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Cytisine derivatives are a group of alkaloids containing the structural core of cytisine, which are mainly distributed in Fabaceae plants with a wide range of pharmacological activities, such as resisting inflammation, tumors, and viruses, and affecting the central nervous system. At present, a total of 193 natural cytisine and its derivatives have been reported, all of which are derived from L-lysine. In this study, natural cytisine derivatives were classified into eight types, namely cytisine type, sparteine type, albine type, angustifoline type, camoensidine type, cytisine-like type, tsukushinamine type, and lupanacosmine type. This study reviewed the research progress on the structures, plant sources, biosynthesis, and pharmacological activities of alkaloids of various types.
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Alkaloids/chemistry , Quinolizines/pharmacology , Azocines/chemistry , FabaceaeABSTRACT
OBJECTIVE@#To evaluate the feasibility and tolerability of metoprolol standard dosing pathway (MSDP) in Chinese patients with acute coronary syndrome (ACS).@*METHODS@#In this multicenter, prospective, open label, single-arm and interventional study that was conducted from February 2018 to April 2019 in fifteen Chinese hospitals. A total of 998 hospitalized patients aged ≥ 18 years and diagnosed with ACS were included. The MSDP was applied to all eligible ACS patients based on the standard treatment recommended by international guidelines. The primary endpoint was the percentage of patients achieving the target dose at discharge (V2). The secondary endpoints included the heart rate and blood pressure at V2 and four weeks after discharge (V4), and percentage of patients experiencing bradycardia (heart rate < 50 beats/min), hypotension (blood pressure < 90/60 mmHg) and transient cardiac dysfunction at V2 and V4.@*RESULTS@#Of the 998 patients, 29.46% of patients achieved the target dose (≥ 95 mg/d) at V2. The total population was divided into two groups: target group (patients achieving the target dose at V2) and non-target group (patients not achieving the target dose at V2). There was significant difference in the reduction of heart rate from baseline to discharge in the two groups (-4.97 ± 11.90 beats/min vs. -2.70 ± 9.47 beats/min, P = 0.034). There was no significant difference in the proportion of bradycardia that occurred in the two groups at V2 (0 vs. 0, P = 1.000) and V4 (0.81% vs. 0.33%, P = 0.715). There was no significant difference in the proportion of hypotension between the two groups at V2 (0.004% vs. 0.004%, P = 1.000) and V4 (0 vs. 0.005%, P = 0.560). No transient cardiac dysfunction occurred in two groups during the study. A total of five adverse events (1.70%) and one serious adverse event (0.34%) were related to the pathway in target group.@*CONCLUSIONS@#In Chinese ACS patients, the feasibility and tolerability of the MSDP have been proved to be acceptable.
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Objective:To investigate the anatomy of rectus femoris muscle flap and the anterolateral thigh muscle flap and their clinical application in reconstruction of large soft tissue defects after the removal of oral malignant tumour.Methods:From December 2006 to June 2009, 8 specimens of Chinese adult cadavers fixed in 10% formaldehyde were dissected to perform anatomy of anterolateral thigh region at the School of Basic Medical Sciences, Kunming Medical University. Anatomical images were analysed using Image-Pro Plus 6.0. Then, a retrospective study was performed on 19 patients who had postoperative defects after oral malignant tumour surgery and the defects were reconstructed with the rectus femoris muscle flap and the anterolateral thigh muscle flap from March 2020 to July 2022 at the Department of Oral and Maxillofacial Surgery, Affiliated Stomatology Hospital, Kunming Medical University. The postoperative defects of the 19 patients ranged from 3.0 cm×6.0 cm to 5.0 cm×10.0 cm. Ten rectus femoris muscle flaps, 8 anterolateral thigh muscle flaps and 1 combined rectus femoris muscle flap and anterolateral thigh muscle flap were used. The muscular flaps sized from 4.0 cm × 8.0 cm to 6.0 cm × 11.0 cm. Regular postoperative outpatient follow-ups were conducted.Results:The lengths of vessels of the harvested rectus femoris muscle flap and anterolateral thigh muscle flap were 63.4 mm± 12.9 mm and 112.5 mm± 19.6 mm, respectively. The starting outer diameters of the lateral circumflex thigh artery, the oblique branch of the lateral circumflex thigh artery and the descending branch of the lateral circumflex thigh artery were 2.92 mm±0.72 mm, 1.88 mm±0.23 mm and 2.29 mm±0.43 mm, respectively. Postoperative follow-up lasted for 7 to 32 months, with 17.5 months in average. Seventeen flaps were completely survived and the rectus femoris muscle flap was completely mucosalised 5 weeks after surgery. However, 2 rectus femoris muscle flaps had necrosis of which one was changed to a tongue flap reconstruction and the other encountered flap necrosis during postoperative radiotherapy and healed after debridement and dressing changes. There was no postoperative complication in the donor sites. Other than the 2 patients, all other 17 patients had satisfactory clinical outcomes.Conclusion:Both of the starting outer diameters and length of vessels of the femoris muscle flap and the anterolateral thigh muscle flap meet the requirements for reconstruction of maxillofacial defects, and both muscular flaps are simple to prepare, in good reconstructive results with few complication, as well as an excellent outcome. They are feasible approaches for reconstruction of large soft tissue defects left after the removal of an oral malignant tumour.
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Objective:To investigate the relationship between change of serum D-dimer (D-D), soluble vascular cell adhesion molecule 1 (sVCAM-1), P-selectin and thrombosis after limb fracture surgery.Methods:289 patients with limb fractures who were treated in the emergency department of Shulan (Hangzhou) Hospital from January 2021 to January 2022 were selected as the study subjects. They were divided into deep vein thrombosis (DVT) group ( n=62) and non-DVT group ( n=227) according to whether DVT occurred after operation. The levels of prothrombin time (PT), activated partial prothrombin time (APTT), D-D, sVCAM-1 and P-selectin were measured before and after operation in all patients. The levels of PT, APTT, D-D, sVCAM-1 and P-selectin were compared between DVT group and non-DVT group. Logistic sequential stepwise regression analysis was used to analyze the risk factors of postoperative thrombosis in patients with limb fractures. Results:There was no statistically significant difference in PT and APTT between 289 patients with limb fractures after operation and before operation (all P>0.05), while the levels of serum D-D, sVCAM-1 and P-selectin after operation were higher than that before operation (all P<0.05). There was no significant difference in general data between DVT group and non-DVT group (all P>0.05); There was no statistically significant difference in PT and APTT before and after operation between DVT group and non-DVT group (all P>0.05). The levels of serum D-D, sVCAM-1 and P-selectin before and after operation in DVT group were higher than those in non-DVT group (all P<0.05). Logistic sequential stepwise regression analysis showed that high levels of D-D, sVCAM-1 and P-selectin were risk factors for thrombosis after limb fracture surgery (all P<0.05). Conclusions:High levels of D-D, sVCAM-1 and P-selectin are risk factors for thrombosis after limb fracture surgery.
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Diabetic wound healing disorder，one of the common chronic complications of diabetes，seriously influences the quality of life of patients and even causes disability and death，bringing a heavy burden to the society. Chinese medicine，a unique and precious resource in China，is safe with definite effect. Oxidative stress plays an important role in the occurrence and development of diabetic wound and the disturbance of antioxidant defense mechanism is among the causes of the lingering diabetic wound. As a vital transcription factor for intracellular redox homeostasis，nuclear factor erythroid 2-related factor 2 （Nrf2） regulates oxidative/heterogenous stress and reduces inflammatory responses. Although it is unnecessary for common wound healing，it is of great importance for diabetic wound healing. Many Chinese medicinals and the active ingredients have been found to enhance diabetic wound healing by mechanisms related to activation of the Nrf2 signaling pathway. Targeted activation of Nrf2 by Chinese medicine can alleviate oxidative stress，inflammatory response，and apoptosis in diabetic wound，thereby delaying further exacerbation of symptoms. Therefore，Nrf2 is regarded as a potential target for drugs to boost diabetic wound healing. This study summarizes the relationship between the Nrf2 signaling pathway and diabetic wound and analyzes the mode of action and possible mechanisms of Chinese medicine and its active ingredients in promoting diabetic wound healing through modulating the Nrf2 pathway，which is expected to serve as a reference for developing drugs for diabetic wound based on this pathway.
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Objective To understand the quality of life in patients with pulmonary tuberculosis within three years after treatment， determine its related factors， and make suggestions for improving the short-term quality of life in patients with pulmonary tuberculosis after treatment. Methods A telephone survey was used to investigate registered tuberculosis patients in Shanghai in 2018 using the short form 12 （SF-12） and the chronic obstructive pulmonary disease （COPD） assessment test questionnaire （CAT）. Results A total of 975 patients with pulmonary tuberculosis who had completed the treatment were included in the study. The total physiological score was determined to be 49.18±10.25， and the total psychological score was 50.27±8.03 （t=5.62，P<0.000 1）. The average CAT score was 13.31±6.08. Multivariate linear regression analysis showed that quality of life was positively associated with high educational level， high monthly family income， and frequent physical exercise， whereas negatively associated with comorbidities， low self-care ability， and changing jobs. Conclusion We should pay more attention to the patients with pulmonary tuberculosis coexistent with COPD and other lung diseases. Measures should be implemented for the improvement in the quality of life， including providing financial support， encouraging regular exercise， and improving lung function.
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Objective:To systematically evaluate the effect of personalized health education on adherence to osteoporosis therapy.Methods:Literatures related to the effect of health education intervention on the medication adherence of patients with osteoporosis with the core contents of guiding standardized medication, long-term medication, adherence to medication, and improvement of patients' health knowledge of diseases published from 1990 to 2021 were searched in the Chinese national knowledge infrastructure (CNKI), Wanfang data database, Chinese biomedical literature database (CBM), Medline, Embase, web of science, The Cochrane Library databases and combining with the related journals of handicrafts. Review manage 5.3 was used to evaluate the heterogeneity of included literature. Fixed effect model or random effect model was used to combine the effect quantity. Stata 16.0 was used to evaluate the publication bias, sensitivity analysis, subgroup analysis and meta regression.Results:A total of 1 924 articles were retrieved and finally 24 randomized-controlled trial (RCTs) were included, including 11 248 patients with osteoporosis in which 5 742 cases with health education, and 5 506 cases with regular care. The literature evaluation showed that the included studies had high heterogeneity, and studies with subjects aged 50-60 [ RR (95% CI)=0.475 (0.344, 0.657), P=0.460], located in North America [ RR (95% CI)=0.888 (0.831, 0.948), P=0.232] and with a sample size greater than 500 [ RR (95% CI)=0.761 (0.660, 0.878), P=0.152] had high homogeneity. Meta-analysis showed that compared with the regular care group, the health education group had higher medication adherence [ RR(95% CI)=0.78(0.69, 0.87), P<0.001]. Conclusion:Osteoporosis patients need to have medication therapy for a long period of time. Individualized health education guidance with long-term medication, standardized medication and adherence to medication can enhance patients' awareness of osteoporosis and can improve adherence to osteoporosis therapy, thus have important effect on the treatment of osteoporosis
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Objective:To analyze the role of patient support system in multidrug-resistant/rifampicin-resistant tuberculosis (MDR/RR-TB) treatment adherence and outcome, and provide evidence for deepening the patient-centered support system.Methods:Based on the stakeholder analysis, definite stakeholders (administrators from the Department of Medical Insurance, and those from the provincial CDC), expectant stakeholders (administrators from regional CDC, health workers from primary CDCs, medical workers from designated MDR/RR-TB hospitals and MDR/RR-TB patients), and latent stakeholders (MDR/RR-TB patient families and their neighbors or colleagues) were selected using a purposive sampling. These stakeholders were subject to a semi-structured interview on patient support. The inclusion of participants ceased after reaching code or thematic saturation and meaning saturation, while thematic framework analysis was applied in interview data.Results:The 25 interviewees included could be categorized into three groups of stakeholders, i. e., 4 definite stakeholders, 19 expectant stakeholders and 2 latent stakeholders. Three themes summarized in this regard were definite stakeholders providing policy support to advance these patients′ access to standardized diagnosis and treatment services; diagnosis and treatment and management support of expectant stakeholders of these patients to encourage their compliance to treatment and enable their access to high quality medical care; and support from latent stakeholders as a critical guarantee for the patients to welcome a desirable treatment outcome. Psychological support provided under MDR/RR-TB basic care program in some provinces contributed positively to raising patients′ compliance. Economic support, treatment support from family menmbers ccould help the patients to welcome desirable outcomes.Conclusions:MDR/RR-TB patient-centered support system operating in the Yangtze River delta provide the patients with MDR/RR-TB diagnostic and treatment services of some accessibility. Given the progress, there are still shortcomings for the respective stakeholders to enhance their attention and collaboration to improve the access and equity to medical service.
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Objective To determine the epidemiological characteristics of measles in Jinshan District of Shanghai in 2002‒2019. Methods This study was a secondary data analysis using measles surveillance system and epidemiological case reports in Jinshan District from January 2002 through December 2019. Temporal characteristics of the disease onset was determined by a concentration method. ArcGis 10.4 and SaTScan 9.6 software were used for spatiotemporal clustering scanning analysis. Results From 2002 through 2019， a total of 289 cases of measles were reported in Jinshan District， Shanghai， with an average annual incidence of 2.24 per 105. The annual incidence fluctuated from 0 to 11.27 per 105. The highest incidence was recorded in 2005 （11.27 per 105）， followed by a decline； however， there was a rebound in 2008 （5.26 per 105） and 2015 （3.30 per 105）. The concentration M was determined to be 0.62， showing a clear seasonality concentrated in February to May. The spatiotemporal scan found that spatial and temporal clusters were more likely to occur in the areas with higher population density such as Zhujing Town and Shihua Community and between 2002 and 2009. The majority of measles cases were infants and children under the age of 1 and adults aged 20‒39 years. Approximately 66.78% of measles cases reported unknown vaccination history. In addition， in children aged 8 month to 4 years， the vaccination rate of measles-containing vaccines in the domestic migrants was much lower than the local residents （χ2=4.44， P=0.035）. Conclusion The incidence of measles in Jinshan District presents a downward trend. The population affected has a "two-way displacement"， which is concentrated in the more populated areas. It is necessary to strengthen measles surveillance and implement supplementary immunization programs with measles-containing vaccines for at-risk populations.
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Objective:To investigate the risk and temporal characteristics of tuberculosis (TB) in persons with latent tuberculosis infection (LTBI) among close contacts of TB patients in Shanghai.Methods:This was a prospective observational study, the study subjects were the close contacts of TB patients who tested positive for Mycobacterium tuberculosis infection by T-cell spot test of tuberculosis infection (T-SPOT.TB) among the registered population in seven districts of Shanghai from 2009 to 2010. Questionnaire interview was applied to investigate the basic information and contact history.The LTBI cases during nine consecutive years were identified through the TB registration and reporting system.Cumulative incidence rate and incidence density of TB in tuberculosis-infected close contacts were estimated. Chi-square test was used to compare the incidence rate among different sex and age groups. Results:In total, 376 pulmonary TB patients and their 982 close contacts were enrolled in the baseline survey from 2009 to 2010. Of them, 287 close contacts were tested positive for Mycobacterium tuberculosis infection by T-SPOT.TB.The median age of persons with LTBI at the time of survey was 54 years old, and 36.6%(105/287) were male. By the end of December 2019, five new TB cases were detected among the 287 close contacts with LTBI. The two-year and five-year cumulative incidence were 0.35% and 1.05%, respectively. The nine-year cumulative incidence was 1.74%(95% confidence interval 0.57%-4.02%). The median duration for TB occurrence was 3.67 years. The incidence density of TB was 1.875/1 000 person-years with a total of 2 666.75 observation person-years. There were no significant differences in the nine-year cumulative incidence of TB among close contacts with LTBI by age and sex ( χ2=0.600, <0.001, respectively, both P>0.05). Conclusion:The occurrence of TB among close contacts with LTBI is concentrated in the first five years after TB contact.
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To systematically review the efficacy of Xuebijing Injection combined with western medicine in the treatment of systemic inflammatory response syndrome(SIRS). In this study, CBM, CNKI, Wanfang, VIP, PubMed and EMbase databases were retrieved for clinical randomized controlled trials on the effect of Xuebijing Injection combined with western medicine in the treatment of SIRS from the establishment of the database to July 31, 2020. After screening, Meta-analysis was conducted by RevMan 5.3 software, trial sequential analysis was conducted by TSA 0.9.5.10 beta software, and the evidence quality level was evaluated by GRADEprofiler 3.6.1 software. Meta-analysis showed that Xuebijing Injection combined with western medicine could reduce white blood cell count(MD=-2.32, 95%CI[-2.44,-2.21], P<0.000 01), C-reactive protein count(MD=-22.70, 95%CI[-29.61,-15.79], P<0.000 01), APACHE Ⅱ score(MD=-2.15, 95%CI[-2.43,-1.87], P<0.000 01), tumor necrosis factor alpha count(SMD=-1.23, 95%CI[-1.48,-0.99], P<0.000 01) and interleukin-6 count(SMD=-0.92, 95%CI[-1.15,-0.69], P<0.000 01), improve treatment efficiency(RR=1.39, 95%CI[1.23, 1.56], P<0.000 01), reduce incidence of multiple organ dysfunction(RR=0.47, 95%CI[0.35, 0.64], P<0.000 01) and mortality(RR=0.22, 95%CI[0.13, 0.37], P<0.000 01), which were better than western medicine treatment alone. Trial sequential analysis showed that in terms of reducing the incidence of multiple organ dysfunction and C-reactive protein count, the cumulative Z value passed through the traditional threshold, TSA threshold and expected information value, and reached the required number of cases. GRADE evaluation showed that the level of evidence was low or very low. According to the findings, Xuebijing Injection combined with western medicine is effective in treating SIRS. However, as the low quality of the included studies may affect the reliability of the conclusion, more high-quality studies shall be included for further verification in the future, so as to provide better suggestions for clinical medication.
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Humans , Drugs, Chinese Herbal , Injections , Randomized Controlled Trials as Topic , Reproducibility of Results , Systemic Inflammatory Response Syndrome/drug therapyABSTRACT
Objective:To know the status of knowledge， attitude and behavior related to sexual and reproductive health in high school students in three regions of East China， and to provide a reference for adolescent reproductive health education. Methods:From August to October in 2019， 614 high school students were invited from 6 high schools in Jiading District and Yangpu District， Shanghai and Taicang City， Jiangsu to participate in this study. We conducted an anonymous self-questionnaire survey using structured questionnaires based on adolescent reproductive health knowledge and literacy. Results:The male to female ratio of all high school students in the study was 1∶1.25， and the average age was （16.1±0.9） years old. The score of pregnancy and abortion knowledge was the lowest among the reproductive health knowledge scores， and the differences among the three regions were statistically significant （P=0.002）. Male high school students （P<0.001）， students in the school with reproductive health education base （P=0.008） and students who wanted to obtain reproductive health education （P=0.002） were more acceptable to premarital sex. The high school students obtained adolescent health knowledge mainly through the internet or mobile phones， and had a demand for reproductive health related services. Conclusion:High school students in the three regions have a more open attitude towards premarital sex， but have a poor grasp of correct and efficient contraceptive knowledge. Responsible departments need to strengthen the health education of relevant knowledge， and at the same time to find new forms of education to effectively improve the level of adolescent reproductive health.
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OBJECTIVE@#To analyze the clinical and genetic features of three patient diagnosed with Kleefstra syndrome.@*METHODS@#Whole exome sequencing (WES) was carried out for the probands and their parents. Suspected variants were validated by Sanger sequencing. Copy number variations (CNV) were detected by CNV-seq and validated by real-time PCR.@*RESULTS@#Proband 1 was found to carry a de novo heterogeneous variant (c.823+1G>T) of the EHMT1 gene, which may affect its expression. Based on the guidelines of the American College of Medical Genetics and Genomics, the variant was predicted to be pathogenic (PVS1+PS2+PM2). Proband 2 was found to carry a de novo missense variant c.439C>G (p.L147V) of the EHMT1 gene, which was predicted to be likely pathogenic (PS2+PM1+PM2+PP3). Proband 3 was found to carry a heterozygous 520 kb deletion at 9q34.3 by CNV-seq. The deletion has encompassed the whole of the EHMT1 gene. Real-time PCR has detected no CNV of this region in her parents.@*CONCLUSION@#Variants of the EHMT1 gene probably underlay the disease in these patients. Genetic testing has provided a basis for their clinical diagnosis.
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Female , Humans , Chromosome Deletion , Chromosomes, Human, Pair 9 , Craniofacial Abnormalities , DNA Copy Number Variations , Genetic Testing , Heart Defects, Congenital , Intellectual Disability/genetics , MutationABSTRACT
Objective:To identify problems and demands for antenatal care (ANC) among pregnant women in different trimesters of pregnancy in Shanghai for optimizing ANC service during the epidemic of COVID-19.Method:s Organized by maternal and child health care institute in the 16 districts of Shanghai, a cross sectional study was conducted among pregnant women who came to pregnancy registration in the community health centers or attended ANC in midwifery hospitals from February 7 to February 12, 2020. Consented participating women completed a semi-structured online questionnaire voluntarily. Data was analyzed using frequency,chi-square test and scoring.Result:s A total of 2 002 valid questionnaires were collected from 183 community health centers and 67 midwifery hospitals. About 94.6%(1 894/2 002) of the pregnant women worried about being infected during the COVID-19 epidemic, and 14.7% (294/2 002) demanded for psychological consultation. Time-lapse appointments for ANC were requested by 87.7% (1 756/2 002) of the participants for avoiding presenting themselves in people-density places. Compared with other pregnancy trimesters, pregnant women in the second trimester were more willing to reduce the frequency of ANC (35.2% versus 39.5% versus 48.1%, P<0.01). Compared with multiparas, primiparas were more willing to have online consultation and guidance (49.2% versus 63.8%, P<0.01). Regarding the needs for health knowledge on COVID-19, personal protection against 2019 novel coronavirus (2019-nCoV) was the most concerned for pregnant women, and 71.0% (1 421/2 002) of them preferred to obtain knowledge through health applications, official Weibo and WeChat. Conclusions:Pregnant women in Shanghai critically concern about the risk of 2019-nCoV infections, and highly demand knowledge and measures on prevention and protection from COVID-19. They ask for having time-lapse appointments for ANC and online access to health information and services. Maternal and child care institutes should understand the demands of pregnant women, optimize the means of ANC service, and provide tailored and accessible health education and service for the safety of mother and child.
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Objective@#To identify problems and demands for antenatal care (ANC) among pregnant women in different trimesters of pregnancy in Shanghai for optimizing ANC service during the epidemic of coronavirus disease 2019 (COVID-19).@*Methods@#Organized by Maternal and Child Health Care institute in the 16 districts of Shanghai, a cross sectional study was conducted among pregnant women who came to pregnancy registration in the community health centers or attended ANC in maternity hospitals from February 7 to February 12, 2020. Consented participating women completed a semi-structured online questionnaire voluntarily. Data was analyzed using frequency and scoring, chi-square test.@*Results@#A total of 2 002 valid questionnaires were collected from 183 community health centers and 67 midwifery hospitals. About 94.6% of the pregnant women worried about being infected during the COVID-19 epidemic, and 14.7% demanded for psychological consultation. Appointment ANC services were requested by 87.7% of the participants for avoiding presenting themselves in people-density places. Compared with other pregnancy trimesters, pregnant women in the second trimester were more willing to reduce the frequency of ANC (48.1% VS. 39.5% VS. 35.2%, P<0.01). Compared with multiparas, primiparas were more willing to have online consultation and guidance (63.8% VS. 49.2%, P<0.01). Regarding the needs for health knowledge on COVID-19, personal protection against 2019-nCoV was the most concerned for pregnant women, and 71.0% of them preferred to obtain knowledge through health applications, official Weibo and WeChat.@*Conclusions@#Pregnant women in Shanghai critically concern about the risk of 2019-nCoV infections, and highly demand knowledge and measures on prevention and protection from COVID-19. They ask for having time-lapse appointments for ANC and online access to health information and services. Maternal and Child Care institutes should understand the demands of pregnant women, optimize the means of ANC service, and provide tailored and accessible health education and service for the safety of mother and child.
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OBJECTIVE@#To delineate the clinical feature and genetic basis of four patients with congenital neutropenia.@*METHODS@#All patients were subjected to whole exome sequencing (WES). Suspected variants were verified by Sanger sequencing.@*RESULTS@#The patients (two boys and two girls), aged 7 to 15 months, suffered from neutropenia and recurrent infections. Bone marrow smears showed a significant decrease in the proportion of rod-shaped and lobulated granulocytes, which suggested impaired development and maturation of bone marrow neutrophils. WES has discovered heterozygous variants (c.496G>A, c.58C>G, c.391G>A and IVS1+5T>A) of the ELANE gene in the patients. Among these, c.58C>G and IVS1+5T>A were unreported previously. Follow up revealed patients 1 and 3 had periodic neutropenia, while patients 2 and 4 had severe congenital neutropenia. After attaining the definite diagnosis, the patients were treated symptomatically.@*CONCLUSION@#The main clinical feature of congenital neutropenia is refractory recurrent bacterial infections, for which mutations of the ELANE gene are a common cause. Two novel pathogenic ELANE variants have been discovered in this study.
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Female , Humans , Infant , Male , Congenital Bone Marrow Failure Syndromes/genetics , Genetic Testing , Leukocyte Elastase/genetics , Mutation , Neutropenia/geneticsABSTRACT
Objective To compare the clinical features of diffuse capillary endothelial proliferative Henoch-Schonlein purpura nephritis ( DEP-HSPN ) with or without crescent formation. Methods The clinical data of 110 children with DEP-HSPN admitted to Dalian Central Hospital and the First Affiliated Hospital of Dalian Medical University from January 2008 to January 2018 were analyzed retrospectively. Among them,24 cases were divided into non crescentic group and 86 cases into crescentic group. The clinical characteristics and prognosis of the two groups were statistically analyzed. Results The clinical manifestations of children without crescent body formation group were all type III. There were 35 cases of type III and 51 cases of type V in the crescent body formation group. Compared with non-crescent formation group,the proportion of gross hematuria(83. 7%(72／86) vs. 29. 2%(7／24),χ2＝10. 396),urine red blood cell count ((112. 4±20. 3)／HP vs. (45. 2±10. 6)／HP,t＝9. 697),24 h urine protein ((2471. 6 ±242. 0) mg／d vs. (1358. 5±109. 3) mg／d,t＝6. 372) and serum creatinine (( 44. 9 ± 9. 6) μmol／L vs. (32. 3±5. 2) μmol／L,t＝5. 390) increased significantly,the serum albumin (( 22. 8±3. 8) g／L vs. ( 35. 1 ±5. 7) g／L,t＝4. 806)decreased significantly (all P＜0. 05). Both groups had non-simple IgA deposits in the mesangial region. The proportion of complete remission and asymptomatic hematuria was 70. 8%( 17／24) and 29. 2%( 7／24) in the non-crescent group, 58. 1%( 50／86) and 41. 9%( 36／86) in the crescent group,respectively,with no significant difference ( χ2＝1. 330,1. 196, all P＞0. 05) . Conclusion When DEP-HSPN is accompanied by crescent formation, gross hematuria, urinary erythrocyte count and the proportion of massive proteinuria increase significantly. Combined immunosuppressive therapy in acute stage and long-term sequential treatment in remission stage can achieve good prognosis.
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Objective To explore the short-term hemodynamic change of fluid challenge (FC) with crystalloid or colloid and define fluid responsiveness at the optimal time in patients with septic shock. Methods A prospective observational study was conducted. Septic shock patients monitored with pulmonary catheters admitted to medical intensive care unit (ICU) of the Peking Union Medical College Hospital from July 2016 to December 2018 were enrolled. All included patients received FC and were divided into two groups according to the type of fluid used, i.e. crystalloid group (normal saline for 500 mL) and colloid group (4% succinyl gelatin for 500 mL). The choice of fluid type was decided by the attending physician. Hemodynamic variables were measured at baseline, and 0 (immediately), 10, 30, 45, 60, 90, 120 minutes after FC, included cardiac index (CI), heart rate (HR), mean artery pressure (MAP), central venous pressure (CVP) and pulmonary arterial wedge pressure (PAWP). Fluid responsiveness was defined as CI increased by more than 10% after FC. The data were analyzed by repeated measurements of variance between the two groups as well as responders and nonresponders. Results Forty patients were included, 20 cases each in colloid group and crystalloid group; of whom 26 were fluid responders with 12 of colloid group and 14 of crystalloid group. Of the 14 nonresponders, 8 were of colloid group and 6 of crystalloid group. ① Compared with before FC, CI (mL·s-1·m-2) was significantly increased in crystalloid and colloid groups after FC (71.7±16.7 vs. 65.0±16.7, 68.3±25.0 vs. 63.3±23.3, both P < 0.05). In the colloid group, volume expansion increased the CI to maximum (76.7±18.3) at 30 minutes after FC, at 120 minutes after FC, a significantly higher CI (70.0±16.7) was also observed (P < 0.05), an increased in CI≥10% was observed at 60 minutes after FC. In the crystalloid group, CI was increased to maximum at 10 minutes (73.3±28.3) and decreased to baseline at 60 minutes, an increased in CI≥10% was also observed at 10 minutes after FC. In addition, there was no significant difference in CI changes between colloidal group and crystalloid group at different time points after FC. ② CI did not change over time in nonresponders groups, whereas in responders CI increased parallelly to that in both crystalloid and colloid groups over time. However, an increased in CI≥10% was observed through the 120 minutes after FC in responders of colloid group compared with that of at 30 minutes after FC in crystalloid group. There was significant difference in CI changes between colloidal group and crystalloid group at 30, 45, 60, 90 minutes after FC (mL·s-1·m-2: 18.3±3.3 vs. 8.3±1.7, 18.3±3.3 vs. 5.0±1.7, 13.3±1.7 vs. 3.3±1.7, 11.7±3.3 vs. 3.3±1.7, all P <0.05). ③ The maximal values of CVP and PAWP were observed at the end of FC. In colloid group, both the two variables were notably higher than that before FC over 120 minutes compared with that of only at 10 minutes in crystalloid group. The MAP in colloid increased to maximum immediately at the end of FC and decreased to baseline at 45 minutes, however, the MAP in crystalloid group and HR of both groups showed no differences over 120 minutes. Conclusions Hemodynamic changes were significantly different between crystalloid and colloid after FC in patients with septic shock. Therefore, the timing of fluid responsiveness assessment should be different individually. The assessment time of colloid group may be prolonged to 30 minutes after FC while that of crystal group can be at 10 minute after FC.
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Objective To investigate the relationship between body mass index (BMI) and cardiac morphological changes and clinical prognosis in patients with hypertrophic cardiomyopathy (HCM). Methods Clinical and followed up data of patients with HCM hospitalized in DrumTower Clinical Medical College of Nanjing Medical University from January 2010 to December 2015 were retrospectively analyzed. Major cardio-cerebral vascular adverse events (MACCE), including cardiac death, exacerbation of re-admission and stroke) were set as primary endpoints. Patients were divided into normal body group [body mass index (BMI)<24 kg/m2], pre-obesity group (24 kg/m2≤BMI<28 kg/m2), and obese group (BMI≥28 kg/m2). Baseline characteristics of each group and the occurrence of MACCE were compared. Results All of 206 patients were followed up for (50.4 ± 18.6) months. Twenty-nine patients (14.1% ) died during follow-up, and cardiac death occured in 17 patients (8.2% ) including 4 patients with cardiac arrrest,11 patients with heart failure. With the increase of BMI, there was no significant difference in left ventricular mass index (LVMI), but left atrium dimension (LAD), left ventricular end diastolic dimension(LVDd) and left ventricular mass (LVM) showed a significant upward trend.Single risk factor analysis indicated that coronary heart disease, NYHA ≥Ⅲ, chronic heart failure duration > 1 year, atrial fibrillation, BMI, LAD, left ventricular ejection fraction (LVEF), LVM, LVMI were correlated with MACCE. Multifactor analysis showed that BMI ( OR=1.335, P=0.006) and NYHA ≥ Ⅲ ( OR=3.513, P=0.046) were independent risk factors for MACCE in HCM patients. Conclusions BMI is significantly associated with MACCE in HCM patients. Weight management is particularly important for patients with HCM.
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Objective To analyze the factors associated with syphilis treatment compliance and adverse pregnant outcomes among pregnant women with syphilis in Shanghai.Methods The prospective cohort was established based on maternal syphilis monitoring system of Shanghai,which included all the pregnant women diagnosed with syphilis during Jan 2013 to Dec 2015.A total of 1 717 pregnant women with syphilis were recruited at the baseline,and 1 147 of them were followed up during treatment and their pregnancy,and the delivery outcomes were recorded.The information of testing/treatment of pregnant women with syphilis and health outcomes of infants were collected.Chi-square test was used for univariate analysis and logistic regression model was used to identify the factors associated with syphilis treatment and adverse pregnant outcomes.Results A total of 685 participants received syphilis treatment during pregnancy,with the treatment rate of 59.7％.Among them,397 (34.6％) patients underwent two courses of complete treatment.The poor educated,unemployed/job-waiting or multipara population had poor compliance to treatment.Only 34.9％ (142/407) of cases diagnosed at last trimester received syphilis treatment and 10.1％ (41/407) completed the treatment.The proportion of non-treponemal conversion were higher in subjects who received syphilis treatment during pregnancy than those who did not (39.1％ [268/285] vs 3.7％ [17/462]).Complete syphilis treatment during pregnancy was protective factor to decrease neonatal death adjusted relative risk ([aRR] =0.05,95％ CI:0.01-0.37,P =0.003) and prematurity/low birth weight (aRR =0.44,95％ CI:0.27-0.70,P =0.001).Strong positive non-treponemal result before delivery increased the risks of neonatal death (aRR =12.89,95％ CI:1.70-100.43,P =0.014) and prematurity/low birth weight (aRR =12.78,95％ CI:152-5.06,P =0.001).Conclusions Factors such as educational level,employment status,and maternal history will affect the compliance of syphilis treatment during pregnancy.Early diagnosis and complete treatment course of syphilis could improve the pregnant outcomes and the health status of infants.