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Marfan syndrome(MFS) is an autosomal dominant connective tissue disease caused by genetic mutation, and its pathogenesis has not been fully clarified. Due to the complex and unpredictable clinical phenotype of MFS, the diagnosis of preclinical or atypical patients becomes difficult, and it is easy to misdiagnose, which delaying the diagnosis and treatment. Therefore, it is of great significance to clarify the mechanism of MFS phenotypic variation for the diagnosis, phenotypic prediction and prognosis evaluation of MFS patients. Here, we review the mechanism and research progress of the clinical phenotypic variation of MFS, with the aim of guiding clinical diagnosis and treatment.
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Tetralogy of Fallot with unilateral absence of pulmonary artery is a rare congenital malformation. There is a lack of unified treatment strategy with controversy exist, and surgeons are confused in the process of clinical decision-making. This review discusses the clinical classification and treatment of Tetralogy of Fallot with unilateral absence of pulmonary artery, with the aim of providing reference for the clinical decision-making for cardiovascular surgeons.
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To explore the composition and diversity of the intestinal microflora of Leopoldamys edwardsi in Hainan Island. In November 2019, DNA was extracted from fecal samples of 25 adult Leopoldamys edwardsi (14 males and 11 females) in Hainan Island at the Joint Laboratory of tropical infectious diseases of Hainan Medical College and Hong Kong University. Based on the IonS5TMXL sequencing platform, single-end sequencing (Single-End) was used to construct a small fragment library for single-end sequencing. Based on Reads shear filtration and OTUs clustering. The species annotation and abundance analysis of OTUs were carried out by using mothur method and SSUrRNA database, and further conducted α diversity and β diversity analysis. A total of 1481842 high quality sequences, belonging to 14 Phyla, 85 families and 186 Genera, were obtained from 25 intestinal excrement samples of Leopoldamys edwardsi. At the level of phyla classification, the main core biota of the Leopoldamys edwardsi contained Firmicutes (46.04%),Bacteroidetes (25.34%), Proteobacteria (17.09%), Tenericutes (7.38%) and Actinobacteria (1.67%), these five phyla account for 97.52% of all phyla. The ratio of Helicobacter which occupied the largest proportion at the genus level was 12.44%, followed by Lactobacillus (11.39%), Clostridium (6.19%),Mycoplasma (4.23%) and Flavonifractor (3.52%). High throughput sequencing analysis showed that the intestinal flora of Leopoldamys edwardsi in Hainan Island was complex and diverse, which had the significance of further research.
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Adult , Animals , Female , Humans , Male , Bacteria/genetics , Feces/microbiology , Gastrointestinal Microbiome/genetics , High-Throughput Nucleotide Sequencing , Intestines , Murinae/geneticsABSTRACT
Hyperoxia is very common in critical patients, which causes varying degrees of pathophysiological damage. Hyperoxia is one of the main reasons for increasing mortality, prolonged hospital stay and poor prognosis of critical patients. Therefore, it is critical to carry out the standardized oxygen therapy. In this paper, we discussed the harmful effects of hyperoxemia to critically ill patients by reviewing the effect of hyperoxemia on cardiovascular and cerebrovascular, hyperoxia-induced lung injury, and the association of hyperoxemia with ventilator-associated pneumonia (VAP) and acute renal kidney injury (AKI). Meanwhile, we provided some management suggestions about standardized clinical oxygen therapy, oxygen therapy related research and clinical management of oxygen therapy, aimed at providing reference and research direction for standardizing clinical oxygenation process, reducing the occurrence of hyperoxemia, and making reasonable method for hyperoxemia prevention.
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Objective To summarize the treatment decision-making strategy and its long-term efficacy for advanced elderly patients with severe valvular heart disease and clear indications for surgery.Methods Clinical data of 196 patients aged 75 years and older firmly diagnosed as severe valvular heart diseases were retrospectively analyzed.The patients were divided into the surgical group (a mean age of 77.4±2.0 years,n=126)and the conservative group(a mean age of 80.5±5.0 years,n =70).Factors affecting therapeutic decision-making were analyzed,and the differences in a long-term survival were compared between the two groups.Results The most common reason for choosing conservative treatment was the recommendation of the doctor giving a preliminary diagnosis and worrying about the high-risk surgery for the patients(62.9%,44/70).Only 26(37.1%)patients in the conservative group were evaluated by cardiac surgeons,among whom 12 (17.1%)patients were considered to have surgical contraindications,and 14 (20.0%) patients themselves or their family members chose conservative treatment for the fear of surgical risks.Patients in the operation group were mainly from the outpatient department of cardiac surgery,and only 8 (6.3 %)cases were referred from department of internal medicine.Logistic regression analysis showed that female,chronic renal insufficiency,advanced age,pneumonia and emergency hospital admissions were independent predictors for the conservative option(P <0.01),while patients with isolated aortic valve disease tended to receive surgical treatment.Overall 5-year survival was higher in the surgical group than in the conservative group (76.4% vs.39.9%,P < 0.01).Cox regression analysis disclosed that the conservative treatment option was the single risk factor for long-term survival in all series.Conclusions Many factors affect the process of therapeutic decision-making for patients with severe valvular heart diseases,and a multidisciplinary collaboration is the best way for the optimal treatment strategy for those patients.
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Objective To summarize the characteristics of acupoints selection in acupuncture-moxibustion treatment of primary dysmenorrhea (PD) based on data mining. Method Clinical literatures published in the recent 10 years related to acupuncture-moxibustion treatment of PD were collected to establish a prescription database by using Excel. The descriptive analysis, association rules analysis and clustering analysis were conducted by Python and Clementine12.0. Result A total of 74 acupoints were involved with a total frequency of 1072. The leading three meridians were Spleen Meridian (325 times), Conception Vessel (260 times) and Bladder Meridian (158 times); the leading acupoints were Sanyinjiao (SP6, 174 times), Guanyuan (CV4, 111 times), Diji (SP8, 79 times), Ciliao (BL32, 73 times) and Qihai (CV6, 67 times). The utilization of the specific acupoints accounted for 66.22% (49/74). The crossing acupoints were predominant (393 times), especially Sanyinjiao, Guanyuan and Zhongji (CV3), followed by the five-Shu points (195 times), Front-Mu points (195 times) and Yuan-Primary points (99 times). The association rules analysis showed that Guanyuan and Sanyinjiao had the most significant correlation. The clustering analysis figured out 8 core clustering groups. Conclusion The characteristics of acupoints selection revealed in this study provide ideas and references for acupuncture-moxibustion treatment of PD in clinic.
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Objective To investigate the clinical characteristics,surgical experience and mid-term outcome of coronary artery bypass graft surgery in patients aged ≤ 40 years.Methods From Jan.,2009 to Dec.,2015,12 patients with median age of (37.9 ± 2.5) years (range 32-40 years,10 males) were diagnosed with coronary artery disease and underwent coronary artery bypass graft surgery at Zhongshan Hospital,Fudan University.The clinical characteristics included five cases with hypertension,three cases with diabetes mellitus,four cases with hyperlipemia,eight cases with smoking history,seven cases with myocardial infarction.Six cases had at least one branch totally occluded.Two cases once underwent percutaneous coronary intervention surgery and implanted with five and three stents respectively.The perioperative data and follow-up results were retrospectively analysed.Results There was no in-hospital death and no death during follow-up period.Surgical techniques included bilateral internal mammary arteries combined with radial artery were operated in six cases,bilateral internal mammary arteries combined with great saphenous vein in two cases,left internal mammary arteries combined with great saphenous vein in four cases,off-pump in nine cases and on-pump in three cases.The mean follow-up time was (47.8 ± 24.3) months.During the follow-up one case suffered with saphenous vein graft restenosis 5 years after the surgery and underwent percutaneous coronary intervention.Other cases were all alive and assessed New York Heart Association's function class Ⅰ-Ⅱ.Conclusions Younger patients usually suffer with severe coronary artery disease when diagnosed because they often have excellent compensative capacity and the pathogenesis is concealed.The mid-term results of coronary artery bypass graft surgery in patients aged ≤40 years are satisfactory.
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Objective To investigate the value of CT guided 125 I seed implantation combined with chemotherapy in the treat-ment of unresectable non-small cell lung cancer .Methods The related data of 42 cases of middle and advanced non-small cell lung cancer treated in this hospital from January 2012 to December 2016 were retrospectively analyzed .The group A (23 cases) received the 125I seed implantation combined with chemotherapy ,while the group B(19 cases) adopted the simple chemotherapy .All cases conducted the chest CT re-examination at 1 ,2 ,6 months after treatment .The curative effects and complications were compared be-tween the two groups .Results The total effect rate (RR) at 1 ,2 ,6 months had statistical difference between the two groups (P<0 .05) .The adverse reactions in the group A were small ,large amounts of pneumothorax (26 .1% ,6/23) ,bone marrow suppression (26 .1% ,6/23) ,nausea and vomiting(30 .4% ,7/23) ,which in the group B were d 5 .3% (1/19) in the group B were bone marrow suppression (36 .8% ,7/19) ,nausea and vomiting (26 .3% ,5/19) .No severe complications were observed .The occurrence rate of adverse reactions had no statistical difference between the two groups (P>0 .05) .The adverse reactions were improved after symp-tomatic treatment .Conclusion CT guided 125 I seed implantation combined with chemotherapy has higher effective rate and more significant effect in treating unresectable non-small cell lung cancer .
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Objective To investigate the extraction method of total flavonoids from the leaves of ficus lacor and the protec tive effects of extraction on the cellular damage to provide a basis for the research on the phamaceutical value of ficus lacor leaves.Methods The ethanol extraction method was adopted to extract the total flavonoids in the leaves of ficus lacor and the extraction efficiency was calculated with rutin as the standard.The rotenone induced human lung adenocarcinoma cellular damage served as the model,then the influencesof the extraction on the cellular viability,cellular morphology,production of reactive oxygen species (ROS) and apoptosis were researched.Results The extraction efficiency of total flavonoids in the leaves of ficus lacor by 60% ethanol was 5.02%;the extraction at the concentration of 32 mg/L could significantly inhibit the decrease of cell viability,cellular shape change,ROS production and apoptosis of A549 cells induced by 100μg/L rotenone.Conclusion The ethanol extraction method can be used to extract the total flavonoids in the leaves of ficus lacor and the extraction has the protective effects on the A549 cellular dam age induced by rotenone,the leaves of ficus lacor have the potential for further researching its pharmaceutical value.
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<p><b>OBJECTIVE</b>To track the source of infection regarding 4 Cholerae outbreaks in Anhui province in 2012 through the application of PulseNet China Database (PNCD).</p><p><b>METHODS</b>Cholerae virulence genes were amplified by PCR and typed by pulse field gel electrophoresis(PFGE). Results from electrophoresis were cluster-analyzed by BioNumericsV4.0 software and compared with PNCD.</p><p><b>RESULTS</b>Virulence gene CT and TCP of the tested vibrio cholera showed both positive. Homology of the strains from four cholera outbreaks was more than 98%, based on the homologous and cluster analysis through enzyme digested PFGE electrophoresis. Those strains were highly homologous with the cholera epidemic strains identified in Hunan, Sichuan,Zhejiang, Shanghai and Hubei by PNCD, with the homology as 100% .</p><p><b>CONCLUSION</b>Four cholera outbreaks in Anhui province, 2012 were highly correlated with the outbreaks occurring in Hunan and Sichuan during the same time period, indicating that PNCD could effectively and quickly tracking down the source of infection on the cholera outbreaks and providing early warning of the situation.</p>
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Animals , Humans , Bacterial Typing Techniques , Methods , China , Epidemiology , Cholera , Epidemiology , Cluster Analysis , DNA, Bacterial , Databases, Factual , Electrophoresis, Gel, Pulsed-Field , Methods , Polymerase Chain Reaction , Vibrio cholerae , GeneticsABSTRACT
Three patients with severe acute pancreatitis [SAP] developed into overt abdominal compartment syndrome [ACS] and confirmed or suspected infection of necrotic tissue. We successfully treated these patients by minimally invasive decompression with the assist of laparoscope after the failures of intensive care treatments. This technique we report here may be another safe and effective management for ACS in SAP
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Humans , Male , Female , Minimally Invasive Surgical Procedures , Pancreatitis , Acute Disease , Disease Management , Decompression, Surgical , Multiple Organ FailureABSTRACT
Sall4, a member of sall4 gene family, plays important roles in embryo development; organogenesis as well as pluripotency maintenance and re-establishment. There are two isoforms of Sall4, Sall4A and Sall4B. The sequence of porcine sall4 gene is still not reported. Because of its distinct role in maintaining the pluripotent state of stem cells, we cloned and sequenced porcine sall4 gene and assessed its expression in pig tissues and embryos. One 2 372 bp nucleotide sequence representing the full-length cDNA of pig sall4 was obtained by 5'and 3'RACE. Analyses of putative protein sequence showed a 70% to 80% identity with isoform Sall4B of human and mouse. Comparing with Sall4A, the identity reduced to 30% to 55% because of the loss of a zinc-finger domain-rich fragment. Assessment of sall4b expression in porcine tissues by Real-time PCR showed that it expressed most strongly in ovary and stronger in spleen, lung, heart and testis. For preimplantation embryos, the expression level was lower in 4-cell embryos compared with other stages. Immuno-fluorescence analysis of Sall4 on porcine preimplantation embryos indicated that it expressed in all the preimplantation embryos and located in nucleus, in blastocyst it preferentially limited in ICM cells. Expression pattern in early embryos suggest that pig sall4b is associated with pluripotency and might be a new and useful reprogramming factor for establishing pig induced pluripotent stem cell lines.
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Animals , Female , Humans , Mice , Amino Acid Sequence , Base Sequence , Cloning, Molecular , DNA-Binding Proteins , Genetics , Embryonic Development , Genetics , Gene Expression Regulation, Developmental , Molecular Sequence Data , Ovary , Metabolism , Swine , Embryology , Genetics , Metabolism , Transcription Factors , Genetics , Transcription, Genetic , PhysiologyABSTRACT
<p><b>OBJECTIVE</b>To constitute a composite skin substitute that can proliferate well with epidermal stem cells and fibroblasts on collagen sponge.</p><p><b>METHODS</b>Epidermal stem cells were selected by rapid attachment to collagen IV for 10-15 min and cultured on 3T3 feeder layers. Collagen was extracted from rat tail. The matrix lattice was fabricated by freeze-dryer and cross-linked with glutaraldehyde. Fibroblasts were inoculated on collagen sponge and cultured for 4 days prior to inoculation of epidermal stem cells to construct composite skin substitute. The composite skin substitute were examined by means of histology, immunohistochemistry and electron microcopy, the histologic appearance was similar to that of normal epidermis.</p><p><b>RESULTS</b>The epidermal stem cells formed large colonies at 7-8 days, expressed K19 antigen. The percentages of cells at G0/G1 phase of cell cycle and the percentage of alpha6 briCD71dim cells in ESC groups were higher than those in the control group. The skin substitute had epidermis and dermis, the histologic appearance was similar to that of normal skin. The artificial skin expressed keratin antigen by immunocytochemical methods.</p><p><b>CONCLUSIONS</b>Epidermal stem cells proliferated well and differentiated properly on this artificial skin dermis which contained fibroblasts. It seemed that the composite skin to be a good equivalent.</p>
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Animals , Humans , Mice , Rats , 3T3 Cells , Cell Culture Techniques , Epidermis , Cell Biology , Skin, Artificial , Stem Cells , Cell Biology , Tissue EngineeringABSTRACT
To investigate the cause of death of a pregnant woman with undefined pneumonia reported from the People's Hospital of Tongling City in Anhui Province on November 8, 2005, the patient's tracheal aspirates and serum samples were collected and tested by RT-PCR and Real-time PCR to detect viral nucleic acids of HA of A/H5N1, A/H7N7, A/H9N1 and A/M. Tracheal aspirates were inoculated into special pathogen free (SPF) embryonated eggs for cultivation and identification of virus. The HA gene of the virus was sequenced and analyzed. Serum samples were tested by HI assay to detect antibody of H5N1. The results showed that HA gene of A/H5N1 virus and A/M were positive in tracheal aspirates by both PCR tests. The serum sample collected on Nov. 9 was A/M gene positive by Real-time PCR. The analysis of HA gene of A/AnHui/1/2005 sequence showed that the receptor specificity and the connecting peptide between HA1 and HA2 were still avian influenza origin. The HI antibody of H5N1 was negative at 7th, 8th, 9th d of disease onset. This undefined pneumonia case was confirmed as the first pregnant woman case of avian influenza (H5N1) virus infection by etiology in the mainland of China.
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Adult , Female , Humans , Pregnancy , Antibodies, Viral , Blood , Hemagglutination Inhibition Tests , Hemagglutinin Glycoproteins, Influenza Virus , Genetics , Influenza A Virus, H5N1 Subtype , Classification , Genetics , Influenza, Human , Virology , Polymerase Chain Reaction , Pregnancy Complications, Infectious , Virology , Trachea , VirologyABSTRACT
<p><b>OBJECTIVE</b>To ascertain the causation of a pregnant woman with undefined pneumonia reported from the People's Hospital of Tongling city in Anhui province on November 2005.</p><p><b>METHODS</b>Epidemiological and clinical information of the case was collected from the keypersons close to the case and referring to the medical record. A medical observation was carried out on the close contacts of the case and sick or dead poultry. Tracheal aspirates being collected were tested by both RT-PCR and real-time PCR to detect viral nucleic acids of A/H5N1, and were inoculated into special pathogen free (SPF) embryonated hens' eggs.</p><p><b>RESULTS</b>The pregnant woman was found to have been contacted with the sick/dead poultry directly on the 4th day before onset of illness. All the 122 close contacts were healthy after a 10-day medical observation. The major clinical features of the case were viral pneumonia with rapidly developed leukopenia and lymphopenia. The progress to acute respiratory distress syndrome and multiple organ dysfunction syndromes was found at clinical presentation. HA and NA gene of A/H5N1 virus were positive. The 8 gene fragments of A/Anhui/1/2005 (H5N1) isolated from the tracheal aspirates had not carried genes from a human virus through reassortment, and the receptor-binding site of the hemagglutinin was polybasic cleavage site.</p><p><b>CONCLUSION</b>This was the first documented case of H5N1 infection in pregnant woman. The immunotolerant state of pregnancy might have predisposed to the fatal outcome of the patient.</p>
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Adult , Female , Humans , Pregnancy , China , Fatal Outcome , Influenza A Virus, H5N1 Subtype , Genetics , Influenza, Human , Pathology , Multiple Organ Failure , Pneumonia , Virology , Polymerase Chain Reaction , Pregnancy Complications, Infectious , Virology , Respiratory Distress Syndrome , Trachea , VirologyABSTRACT
Better therapeutic effects have been achieved in the treatment of 112 simple obesity cases,mainly with acupuncture therapy plus auxiliary treatment of acupuncture weight loss apparatus. The results showed that the effective rate was 85% in 112 cases by one course of the treatments, 92% in 84 cases by two courses of the treatments, and 98% in 43 cases by three courses of the treatments. The therapeutic effect in combination with acupuncture apparatus was better in long courses of the treatment than in the short courses of the treatments, in male than in female, in young age than in old age, in mild case than in severe condition, in short duration than in long duration, in excessive pattern than in deficient pattern, in those without complications than in those with complications, and in those without family history than in those with family history, indicating that early treatment and prolonged treatment can enhance the therapeutic effects in weight loss by acupuncture.