Objective To evaluate prenatal sonograpic findings and clinical value in fetus with congenital portal-hepatic venous shunt.Methods The prenatal ultrasonographic characteristic of ten cases of congenital portal-hepatic venous shunt were analyzed and summarized.Results Three cases had portal vein-left hepatic venous shunt,two cases with portal vein-right hepatic venous shunt,two cases with situs inversus of left right portal vein,and three cases with multiple shunt among portal and hepatic veins.All the 10 cases with portal-hepatic venous shunt had common sonographic features,①Two-dimensional ultrasound revealed the distal hepatic veins.The hepatic vein and portal vein were both visualized on the same plane and connected by an anechoic area.②Vascular signal were noted inside the anechoic area.③Blood flow inside the anechoic area demonstrated by spectral Doppler was similar to that of the portal vein.Conclusions Prental ultrasonography is valuable in the diagnosis of fetal portal-hepatic venous shunt.

Objective To evaluate the application value of bedside lung ultrasound in diagnosis of neonatal severe pneumonia.Methods Bedside lung ultrasound and chest X-ray were performed in 59 the newborn patients admitted to the newborn department for suspected severe pneumonia,from October 2015 to March 2016.According to the characteristics of the chest X-ray,patients were divided into consolidation group (widely spot shadow in double lung and airbronchograms) and the unconsolidation group (enlargement,fuzzy texture in double lung or small patch sample changes) Differences in ultrasonic characteristics were compared between the two groups.Results Ultrasonic image characteristics were pulmonary consolidation with air bronchograms,A-line disappearance,pleural line abnormalities.Among 23 patients with chest X-ray,21 patients were found pulmonary consolidation by lung ultrasound,and all 36 patients in unconsolidation group were not found pulmonary consolidation by lung ultrasound,and there was no significant difference in the detection rate by lung ultrasound between the two groups (P＞0.05) The pulmonary ultrasound results of 20 cases in the consolidation group showed A-line disappearance,37 cases in the unconsolidation group showed A-line disappearance.There was no statistical significant differences between the two groups (P＞0.05).The pulmonary ultrasound results showed 11 cases in the consolidation group and 15 cases in the unconsolidation group with pleural line abnormalities,there was no statistically significant differences between the two groups (P＞O.05).Conclusions Ultrasound image characteristics of neonatal severe pneumonia include pulmonary consolidation and air bronchograms,A-line disappearance,pleural line abnormalities.Compared lung ultrasound and chest X-ray result,there was no difference between consolidation group and unconsolidation group.Both of the two methods can be used for evaluating the diagnosis of pneumonia.

Objective: To evaluate the clinical value of color Doppler signal at the nasal lip in the diagnosis of fetal cleft palate. Methods: Twenty fetuses diagnosed with fetal cleft lip and cleft palate by targeted ultrasonography and magnetic resonance imaging were enrolled in this study. Color Doppler was used during each ultrasound scan. All prenatal diagnoses were confirmed either by postnatal follow-up or autopsy. The location and degree of cleft lip and palate was also recorded. During inspiration or swallowing color signal acrossed the defects at the fetal palate was indicated cleft palate, and no color signal acrossed the defects at the fetal palate was indicated without cleft palate. Two dimensional ultrasound combined with color Doppler was used to diagnose cleft lip and palate in midsagittal plane or paratsagittal plane, then compared with the results of post-natal evaluation or abortion, and the value of color signal at the nasal lip in the diagnosis of fetal cleft palate was analyzed. Results: Twenty cases of postpartum fetus (including post-natal evaluation or abortion ) confirmed 15 cases of cleft lip and palate, 5 cases of cleft lip (2 cases with upper alveolar cleft). For prenatal diagnosis, 5 cases of cleft lip and 15 cases of cleft lip and palate were diagnosed by two-dimensional ultrasound, of which 3 cases of cleft lip and palate were misdiagnosed as cleft lip, whereas 3 cases of cleft lip were misdiagnosed as cleft lip and palate with 2 cases combined with upper alveolar cleft. Two-dimensional ultrasound in the diagnosis of cleft lip and palate is consistent with postnatal results by additional evaluation of the color signal in nasolabial area in median sagittal section. In the cleft lip and cleft lip with the alveolar cleft fetuses, the color signal in nasolabial area was undetectable during fetal respiratory-like movement or swallowing by ultrasound.However, it was detectable in cleft lip and palate fetuses. A total of 3 cases of simple cleft lip and 17 cases of cleft lip and palate were detected by magnetic resonance imaging(MRI). In 2 cases of cleft lip and palate diagnosed by MRI, no blood flow signal was detected at the nasolabial area. Conclusions The connected color signal in nasolabial area assessed by color Doppler ultrasound during fetal respiratory-like movement or swallowing showed an promising value for prenatal diagnosis of cleft palate. Cleft palate could be ruled out if the connected color signal was undetectable in nasolabial area.

Objective To evaluate the clinical value of color Doppler signal at the nasal lip in the diagnosis of fetal cleft palate . Methods Twenty fetuses diagnosed with fetal cleft lip and cleft palate by targeted ultrasonography and magnetic resonance imaging were enrolled in this study . Color Doppler was used during each ultrasound scan . All prenatal diagnoses were confirmed either by postnatal follow‐up or autopsy . T he location and degree of cleft lip and palate was also recorded . During inspiration or swallowing color signal acrossed the defects at the fetal palate was indicated cleft palate ,and no color signal acrossed the defects at the fetal palate was indicated without cleft palate . T wo dimensional ultrasound combined with color Doppler was used to diagnose cleft lip and palate in midsagittal plane or paratsagittal plane ,then compared with the results of post‐natal evaluation or abortion ,and the value of color signal at the nasal lip in the diagnosis of fetal cleft palate was analyzed . Results Twenty cases of postpartum fetus ( including post‐natal evaluation or abortion ) confirmed 15 cases of cleft lip and palate ,5 cases of cleft lip ( 2 cases with upper alveolar cleft) . For prenatal diagnosis ,5 cases of cleft lip and 15 cases of cleft lip and palate were diagnosed by two‐dimensional ultrasound ,of which 3 cases of cleft lip and palate were misdiagnosed as cleft lip ,w hereas 3 cases of cleft lip were misdiagnosed as cleft lip and palate with 2 cases combined with upper alveolar cleft . T wo‐dimensional ultrasound in the diagnosis of cleft lip and palate is consistent with postnatal results by additional evaluation of the color signal in nasolabial area in median sagittal section . In the cleft lip and cleft lip with the alveolar cleft fetuses ,the color signal in nasolabial area was undetectable during fetal respiratory‐like movement or swallowing by ultrasound .However ,it was detectable in cleft lip and palate fetuses . A total of 3 cases of simple cleft lip and 17 cases of cleft lip and palate were detected by magnetic resonance imaging( M RI) . In 2 cases of cleft lip and palate diagnosed by M RI ,no blood flow signal was detected at the nasolabial area . Conclusions The connected color signal in nasolabial area assessed by color Doppler ultrasound during fetal respiratory‐like movement or swallowing showed an promising value for prenatal diagnosis of cleft palate . Cleft palate could be ruled out if the connected color signal was undetectable in nasolabial area .

Objective To construct a database containing multiple kinds of diseases that can provide "real world"data for first-aid clinical research. Methods Structured or non-structured information from hospital information system, laboratory information system, emergency medical system, emergency nursing system and bedside monitoring instruments of patients who visited department of emergency in PLA General Hospital from January 2014 to January 2018 were extracted. Database was created by forms, code writing, and data process. Results Emergency Rescue Database is a single center database established by PLA General Hospital. The information was collected from the patients who had visited the emergency department in PLA General Hospital since January 2014 to January 2018. The database included 530 585 patients' information of triage and 22 941 patients' information of treatment in critical rescue room, including information related to human demography, triage, medical records, vital signs, lab tests, image and biological examinations and so on. There were 12 tables (PATIENTS, TRIAGE_PATIENTS, EMG_PATIENTS_VISIT, VITAL_SIGNS, CHARTEVENTS, MEDICAL_ORDER, MEDICAL_RECORD, NURSING_RECORD, LAB_TEST_MASTER, LAB_RESULT, MEDICAL_EXAMINATION, EMG_INOUT_RECORD) that containing different kinds of patients' information. Conclusions The setup of high quality emergency databases lay solid ground for scientific researches based on data. The model of constructing Emergency Rescue Database could be the reference for other medical institutions to build multiple-diseases databases.

Objective:To analysis the income and expenditure of HIV-positive pregnant women and the catastrophic health expenditure of their households in high AIDS endemic areas in Liangshan prefecture.Methods:From December 2018 to January 2019, a total of 250 pregnant women were recruited from 2 of 17 counties in Liangshan Prefecture, including 133 HIV-positive pregnant women and 117 HIV-negative pregnant women. The data, including basic information of pregnant women, basic information of the family, annual family income in 2017, annual family health expenditure in 2017, and maternal and child-related expenditure in 2017, were collected for analyzing the incidence of catastrophic health expenditure in the family.Results:The average annual income and average annual health expenditure of HIV-positive pregnant women households were 7 000 CNY and 2 000 CNY, while those of HIV-negative pregnant women households were 10 000 CNY and 3 000 CNY, respectively. Based on the criteria of 15%, 25% and 40%, the incidence of catastrophic health expenditure of HIV-positive pregnant households was 39.10%, 33.83% and 27.82%, with average differences of 34.84%, 31.17% and 26.65%, respectively, while that of HIV-negative pregnant women households was 38.46%, 33.33% and 23.93%, with average differences of 31.68%, 28.35% and 24.22%, respectively.Conclusion:The incidence of catastrophic health expenditure of pregnant households in high AIDS endemic areas in Liangshan prefecture is high. The incidence of HIV-positive families is slightly higher than that of HIV-negative families. We suggest that medical insurance compensation in Liangshan prefecture should be improved to reduce the impact of catastrophic health expenditure.

Objective:To analysis the income and expenditure of HIV-positive pregnant women and the catastrophic health expenditure of their households in high AIDS endemic areas in Liangshan prefecture.Methods:From December 2018 to January 2019, a total of 250 pregnant women were recruited from 2 of 17 counties in Liangshan Prefecture, including 133 HIV-positive pregnant women and 117 HIV-negative pregnant women. The data, including basic information of pregnant women, basic information of the family, annual family income in 2017, annual family health expenditure in 2017, and maternal and child-related expenditure in 2017, were collected for analyzing the incidence of catastrophic health expenditure in the family.Results:The average annual income and average annual health expenditure of HIV-positive pregnant women households were 7 000 CNY and 2 000 CNY, while those of HIV-negative pregnant women households were 10 000 CNY and 3 000 CNY, respectively. Based on the criteria of 15%, 25% and 40%, the incidence of catastrophic health expenditure of HIV-positive pregnant households was 39.10%, 33.83% and 27.82%, with average differences of 34.84%, 31.17% and 26.65%, respectively, while that of HIV-negative pregnant women households was 38.46%, 33.33% and 23.93%, with average differences of 31.68%, 28.35% and 24.22%, respectively.Conclusion:The incidence of catastrophic health expenditure of pregnant households in high AIDS endemic areas in Liangshan prefecture is high. The incidence of HIV-positive families is slightly higher than that of HIV-negative families. We suggest that medical insurance compensation in Liangshan prefecture should be improved to reduce the impact of catastrophic health expenditure.

The core components of the Hippo signaling pathway encompass upstream regulatory molecules,core kinase cascade complexes,and downstream transcriptional regulation complexes.This pathway modulates cellular behaviors by influencing the effector molecules of its core components and plays a pivotal role in immune regulation.Effector molecules,such as Yes-associated protein(YAP),transcriptional coactivator with PDZ-binding motif(TAZ),transcriptional enhanced associate domain transcriptional factor(TEAD),monopolar spindle-one binder(MOB1),large tumor suppressor(LATS),can stimulate fibroblast-like synovial cell migration and invasion in rheumatoid arthritis,regulate osteoarthritis disease progression,promote pathological new bone formation in ankylosing spondylitis,sustain submandibular gland development while delaying Sjogren's syndrome progression,mediate alpha-smooth muscle actin in systemic sclerosis,and refine the regulation of target genes associated with pulmonary fibrosis.This article provides an overview of the regulatory mechanisms involving Hippo signaling pathway-related effector molecules in the pathogenesis and progression of rheumatic immune system diseases,to serve as a reference for exploring novel therapeutic targets of rheumatic immune system diseases.

Objective:To explore the performance of the commonly used whole blood C-reactive protein (CRP) detection systems and give related recommendation on the performance requirements of detection systems.Methods:A total of 7 540 venous blood samples from 26 maternal, child and children′s hospitals were collected to conduct this multi-center study on the analytical performance of 5 commonly used whole blood CRP detection systems from March to April in 2019. The blank check, carryover, repeatability, intermediate precision, linearity, sample stability, influence of hematocrit/triglyceride/bilirubin, comparison with SIEMENS specific protein analyzer and trueness were evaluated. The 5 systems included BC-5390CRP autohematology analyzer, AstepPLUS specific protein analyzer, Ottoman-1000 Automated Specific Protein POCT Workstation, i-CHROMA Immunofluorometer equipment Reader and Orion QuikRead go detecting instrument. The 5 systems were labeled as a, b, c, d and e randomly.Results:Within the 5 systems, all values of blank check were less than 1.00 mg/L, the carryovers were lower than 1.00%. The repeatability of different ranges of CRP concentrations including 3.00-10.00, 10.00-30.00 and>30.00 mg/L were less than 10.00%, 6.00% and 5.00%, respectively, and the intermediate precision was less than 10.00%. The linearity correlation coefficients of the 5 systems were all above 0.975, while the slope was within 0.950-1.050. Whole blood samples were stable within 72 hours both at room temperature (18-25 ℃) and refrigerated temperature (2-8 ℃). The CRP results were rarely influenced by high triglyceride or bilirubin, except for the immmunoturbidimetric test based on microparticles coated with anti-human CRP F(ab) 2 fragments. When triglyceride was less than 15.46 mmol/L, the deviation of CRP was less than 10.00%. When bilirubin was less than 345.47 μmol/L, the deviation of CRP was less than 10.00%. CRP was more susceptible to Hct on the systems without Hct correction. The deviation of CRP between different Hct dilution concentration and 40% dilution concentration can reach as high as 67.48%. The correlation coefficients ( r) of 5 systems were all more than 0.975 in the range of 0-300.00 mg/L compared with Siemens specific protein analyzer. All systems passed the trueness verification using the samples with specified values of 12.89 and 30.60 mg/L. Conclusion:The performance of 5 systems can basically meet the clinical needs, but it is suggested that the whole blood CRP detection system without automatic Hct correction should be modified manually.

Objective:To investigate the efficacy and safety of plasma exchange(PE) in the treatment of autoimmune hemolytic anemia in children.Methods:The data from 8 hospitals in China during November 2014 to April 2017 were collected, and the clinical characteristics of PE in children with AHA were analyzed retrospectively.Results:A total of 21 children with AHA were included in the study, including 17 cases from PICU and 4 cases from pediatric kidney ward, with 11 boys and 10 girls, and the median age was 3.64(0.25, 11.10)years old, and median hospital stay was 12(4, 45)days.There were 15 cases(71.4%) with infection, 2 cases(9.5%)with autoimmune diseases, 4 cases(19.0%) with unknown.Consciousness disturbance occurred in 4 patients before replacement and recovered to normal after PE.The volume of blood decreased in two cases(9.5%) and completely relieved.There were 20 cases of anemia (95.2%), 15 cases were normal after PE, and 5 cases were improved.Jaundice occurred in 18 cases (85.7%), 12 cases were normal after PE, 6 cases were improved.Hepatosplenomegaly was found in 11 cases, 10 cases were normal after PE, 1 case was improved.After PE, the hemoglobin and red blood cell count increased, while the total bilirubin, indirect bilirubin, urea nitrogen and lactate dehydrogenase decreased, there were significant differences between pre-and post-replacement ( P<0.05). Only 1 case had allergic reaction, which was improved after symptomatic treatment, and PE was continued.After PE, 2 cases (9.5%) had complete remission, 16 cases (76.2%) had partial remission and 3 cases (14.3%) had been discharged. Conclusion:PE therapy can obviously improve the clinical symptoms and laboratory indexes of children with AHA who have failed to respond to conservative treatment.It can be used as a treatment measure for children with severe AHA and has a good safety.