Objective:To explore a classification method for critically ill emergency patients undergoing intra-hospital transport based on cluster analysis, and analyze the changes in vital signs between different categories, so as to provide references for targeted nursing interventions.Methods:Using the convenient sampling method, a total of 416 critically ill patients who were transferred from emergency department to ICU in 2 hospital areas of Beijing Tongren Hospital, Capital Medical University from January to December 2021 were selected as the research objects. General data and vital signs before and after transport were collected. Five vital signs, including body temperature, heart rate, respiratory rate, systolic blood pressure and peripheral capillary blood oxygen saturation measured at the last time before transport, were taken as cluster variables. Patients were classified based on second-order cluster analysis and compared among different categories.Results:The 416 patients included in this study could be divided into 3 categories, such as stable group, low oxygen saturation group and high heart rate group, and the clustering contour coefficient was 0.30. The systolic blood pressure before and after transport of the three groups were compared, and the differences were statistically significant (all P<0.05). Moreover, there was a statistically significant difference in respiratory frequency between the low oxygen saturation group and the high heart rate group (both P<0.05) . Conclusions:It was of great significance to monitor the vital signs of the critically ill patients transferred in the hospital, and the classified management of patients can better guide the clinical practice of transportation.

OBJECTIVE: To explore the clinical characteristics and genetic etiology for a child with atypical Hemolytic uremic syndrome (aHUS) in conjunct with nephrotic level proteinuria. METHODS: A child patient who had visited the Affiliated Hospital of Qingdao University on June 25, 2020 was selected as the study subject. Clinical data of the patient was collected. Whole exome sequencing (WES) was carried out for the child, and candidate variant was verified by Sanger sequencing of the child and his parents. RESULTS: The child, an 8-month-old male, had presented mainly with edema, oliguria, hematuria, nephrotic level proteinuria, anemia, thrombocytopenia, increased creatinine and urea, hypercholesterolemia but normal complement levels. Genetic testing revealed that he has harbored compound heterozygous variants of the DGKE gene, namely c.12_18dupGAGGCGG (p.P7fs*37) and c.1042G>T (p.D348Y), which were respectively inherited from his father and mother. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), the variants were classified as likely pathogenic and variant of uncertain significance, respectively. By combining his clinical manifestations and results of genetic testing, the child was diagnosed with aHUS with nephrotic level proteinuria. CONCLUSION For infants and young children with aHUS in conjunct with nephrotic level proteinuria, variants of the DGKE gene should be screened. Above finding has expanded the mutational spectrum of the DGKE gene.
Infant ; Female ; Humans ; Child ; Male ; Child, Preschool ; Atypical Hemolytic Uremic Syndrome/diagnosis* ; Mutation ; Genetic Testing ; Thrombocytopenia/genetics* ; Proteinuria/genetics*

Objective:To explore the therapeutic characteristics of population with gout achieving treat-to-target (T2T) indicators through real-world research and evaluate their safety.Methods:A total of 3 287 patients diagnosed with gout by rheumatologists in 21 first-class tertiary hospitals in 10 provinces, municipalities, and autonomous regions in China from January 2015 to December 2021 were included in this polycentric cross-sectional study. The database included patients′ general information, disease characteristics, and clinical application of traditional Chinese and Western medicine treatment measures. SPSS and Excel software were used for data analysis. Frequency analysis, cluster analysis, and factor analysis were used to summarize the characteristics and rules of treatment measures for patients with gout who achieved the target after treatment. The occurrence of adverse events (AE) was recorded during treatment.Results:After treatment, 691 visits (7%) achieved the serum urate (SUA) target, and the most frequent use of urate-lowering therapy (ULT) was febuxostat, followed by benzbromarone. The most common treatment options were following: GroupⅠ: traditional Chinese medicine (TCM) decoction-TCM external treatment-physical exercise-proprietary Chinese medicine; GroupⅡ: ferulic acid-nonsteroidal anti-inflammatory drugs (NSAIDs); Group Ⅲ: allopurinol-sodium bicarbonate-benzbromarone; Group Ⅳ: glucocorticoid-colchicine; Group Ⅴ: febuxostat. A total of 5 898 visits (60%) chieved manifestations of joint pain VAS scores target, and the most frequently used drug to control joint symptoms was NSAIDs. The frequency of use of drugs to control joint symptoms were 2 118 times (usage rate reached 35.9%), while the frequency of ULT were 2 504 times (usage rate reached 42.5%), which was higher than the joint symptom control drug. The most common treatment options were following: Group Ⅰ: proprietary Chinese medicine-TCM decoction-TCM external treatment-physical exercise; Group Ⅱ: NSAIDs-colchicine hormones; Group Ⅲ: allopurinol, Group Ⅳ: benzbromarone; Group Ⅴ: febuxostat. A total of 59 adverse events occurred during treatment.Conclusion:The proportions of gout patients who reach target serum urate level & good control of joint symptoms are both very low, and ULT and anti-inflammatory prescription patterns are very different from international guidelines, so it is necessary to strengthen the standardized management of gout patients. At the same time, life intervention measures account for a certain proportion of the treatment plans for the T2T population, and further exploration is needed.

Background: and Purpose To examine the clinical and safety outcomes after endovascular treatment (EVT) for acute basilar artery occlusion (BAO) with different anesthetic modalities. Methods: This was a retrospective analysis using data from the Endovascular Treatment for Acute Basilar Artery Occlusion (ATTENTION) registry. Patients were divided into two groups defined by anesthetic modality performed during EVT: general anesthesia (GA) or non-general anesthesia (non-GA). The association between anesthetic management and clinical outcomes was evaluated in a propensity score matched (PSM) cohort and an inverse probability of treatment weighting (IPTW) cohort to adjust for imbalances between the two groups. Results: Our analytic sample included 1,672 patients from 48 centers. The anesthetic modality was GA in 769 (46.0%) and non-GA in 903 (54.0%) patients. In our primary analysis with the PSM-based cohort, non-GA was comparable to GA concerning the primary outcome (adjusted common odds ratio [acOR], 1.01; 95% confidence interval [CI], 0.82 to 1.25; P=0.91). Mortality at 90 days was 38.4% in the GA group and 35.8% in the non-GA group (adjusted risk ratio, 0.95; 95% CI, 0.83 to 1.08; P=0.44). In our secondary analysis with the IPTW-based cohort, the anesthetic modality was significantly associated with the distribution of modified Rankin Scale at 90 days (acOR: 1.45 [95% CI: 1.20 to 1.75]). Conclusion In this nationally-representative observational study, acute ischemic stroke patients due to BAO undergoing EVT without GA had similar clinical and safety outcomes compared with patients treated with GA. These findings provide the basis for large-scale randomized controlled trials to test whether anesthetic management provides meaningful clinical effects for patients undergoing EVT.

Objective:In general, patients with seropositive rheumatoid arthritis (RA) are considered to show an aggressive disease course. However, the relationship between the two subgroups in disease severity is controversial. Our study is aimed to compare the clinical characteristics and prognosis of double-seropositive and seronegative RA in China through a real-world large scale study.Methods:RA patients who met the 1987 American College of Rheumatology (ACR) classification criteria or the 2010 ACR/European Anti-Rheumatism Alliance RA classification criteria, and who attended the 10 hospitals across the country from September 2015 to January 2020, were enrolled. According to the serological status, patients were divided into 4 subgroups [rheumatoid factor (RF)(-) anti-cyclic citrullinated peptide (CCP) antibody (-), RF(+), RF(+) anti-CCP antibody(+), anti-CCP antibody(+)] and compared the disease characteristics and treatment response. One-way analysis of variance was used for measurement data that conformed to normal distribution, Kruskal-Wallis H test was used for measurement data that did not conform to normal distribution; paired t test was used for comparison before and after treatment within the group if the data was normally distributed else paired rank sum test was used; χ2 test was used for count data. Results:① A total of 2 461 patients were included, including 1 813 RF(+) anti-CCP antibody(+) patients (73.67%), 129 RF(+) patients (5.24%), 245 RF(-) anti-CCP antibody(-) patients (9.96%), 74 anti-CCP antibody(+) patients (11.13%). ② Regardless of the CCP status, RF(+) patients had an early age of onset [RF(-) anti-CCP antibody(-) (51±14) years old, anti-CCP antibody(+) (50±15) years old, RF(+) anti-CCP antibody(+) (48±14) years old, RF(+)(48±13) years old, F=3.003, P=0.029], longer disease duration [RF(-) anti-CCP antibody(-) 50 (20, 126) months, anti-CCP antibody(+) 60(24, 150) months, RF(+) anti-CCP antibody(+) 89(35, 179) months, RF(+) 83(25, 160) months, H=22.001, P<0.01], more joint swelling counts (SJC) [RF(-) anti-CCP antibody(-) 2(0, 6), Anti-CCP antibody(+) 2(0, 5), RF(+) anti-CCP antibody(+) 2(0, 7), RF(+) 2(0, 6), H=8.939, P=0.03] and tender joint counts (TJC) [RF(-) anti-CCP antibody(-) 3(0, 8), anti-CCP antibody(+) 2(0, 6), RF(+) anti-CCP antibody(+) 3(1, 9), RF(+) 2(0, 8), H=11.341, P=0.01] and the morning stiff time was longer [RF(-) anti-CCP antibody(-) 30(0, 60) min, anti-CCP antibody(+) 20(0, 60) min, RF(+) anti-CCP antibody(+) 30(10, 60) min, RF(+) 30(10, 60) min, H=13.32, P<0.01]; ESR [RF(-) anti-CCP antibody(-) 17(9, 38) mm/1 h, anti-CCP antibody(+) 20(10, 35) mm/1 h, RF(+) anti-CCP antibody(+) 26(14, 45) mm/1 h, RF(+) 28(14, 50) mm/1 h, H=37.084, P<0.01] and CRP [RF(-) anti-CCP antibody(-) 2.3 (0.8, 15.9) mm/L, Anti-CCP antibody(+) 2.7(0.7, 12.1) mm/L, RF(+) anti-CCP antibody(+) 5.2(1.3, 17.2) mm/L, RF (+) 5.2(0.9, 16.2) mm/L, H=22.141, P<0.01] of the RF(+)patients were significantly higher than RF(-) patients, and RF(+) patients had higher disease severity(DAS28-ESR) [RF(-) anti-CCP antibody(-) (4.0±1.8), anti-CCP antibody(+) (3.8±1.6), RF(+) anti-CCP antibody(+) (4.3±1.8), RF(+) (4.1±1.7), F=7.269, P<0.01]. ③ The RF(+) anti-CCP antibody(+) patients were divided into 4 subgroups, and it was found that RF-H anti-CCP antibody-L patients had higher disease severity [RF-H anti-CCP antibody-H 4.3(2.9, 5.6), RF-L anti-CCP antibody-L 4.5(3.0, 5.7), RF-H anti-CCP antibody-L 4.9(3.1, 6.2), RF-L anti-CCP antibody-H 2.8(1.8, 3.9), H=20.374, P<0.01]. ④ After 3-month follow up, the clinical characteristics of the four groups were improved, but there was no significant difference in the improvement of the four groups, indicating that the RF and anti-CCP antibody status did not affect the remission within 3 months. Conclusion:Among RA patients, the disease activity of RA patients is closely related to RF and the RF(+) patients have more severe disease than RF(-) patients. Patients with higher RF titer also have more severe disease than that of patients with low RF titer. After 3 months of medication treatment, the antibody status does not affect the disease remission rate.

Objective: To identify the risk factors for early fluid overload(FO)following repair in the pediatric patients with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) complicated with moderate or severe left ventricular dysfunction (left ventricular ejection fraction [LVEF]<50%) and evaluate the effect on clinical outcomes. Methods: Forty-three pediatric patients with ALCAPA complicated with moderate or severe left ventricular dysfunction, aged 2-128 months, weighing 4.5-34.5 kg, with New York Heart Association Ⅲ or Ⅳ, undergoing ALCAPA repair, were enrolled in this study.The pediatric patients were divided into FO≥5% group (n=14) and FO<5% group (n=29) according to the FO developed within 24 h after operation. The pediatric Risk, Injury, Failure, Loss, and End-Stage Renal Disease criterion was used to diagnose acute kidney injury developed after operation. Factors including age, height, weight, preoperative LVEF, preoperative biomarkers, operative data, postoperative ventilation time, duration of intensive care unit(ICU)stay and related postoperative clinical outcome parameters were recorded.The risk factors of which P values were less than 0.05 would enter the multivariate logistic regression analysis to stratify the risk factors for FO≥5% developed within 24 h after operation.The effect of FO≥5% on postoperative severe acute kidney injury (Injury and Failure), ventilation time, duration of ICU stay and etc. was assessed. Results: Fourteen cases developed early postoperative FO≥5%, and the incidence was 33%.The results of the logistic regression analysis showed that lower preoperative LVEF was an independent risk factor for early postoperative FO≥5% (P<0.05). Compared with FO<5% group, the postoperative ventilation time and duration of ICU stay were significantly prolonged, the number of pediatric patients who developed pulmonary infection and required reintubation was increased, the number of pediatric patients in whom duration of ICU stay was more than 14 days was increased (P<0.05), and no significant change was found in the other parameters of clinical outcomes in FO≥5% group (P>0.05). Conclusion Lower preoperative LVEF is a risk factor for early postoperative FO in pediatric patients with ALCAPA complicated with a moderate or severe left ventricular dysfunction undergoing repair, and it is not helpful for clinical outcomes in pediatric patients when postoperative early FO≥5% occurs.

Objective Toinvestigatethecharacteristicsofclinicalpathologyand MRIofintracranialsolitaryfibroustumor/hemangiopericytoma (SFT/HPC).Methods ThisstudyanalyzedtheMRIimages,pathologicalandclinicaldataof14SFT/HPCpatientsretrospectively. AllthecasesweresubjectedtoMRIplainscanandenhancementexamination.CharacteristicsofMRIofallcaseswerereviewedtogetherwith clinicopathologicchanges.Results AllSFT/HPClesionswerelocatedintheintracranialbutextra-cerebralspace.6werelocatedabovethe tentoriumofcerebellum,and2werelocatedbelowit.Lesionsof6patientswereacrossthetentoriumofcerebellumandspreadfrom supratentorialtosubtenorialspace.Amongalllesions,4wereroundinshape,10werelobulated,and3weresmallnodulesaroundthe edge.9ofthemexhibitednecrosisandcysticstructures.11lesionsshowedhypointensityand3casesshowedisointensityonT1WI.All thelesionswereheterogeneoushyperintensetyonT2WI,and5ofthemdisplayed"yin-yang"patternonT2WI.11casesexhibitededema.Signalof vascularvoidflow wasobservedin6cases.Thesolidpartsofthetumorsshowedsignificanthomogeneousenhancementon MRI. StrongpositiveSTAT6stainingwasobservedforthenuclearoftumorcells.Conclusion ItisdifficulttodifferentiateSFT/HPCfrom meningeoma.The"yin-yang"patternonT2WIisthecharacteristicofSFT/HPC.Inaddition,nuclearpositivestainingofSTAT6isalsospecific featureofSFT/HPCcell.

Objective? To retrieve and analyze the related researches on intensive care unit(ICU) physical environment from 2013 to 2017, and to provide reference for further improvement of the physical environment and quality of medical and nursing care in ICU. Methods? Relevant literature on ICU physical environment included from 2013 to 2017 by the China National Knowledge Infrastructure(CNKI), Wanfang Data Knowledge Service Platform, VIP Database for Chinese Technical Periodicals, Sinomed, Pubmed, Web of Science,and Scopus database were retrieved and analyzed bibliometrically on the internal and external features of the articles by Excel and CiteSpace software. Results? A total of 287 domestic and oversea papers on ICU physical environment were retrieved, the number of published articles showed an overall stable trend. The biggest number of published articles went to Guangdong Province, accounting for 14.1% of the articles in Chinese language. The United States had the biggest number of published papers in English language (43.8%). Literature journals mainly focused on infection control, critical medicine and critical care nursing journals, with the largest number of articles published by a single author(23.0%). The main types of research were clinical research (79.1%), fund-supported literature accounted for 26.5% of all the articles, and research directions were mainly about infection (40.0%), "nosocomial infection" and "pathogenic bacteria", "environment" and"object surface", "premature infant" and "growth and development", "sleep" and "noise" were closely related. Conclusions? The researches on ICU physical environment involves aspects including object surface, noise, light, air quality, temperature and ward design. Infection control in ICU environment and the influences of ICU physical environment on patient's biological rhythm and the growth and development of preterm infants are hot research issues in recent years. However, the geographical distribution of research is not balanced,and the overall quality of research evidence needs to be improved.

Objective To study the changes of gonadotropin releasing hormone agonist (GnRH-a) in pinopodes during luteal phase and to explore the possible mechanism of GnRH-a in luteal phase support of assisted reproductive technology (ART).Methods Totally 40 primary infertility women who were treated with ART due to male factors were enrolled,according to the order of the group they were randomly divided into experimental group and control group.On the 7th day after ovulation,the experimental group received a subcutaneous injection of 0.1 mg of GnRH-a,while the control group received a subcutaneous injection of placebo only (0.9％ salinc 2 ml),3 days later they came to the clinic again.Serum estradiol and progesterone levels were measured before and after treatment in each group.Pinopodes were collected for electron microscopic examination.Levels of ER and PR were detected by western blot.Results (1) There was no significant difference between the experimental group and the control group in the estrogen level before and after the treatment (all P＞0.05).The level of progesterone in the experimental group after treatment [(66.8± 14.9) nmol/L] was significantly higher than that before treatment(P＜0.05);also significantly higher than the same period of the control group (P＜0.05).(2) There was no significant difference in the expression of ER protein in the experimental group before and after treatment (P＞0.05).The expression of PR in the experimental group after treatment was significantly lower than that before treatment (P＜0.05);also lower than the same period of the control group (P＜0.05).(3) Expression amount of pinopodes in the experimental group after treatment was significantly higher than that before treatment [65％ (13/20) versus 25％ (5/20),P＜ 0.05],and the development trend was more mature [the percentage of maturation:75％ (15/20) versus 35％ (7/ 20),P＜0.05].Expression amount of pinopodes after treatment and the percentage of maturation in the experimental group were significantly higher than those in the same period of control group (P＜0.05).Conclusion GnRH-a in luteal phase support may play a role through the corpus luteum,which may promote the secretion of progesterone,downregulation of PR expression,promote the growth of pinopodes,and improve the endometrial receptivity.

Objective To discuss the diagnostic performance of MSCT in cystic nephroma(CN).Methods The MSCT findings of CN in 7 patients proved by operation and pathology were retrospectively reviewed.Results All of 7 cases were isolated cystic lesion, including 5 males and 2 females.MSCT scanning showed cystic tumor in the renal parenchyma with well-defined boundary.All cases showed regular cystic wall and septa with no attached cystic wall nodule.The cystic wall and septa appeared homogeneous enhancement on contrast-enhanced CT,while the cystic space showed inhomogeneous density and lack of enhancement.Conclusion The MSCT findings of CN may benefit the definite diagnosis.