combined with conventio-nal treatment was comparable to that of similar Chinese patent medicines(RR=0.98, 95%CI[0.57, 1.69], P=0.94) or conventio-nal treatment(RR=0.73, 95%CI[0.38, 1.42], P=0.35), and the adverse reactions were mainly gastrointestinal discomforts. According to the available data, Gusongbao preparation combined with conventional treatment is more effective than conventional treatment alone in increasing lumbar spine(L2-L4) bone mineral density and femoral neck bone mineral density, reducing low back pain, and improving clinical efficacy. The adverse reactions of Gusongbao preparation were mainly gastrointestinal discomforts, which were mild.

The relationship between classroom environment and myopia / 中华流行病学杂志

Objective: Based on a cohort and intervention study of the Eastern Chinese Student Surveillance, Cohort and Intervention Study (ES-SCI), this research aims to explore the correlation between monitor of the school environment and longitudinal data on myopia and provide evidence for the government myopia intervention strategy. Methods: This survey adopts the stratified cluster sampling method with the school as the unit. Students from grade 1 to grade 3 were selected according to the whole class to monitor the school environment in the classroom. Students will use the full-automatic computer optometer (TOPCON RM800) to conduct optometry from 2019 to 2021 under the condition of mydriasis to perform refractive eye examinations. Meantime eye axis length monitoring was also conducted. Cox proportional risk regression model was used to explore the relationship between school environmental monitoring and the occurrence and development of students' myopia. Results: From 2019 to 2021, 2 670 students from 77 classrooms participated in the observation study. The students' diopter after right/left eye mydriasis decreased in varying degrees (P<0.001), and the axial length of the right/left eye increased in various degrees (P<0.001). The weighted qualified rate of per capita area of primary school classrooms increased from 18.0% in 2019 to 26.0% in 2021, the weighted average illuminance pass rate of blackboard surface increased from 23.8% in 2019 to 26.4% in 2021, and the weighted average illuminance pass rate of classroom table decreased from 86.7% in 2019 to 77.5% in 2021. The trend chi-square test was significant (P<0.05). Cox proportional risk regression showed that after correcting for the grade, gender, parental myopia, diet, sleep, near work (sitting posture, working time, electronic mobile equipment, eye exercises), and outdoor activities, the per capita area of 1.36- m2 was the protective factor of eye axis length (HR=0.778, 95%CI: 0.659-0.918, P=0.003); The average reflection ratio of blackboard 0.15-0.19 was the protective factor of eye axis length (HR=0.685, 95%CI: 0.592-0.793, P<0.001); The average illumination of the blackboard 150-, 300-, 500- lx was the protective factor of the eye axis length (HR=0.456, 95%CI: 0.534-0.761, P<0.001; HR=0.794, 95%CI: 0.705-0.895, P<0.001; HR=0.690, 95%CI: 0.619-0.768, P<0.001). The blackboard evenness 0.40-0.59 was the risk factor of eye axis length (HR=1.528, 95%CI: 1.018-2.293, P=0.041), and the blackboard evenness 0.80- was the protection factor of eye axis length (HR=0.542, 95%CI: 0.404-0.726, P<0.001). The evenness of the desktop 0.40-0.59 was the protective factor of eye axis length (HR=0.820, 95%CI: 0.698-0.965, P=0.017). The average illuminance of 150-, 300-, 500- lx was the protective factor of a diopter (HR=0.638, 95%CI: 0.534-0.761, P<0.001; HR=0.911, 95%CI: 0.848-0.978, P=0.011; HR=0.750, 95%CI: 0.702-0.801, P<0.001). The average illumination of desktop 500- lx was a protective factor of a diopter (HR=0.855, 95%CI: 0.763-0.958, P=0.007). Conclusion: School environmental monitoring indicators, such as meeting per capita area standards, passing blackboard, and desk top-related indicators, all play protective effects on myopia development in students.

Value of endoscopic ultrasonography in the diagnosis of muddy stones of the common bile duct / 临床肝胆病杂志

Objective To investigate whether endoscopic ultrasonography (EUS) can be an alternative method for diagnostic endoscopic retrograde cholangiopancreatography (ERCP) by comparing the ability of EUS versus CT and transabdominal ultrasonography (TUS) in the diagnosis of muddy stones of the common bile duct. Methods A prospective study was conducted for 53 patients suspected of muddy stones of the common bile duct who attended Nanxishan Hospital of Guangxi Zhuang Autonomous Region from July 2019 to December 2021, and all patients underwent EUS, TUS, and CT before ERCP. With ERCP and endoscopic sphincterotomy (EST) for removing muddy stones of the common bile duct as the gold standard for the diagnosis of muddy stones of the common bile duct, EUS, TUS, and CT were compared in terms of their ability to display the muddy stones of the common bile duct. The chi-square test or the Fisher's exact test was used for comparison of categorical data between groups. Results In the 53 patients, EUS, TUS, and CT had a positive rate of 88.68%, 50.94%, and 62.26%, respectively, in detecting muddy stones of the common bile duct. As for the positive results confirmed by EST under ERCP, EUS had a sensitivity of 93.75%, a specificity of 60.00%, and an accuracy of 90.57% in detecting muddy stones of the common bile duct, while TUS had a sensitivity of 56.25%, a specificity of 100.00%, and an accuracy of 60.38% and CT had a sensitivity of 66.67%, a specificity of 80.00%, and an accuracy of 67.92%. There was a significant difference between EUS and CT in the accuracy in detecting muddy stones of the common bile duct ( χ 2 =8.26, P =0.004), and there was also a significant difference in diagnostic accuracy between EUS and TUS ( χ 2 =13.05, P < 0.001). Conclusion EUS is more accurate than TUS and CT in the diagnosis of muddy stones of the common bile duct, and instead of ERCP, EUS is thus recommended for suspected muddy stones of the common bile duct when TUS and CT fail to identify the lesions in clinical practice, so as to make a confirmed diagnosis and reduce related costs and complications.

Analysis of disease burden and risk factors of diabetic kidney disease in China from 1990 to 2019 / 中华肾脏病杂志

Objective:To explore the changes of disease burden and risk factors of chronic kidney disease (CKD) due to type 1 and type 2 diabetes mellitus in China from 1990 to 2019, and to provide reference data for the prevention and control of diabetic kidney disease (DKD).Methods:The Chinese DKD data were obtained from the 2019 Global Burden of Disease (GBD) database. The morbidity, prevalence, mortality, years lived with disability (YLD), years of life lost (YLL), and disability-adjusted life year (DALY) were used to compare the disease burden of CKD due to type 1 and type 2 diabetes mellitus from 1990 to 2019. In addition, the risk factors of DKD were analyzed.Results:The numbers of CKD patients due to type 1 and type 2 diabetes mellitus in China were 574 (95% UI 495-665) and 31 076 (95% UI 28 152-33 909) thousand, and the numbers of new cases were 9 (95% UI 8-11) and 434 (95% UI 390-481) thousand in 2019, respectively. The numbers of death were 13 (95% UI 8-18) and 63 (95% UI 50-77) thousand, respectively. The age groups with the largest number of patients and new cases of CKD due to type 1 diabetes mellitus were 30-34 years old and <5 years old, respectively. The age group with the largest number of patients and new cases of CKD due to type 2 diabetes mellitus were 50-54 years old and 70-74 years old, respectively. From 1990 to 2019, the age-standardized prevalence rate of DKD patients in China was relatively stable, but the age-standardized incidence rate and YLD rate showed an upward trend, while the age-standardized mortality rate, YLL rate, and DALY rate showed a downward trend. The main risk factors associated with DKD death were high fasting plasma glucose, kidney dysfunction, high systolic blood pressure, high body mass index, high sodium diet, and lead exposure. The proportions of DKD death caused by high systolic blood pressure and high body mass index in the Chinese population were still increasing. Conclusions:From 1990 to 2019, the age-standardized incidence and YLD rate of DKD in China shows an upward trend, while the age-standardized prevalence rate is relatively stable, and the age-standardized mortality rate, YLL rate, and DALY rate show a decreasing trend. High fasting glucose, renal failure, high systolic blood pressure, high body mass index, high sodium diet, and lead exposure are risk factors associated with death in DKD patients. With the progress of aging, the disease burden of DKD in China will continuously increase. Future work should be focused on population-specific interventions, taking into consideration the risk factors identified within the study.

Protective effect and mechanism of arbutin on cisplatin-induced renal tubular epithelial cell injury / 中华肾脏病杂志

The aim of this study was to elucidate the protective effect of arbutin on cisplatin (Cis) induced renal tubular epithelial cell injury and its mechanism. Cell counting kit-8 was used to detect the viability of renal tubular epithelial cells. The toxicity of arbutin on renal tubular epithelial cells at different concentrations and the appropriate concentration of arbutin to protect cells against cisplatin were observed. The renal tubular epithelial cells were divided into control group, arbutin group, Cis group and arbutin+Cis group. Flow cytometry, quantitative real-time PCR and Western blotting were used to detect the levels of apoptosis and inflammation. The results showed that arbutin had no significant toxic effect on renal tubular epithelial cells in the mentioned concentration range (0-200 μmol/L). When the concentration of arbutin exceeded 100 μmol/L, it showed a protective effect on renal tubular epithelial cells. Arbutin intervention significantly reduced cisplatin-induced apoptosis of renal tubular epithelial cells and the increase of inflammation-related molecules p-p65 and interleukin-18. In addition, arbutin intervention reversed the cisplatin-induced reduction of Bcl-2 in renal tubular epithelial cells. These findings suggest that arbutin can attenuate cisplatin-induced renal tubular epithelial cell injury through anti-apoptotic and anti-inflammatory responses, which may be expected to be a new potential therapeutic drug for acute kidney injury.

Impact of microplastics on children s health / 中国学校卫生

Abstract@#As a new type of pollutant, microplastics have attracted extensive attention. Children in a critical stage of growth and development are vulnerable to microplastics. Summarzing the relevant laws and regulations and the source of microplastics, the paper demonstrates the ways of microplastics entering human body, some toxic effects of microplastics found in recent experimental studies and their potential hazards to children s health are introduced in detail.

Total Saponins of Panax notoginseng Activate Akt/mTOR Pathway and Exhibit Neuroprotection in vitro and in vivo against Ischemic Damage / 中国结合医学杂志

OBJECTIVE@#To reveal the neuroprotective effect and the underlying mechanisms of a mixture of the main components of Panax notoginseng saponins (TSPN) on cerebral ischemia-reperfusion injury and oxygen-glucose deprivation/reoxygenation (OGD/R) of cultured cortical neurons.@*METHODS@#The neuroprotective effect of TSPN was evaluated by 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyl tetrazolium bromide (MTT) assay, flow cytometry and live/dead cell assays. The morphology of dendrites was detected by immunofluorescence. Middle cerebral artery occlusion (MCAO) was developed in rats as a model of cerebral ischemia-reperfusion. The neuroprotective effect of TSPN was evaluated by neurological scoring, tail suspension test, 2,3,5-triphenyltetrazolium chloride (TTC) and Nissl stainings. Western blot analysis, immunohistochemistry and immunofluorescence were used to measure the changes in the Akt/mammalian target of rapamycin (mTOR) signaling pathway.@*RESULTS@#MTT showed that TSPN (50, 25 and 12.5 µ g/mL) protected cortical neurons after OGD/R treatment (P<0.01 or P<0.05). Flow cytometry and live/dead cell assays indicated that 25 µ g/mL TSPN decreased neuronal apoptosis (P<0.05), and immunofluorescence showed that 25 µ g/mL TSPN restored the dendritic morphology of damaged neurons (P<0.05). Moreover, 12.5 µ g/mL TSPN downregulated the expression of Beclin-1, Cleaved-caspase 3 and LC3B-II/LC3B-I, and upregulated the levels of phosphorylated (p)-Akt and p-mTOR (P<0.01 or P<0.05). In the MCAO model, 50 µ g/mL TSPN improved defective neurological behavior and reduced infarct volume (P<0.05). Moreover, the expression of Beclin-1 and LC3B in cerebral ischemic penumbra was downregulated after 50 µ g/mL TSPN treatment, whereas the p-mTOR level was upregulated (P<0.05 or P<0.01).@*CONCLUSION@#TSPN promoted neuronal survival and protected dendrite integrity after OGD/R and had a potential therapeutic effect by alleviating neurological deficits and reversing neuronal loss. TSPN promoted p-mTOR and inhibited Beclin-1 to alleviate ischemic damage, which may be the mechanism that underlies the neuroprotective activity of TSPN.

Follow-up study of fetal cardiac birth defects after prenatal diagnosis and graded counseling / 中华妇产科杂志

Objective:To explore accurate prenatal diagnosis, full-coverage graded counseling and follow-up for the fetus with cardiac birth defects (CBD).Methods:CBD fetus diagnosed prenatal by echocardiography from January 2018 to December 2020 in Guangdong Provincial People's Hospital were enrolled. Fetal CBD was graded (Ⅰ-Ⅵ) according to prognosis and possible operation time after birth, and the classification criteria and common diseases included were proposed. After the prenatal grading counseling, the outcome of the fetus was followed-up. The induced labor rate, live birth rate, prenatal and postnatal ultrasound diagnosis coincidence rate and other indicators were calculated. The disease composition ratio, prognosis of fetus with different grades and the outcome of integrated treatment were analyzed.Results:The detection rate of fetal CBD was up to 16.2% (1 971/12 188), 30 cases of which were excluded. A total of 1 941 cases were included in this study, including 196 cases (10.1%) of gradeⅠ, 433 cases (22.3%) of gradeⅡ, 615 cases (31.7%) of grade Ⅲ, 261 cases (13.4%) of grade Ⅳ, 388 cases (20.0%) of gradeⅤ, 48 cases (2.5%) of grade Ⅵ. Grade Ⅱ and gradeⅢ (the operation time was within 1 year after birth) accounted for 54.0% (1 048/1 941). The distribution of some diseases in different grades had obvious proportion advantage, which was representative. Among 1 747 CBD fetus, 736 cases (induced labor rate 42.1%) chose to terminate pregnancy due to CBD. Of the 1 010 live births, 975 cases (96.5%) had the same prenatal and postnatal diagnosis, 3 cases were missed diagnosis and 32 cases were misdiagnosed. The diagnostic accuracy of live births with severe and complex congenital heart disease was 383 out of 389 (98.5%). A total of 258 cases have received surgery or intervention. The age at the time of surgery or intervention was different among grades（ χ2 =47.3， P<0.001）. With the improvement of prognosis from gradeⅠ to Ⅴ, the live birth rate increased and the induced labor rate decreased accordingly; the difference between grades was significant（ χ2 =623.6， P<0.001）. Conclusions:Prenatal diagnosis and graded counseling is important in the integrated model. Fetal CBD grading could refine post-natal treatment strategies, guide delivery decisions and become an evaluation standard.

Clinical outcomes and follow-up results of 300 cases of transposition of great arteries intervened by the arterial switch operation / 中华实用儿科临床杂志

Objective:To summarize and analyze the efficacy, experience and follow-up results of 300 cases of transposition of the great arteries (TGA) intervened by arterial switch operation.Methods:It was a retrospective, single-center study involving 300 TGA patients intervened by arterial switch operation between January 2010 and December 2017 in Guangdong Provincial People′s Hospital.Their clinical data were retrospectively analyzed.There were 236 male patients and 64 females.Among them, 128 cases (42.7%) were TGA with ventricular septal defect (TGA/VSD), and 172 cases (57.3%) were TGA with intact ventricular septal defect (TGA/IVS). The mean age and weight at operation were (23.8±39.2) cases days, and (3.5±0.8) kg, respectively.There were 193 cases (64.3%) with usual coronary artery patterns, and 107 cases (35.7%) with unusual coronary artery patterns.Among the 107 cases with unusual coronary artery patterns, 21 cases (7.0%) were involved with the intramural coronary artery, and 17 (5.7%) presented the single-ostium coronary pattern.Non normal distribution data were used the Mann- Whitney U test.Categorical measures were compared by Chi- square test or Fisher′ s exact test.Survival probability and freedom from events were calculated by the Kaplan-Meier method, and difference in survival probability by the Log Rank test. Results:All patients were successfully intervened by arterial switch operation, 73.3% of patients with TGA/IVS underwent the surgery within 3 weeks after birth, and 85.9% of patients with TGA/VSD underwent surgery within 3 months.The mean cardiopulmonary bypass time and aortic occlusion time were (193±68) min, and (122±39) min, respectively.Twenty-five patients (8.3%) died in hospital.Thirty cases had low cardiac output syndrome, 1 implanted with a permanent pacemaker due to complete atrioventricular block.A total of 254 patients were followed up for 1 month to 10 years.Three patients with single-ostium coronary pattern died at the follow-up period.The 5-year and 10-year survival rates were both 90.7%.During the follow-up, 49 cases (49/254 cases, 19.3%) had pulmonary artery stenosis, 66 cases (66/254 cases, 26.0%) had aortic valve regurgitation, 47 cases (47/254 cases, 18.5%) had pulmonary valve regurgitation, and 4 (4/254 cases, 1.6%) had aortic anastomotic stenosis.Among the 21 patients (21/254 cases, 8.3%) requiring reintervention, 17 patients (17/254 cases, 6.7%) underwent a total of 18 reinterventions, including 12 interventions of pulmonary artery plasty, 4 of percutaneous balloon pulmonary valvuloplasty, 1 of aortic reconstruction at anastomosis and 1 of pacemaker exchange due to battery exhaustion.Conclusions:Arterial switch operation is the optimal treatment for TGA.The long-term follow-up results of arterial switch operation are satisfactory in TGA children, with a low risk of long-term reoperation.

Case report of pediatric systemic lupus erythematosus combined with hypophosphatasia / 中华实用儿科临床杂志

The clinical data of a child with systemic lupus erythematosus (SLE) and hypophosphatasia (HPP) admitted to the Department of Pediatrics, the Second Hospital of Jilin University in December 2015 were retrospectively analyzed.The patient was a 10-year-old boy who was hospitalized because of fever and facial rashes in the past 4 days.He had a history of HPP for 7 years.His clinical manifestations included skeletal and dental dysplasia, oral ulcers, buccal erythema and renal lesions.Laboratory examination showed a low level of serum alkaline phosphatase, whole blood cell count decreased, antinuclear antibody(ANA) 1∶1 000, anti-double strand DNA antibodies positive, anticardiolipin antibodies positive, complement 3 (C 3) and C 4 decreased.Therefore, he was diagnosed with SLE.After glucocorticoid, immunosuppressant and symptomatic treatment, the child′s condition improved and he discharged from the hospital.He was followed up regularly, and died 2 years after the diagnosis of SLE.SLE complicated with HPP is extremely rare in clinical practice, and the symptoms may overlap.Hence these two diseases should be differentiated.

Case report of pediatric systemic lupus erythematosus combined with hypophosphatasia / 中华实用儿科临床杂志

The clinical data of a child with systemic lupus erythematosus (SLE) and hypophosphatasia (HPP) admitted to the Department of Pediatrics, the Second Hospital of Jilin University in December 2015 were retrospectively analyzed.The patient was a 10-year-old boy who was hospitalized because of fever and facial rashes in the past 4 days.He had a history of HPP for 7 years.His clinical manifestations included skeletal and dental dysplasia, oral ulcers, buccal erythema and renal lesions.Laboratory examination showed a low level of serum alkaline phosphatase, whole blood cell count decreased, antinuclear antibody(ANA) 1∶1 000, anti-double strand DNA antibodies positive, anticardiolipin antibodies positive, complement 3 (C 3) and C 4 decreased.Therefore, he was diagnosed with SLE.After glucocorticoid, immunosuppressant and symptomatic treatment, the child′s condition improved and he discharged from the hospital.He was followed up regularly, and died 2 years after the diagnosis of SLE.SLE complicated with HPP is extremely rare in clinical practice, and the symptoms may overlap.Hence these two diseases should be differentiated.

Analysis of a child with holoprosencephaly due to variant of SIX3 gene / 中华医学遗传学杂志

OBJECTIVE@#To explore the genetic basis of a child with holoprosencephaly.@*METHODS@#Genomic DNA of the child was extracted and subjected to whole exome sequencing. Suspected variant was verified by Sanger sequencing of her family members.@*RESULTS@#Cranial MRI suggested lobulated holoprosencephaly with partial absence of corpus callosum. Genetic testing revealed that she has carried a heterozygous c.517C>G (p.His173Asp) variant of the SIX3 gene, for which both of her parents were of wild type. Based on the American College of Medical Genetics and Genomics guidelines, the c.517C>G variant of SIX3 gene was predicted to be pathogenic (PS2+PM1+PM2+PM5+PP3).@*CONCLUSION@#The SIX3 gene c.517C>G variant probably underlay the multiple malformations in this child. Above finding has enabled her definite diagnosis.

Efficacy observation of Da Vinci robotic-assisted laparoscopic resection for liver benign tumors / 肿瘤研究与临床

Objective:To explore the safety and feasibility of Da Vinci robotic-assisted laparoscopic resection in treatment of liver benign tumors.Methods:The clinical data of 62 patients with liver benign tumors admitted to Fujian Medical University Union Hospital from January 2016 to December 2019 were retrospectively analyzed. All patients were divided into 2 groups: 25 cases undergoing Da Vinci robotic-assisted laparoscopic resection (the robotic group) and 37 cases undergoing conventional laparoscopic resection for liver benign tumors (the laparoscopic group). The operation duration, intraoperative blood loss, postoperative abdominal drainage tube removal time, incidence of postoperative complications, postoperative hospital stay time of both groups were compared.Results:Operations of all 62 patients were successfully completed. The operation time of the robotic group was longer than that of the laparoscopic group [(192±52) min vs. (158±41) min], intraoperative blood loss of the robotic group was less than that of the laparoscopic group [(159±67) ml vs.(213±59) ml], and differences were statistically significant between the two groups (both P < 0.05). The postoperative abdominal drainage tube removal time of the robotic group and the laparoscopic group was (7.0±1.5) d and (7.2±1.3) d, the incidence of postoperative complications was 8.0% (2/25) and 5.4% (2/37), and the postoperative hospital stay time was (7.0±2.4) d and (7.3±2.2) d, respectively; and differences were statistically significant between both groups (all P > 0.05). Conclusion:Da Vinci robotic-assisted laparoscopic resection is a safe and effective operation method in treatment of liver benign tumors with advantages of small wound and less blood loss.

Analysis of diagnosis and treatment of scimitar syndrome in 13 cases / 中华胸心血管外科杂志

Objective:To investigate the clinical features, diagnosis, treatment and prognosis of Scimitar syndrome.Methods:A retrospective analysis of clinical data of 13 children with scimitar syndrome from January 2013 to November 2020, including clinical symptoms, chest X-ray, echocardiography, cardiac CT and cardiac catheterization prognosis and follow-up.Results:13 children with scimitar syndrome were diagnosed, including 7 girls and 6 boys with a average age of 17 months(21 days to 10 years).3 cases <5 kg in weight. Ten patients presented with the infantile form and 3 with the adult form of scimitar syndrome. 13 infantile form had lower respiratory tract infections, heart failure, and growth retardation, of which 8 cases were with severe pulmonary arterial hypertension. 3 adult form were diagnosed because of heart murmur. 12 cases had coexisting cardiac lesions, including 12 atrial septal defect, 2 patent ductus arteriosus, 1 right ventricular double outlet/ventricular septal defect, 4 right lung and right pulmonary artery dysplasia, 2 right lung dysplasia, 6 additional systemic arterial supply to the right lung. Ten patients had pulmonary venous drainage correction surgery, one patient only underwent right ventricular double-outlet correction, three patients died of severe pulmonary hypertension; one patient lost the opportunity of surgery due to obstructive pulmonary hypertension, and one patient was complicated by nervous system The disease gave up treatment. One corrected case was stenosed during discharge and 2 corrected children became stenotic during follow-ups.Conclusion:Found with the median or dextrocardial heart, recurrent respiratory infections, or unexplained pulmonary hypertension, the possibility of scimitar syndrome should be considered. The combination of echocardiography and cardiac CTA can confirm the diagnosis as soon as possible. The risk factors for mortality included infantile form and severe preoperative pulmonary hypertension. Long-term follow-up is still required after operation, and surgical intervention is required again if necessary.

Analysis school absenteeism in Nutrition Improvement Program for Rural Compulsory Education Students area from 2012-2017 / 中国学校卫生

Objective@#To investigate the changes of school absenteeism among students received Nutrition Improvement Program for Rural Compulsory Education Student (NIPCES), and to provide basic data for further nutritional health improvement for rural students.@*Methods@#Among 699 NIPCES monitored counties among 22 provinces across western and central China, 10%-30% of elementary and junior high schools were randomly selected as monitoring schools within each stratification according to the food supply pattern (school canteen, company and mix) in each county, school absenteeism information were collected and analyzed from 2012 to 2017.@*Results@#The total rate of student absenteeism was 26.0 per 10 000. From 2012 to 2016, it dropped from 30.8 per 10 000 to 23.4 per 10 000 year by year, but it increased again in 2017(28.2 per 10 000). The rate of school absenteeism in the western region (29.4 per 10 000) was higher than that in the central region (21.5 per 10 000); The sick leave rate (15.0 per 10 000) of primary school students was higher than that of junior high school students (13.4 per 10 000), and the rate of personal leave absences (16.9 per 10 000) of junior high school students was higher than that of elementary school students(9.9 per 10 000); the absentees rate in mixed-food supply schools (28.5 per 10 000) was higher than that in canteen food supply schools(26.4 per 10 000), the latter was higher than that of company food supply schools(25.0 per 10 000). The rate of sick leave absenteeism was highest in December(18.4 per 10 000), and the rate of personal leave absenteeism in June was highest(14.6 per 10 000).@*Conclusion@#The rate of school absenteeism among students in the NIPCES area showed an overall downward trend year by year. The absentee rate of students varies by regions, grade, food supply patterns and school locations.

Risk factors for metabolic bone disease of prematurity in very/extremely low birth weight infants: a multicenter investigation in China / 中国当代儿科杂志

OBJECTIVE@#To investigate the incidence rate and risk factors for metabolic bone disease of prematurity (MBDP) in very low birth weight/extremely low birth weight (VLBW/ELBW) infants.@*METHODS@#The medical data of 61 786 neonates from multiple centers of China between September 1, 2013 and August 31, 2016 were retrospectively investigated, including 504 VLBW/ELBW preterm infants who met the inclusion criteria. Among the 504 infants, 108 infants diagnosed with MBDP were enrolled as the MBDP group and the remaining 396 infants were enrolled as the non-MBDP group. The two groups were compared in terms of general information of mothers and preterm infants, major diseases during hospitalization, nutritional support strategies, and other treatment conditions. The multivariate logistic regression analysis was used to investigate the risk factors for MBDP.@*RESULTS@#The incidence rate of MBDP was 19.4% (88/452) in VLBW preterm infants and 38.5% (20/52) in ELBW preterm infants. The incidence rate of MBDP was 21.7% in preterm infants with a gestational age of < 32 weeks and 45.5% in those with a gestational age of < 28 weeks. The univariate analysis showed that compared with the non-MBDP group, the MBDP group had significantly lower gestational age and birth weight, a significantly longer length of hospital stay, and a significantly higher incidence rate of extrauterine growth retardation (@*CONCLUSIONS@#A lower gestational age, hypocalcemia, extrauterine growth retardation at discharge, and neonatal sepsis may be associated an increased risk of MBDP in VLBW/ELBW preterm infants. It is necessary to strengthen perinatal healthcare, avoid premature delivery, improve the awareness of the prevention and treatment of MBDP among neonatal pediatricians, and adopt positive and reasonable nutrition strategies and comprehensive management measures for preterm infants.