Objective To investigate the management of pregnancy and cardiovascular complications in women with Marian syndrome(MFS).Methods From October 1994 to September 2006, 30 patients with MFS undergoing cardiovascular surgery were studied retrospectively.Results In the labor of 46 offsprings given birth by 30 women,5 cases(11%)were performed elective cesarean section because of the existence of aortic complication,and 12(26%)were diagnosed as MFS.The gestation in two patients was terminated due to deterioration of aortic abnormalities during their third trimester,and they received surgical treatment with Bcntall procedure.Two developed acute aortic dissection during labor and post delivery respectively.With the manipulation of anticoagulation peripartum,one who had the implantation with mechanical prosthesis went through pregnancy and delivery uneventfully.The average duration between delivery and cardiovascular surgery was(15?9)years.Conclusions Vaginal delivery can be done safely in patients with the MFS who do not have or have mild cardiovascular system abnormalities,aortic dissection,or other important cardiac abnormalities,cesarean section should be the preferred method of delivery.Women with MFS are at increased risk for dissection and congestive heart failure during pregnancy and should be counseled before pregnancy about these risks,as well as the inheritance of the condition.

Photothermal therapy, as a new non-invasive therapy technique, has been developing rapidly since 1980s, especially in tumor therapy. Compared with traditional therapy, it has demonstrated irreplaceable effects. At present, the materials used for photothermal therapy are mainly various nanoparticles as well as various nanocomposites designed based on it. Because some nanocomposites have integrated the properties of photothermal therapeutic agents and imaging contrast agents, they have played an important role in the integrated study of tumor diagnosis and treatment. Based on the physical and chemical properties, optical properties, advantages and disadvantages of the four kinds of nano-therapeutic agents in the study of photothermal therapy, this paper reviews the progress of the experimental research on the integration of the four kinds of nano-photothermal agents in tumor diagnosis and treatment in recent years.

Objective To investigate the impacts of LINC00943 on proliferation,apoptosis,and invasion of esophageal squamous cell carcinoma(ESCC)cells by regulating the miR-126-5p/HOXB2 axis.Methods QRT-PCR was applied to detect the expression of LINC00943 in 45 ESCC tissues and cell lines;The effects of LINC00943 knockdown on the proliferation,apoptosis and invasion of KYSE30 cells were detected by MTT assay,flow cytometry and Transwell chamber.Western blot was applied to detect the expression of proliferating cell nuclear antigen(PCNA),Bcl-2 associated X protein(Bax),anti apoptotic factor B cell lymphomatoma-2(Bcl-2),cleaved caspase-3,matrix metalloproteinase-2(MMP-2),MMP-9,and HOXB2;dual luciferase reporter gene experiment was applied to verify the relationship between miR-126-5p and LINC00943 and HOXB2;the ESCC nude mouse in vivo model was constructed and separated into si-NC and si-LINC00943 groups,the tumor mass and volume were measured,qRT-PCR was applied to detect the expression of miR-126-5p in transplanted tumor tissue,immunohistochemistry was applied to detect the expression of HOXB2 protein in transplanted tumor tissue,and TUNEL staining was applied to detect cell apoptosis in tumor tissue.Results The expression of LINC00943 mRNA increased in ESCC tissues and cell lines(P＜0.05);Compared with the si-NC group,the proliferation activity,migration and invasion cell number,HOXB2,PCNA,MMP-2,MMP-9 and Bcl-2 protein expression of KYSE30 cells in the si-LINC00943 group were decreased,and the expression of miR-126-5 p,Bax,Cleaved Caspase-3 and apoptosis rate were increased(P＜0.05);downregulation of miR-126-5p was able to weaken the inhibitory effect of LINC00943 knockdown on the malignant behavior of KYSE30 cells(P＜0.05);dual luciferase reporter gene experiment confirmed the targeted regulatory relationship between miR-126-5p and LINC00943,and miR-126-5p and HOXB2(P＜0.05);The ESCC nude mouse model was constructed in vivo and divided into si-NC and si-LINC00943 groups.The tumor mass,tumor volume,expression of miR-126-5p and HOXB2 in transplanted tumor tissues and apoptosis rate of tumor cells were measured.(P＜0.05).Conclusion LINC00943 is highly expressed in ESCC tissues and cell lines.Knocking down LINC00943 may inhibit the expression of HOXB2 by up-regulating the expression of miR-126-5p,promote the apoptosis of ESCC cells,and inhibit their proliferation,migration and invasion.

Objectives: To explore the clinical experience for a bridge therapy of percutaneous balloon aortic valvuloplasty (PBAV) in treating the patients with severe aortic stenosis (AS). Methods: A total of 37 patients with severe AS who were not suitable for surgical valvular replacement received PBAV in our hospital from 2011-03 to 2017-03 were retrospectively studied. The patient's mean age was (74±12) years, their clinical and anatomical features, efficacy and safety of operation were observed and the outcomes were evaluated by follow-up study. Results: Patients presented the high surgical risk and worse cardiac function, 50% of them had bicuspid leaflet morphology with severe calcification [HU850=(856.0±658.2) mm3]. Balloon size was chosen by the intra-operative supra-annular diameters; at 7 days after operation, aortic valve orifice area (AVOA) was increased from (0.37±0.10) cm2to (0.87±1.10) cm2, the mean trans-aortic valve gradient pressure decreased form (55.1±22.9) mmHg to (44.8±17.8) mmHg, P<0.001 and LVEF elevated form(35.8±14.3)% to(41.0±12.2)%,P<0.001.There were 4 patients died in hospital,1 received permanent pacemaker and 1 developed severe aortic valve regurgitation. The patients were followed-up for (16.5±11.1)months after operation, 13/37 (35.1%) patients were in transition to surgical or transcatheter aortic valve replacement (TAVR). Conclusions: PBAV may have good early clinical efficacy in severe AS patients who were not suitable for surgical valvular replacement and TAVR; PBAV could be expected to become a bridge therapy, smaller supra-annular diameter was safe and effective for patients having bicuspid leaflet with severe calcification.

The purpose of this study was to identify the tick species native to Xindi Township,Yumin County,Xinjiang,China.Preliminary morphological identification of parasitic ticks collected from animals in the area was conducted with an ultra-depth of field three-dimensional VHX 600 digital stereo microscope.Total DNA of the ticks was extracted,amplified by PCR based on the COI and ITS2 gene loci,and the posi-tive PCR products were sequenced.The sequence were a-ligned with reference sequences from the NCBI database were aligned with the Basic Local Alignment Search Tool.A genet-ic phylogenetic tree was generated with the neighbor-joining method of MEGA 7.0 software to determine the evolutionary biological characteristics of ticks.Morphological identification showed that the ticks collected from Xindi Township of Yu-min County were consistent with the characteristics of Hya-lomma asiaticum.An evolutionary tree based on the COI and ITS2 gene sequences showed that the ticks collected in this study were clustered with known H.asiaticum sequences.The PCR products of COI and ITS2 were sequenced and compared,which confirmed that the collected tick species were H.asiaticum,in agreement with the morphological and molecular biological results.These findings help to clarify the distribution of ticks in Xindi Township of Xinjiang,and provide basic data for the analysis of tick genetic and evolutionary characteristics,as reference for surveillance and control of ticks in the Xinjiang Uygur Autonomous Region.

ObjectiveTo observe the efficacy and safety of Uyghur medicine Yangxin Dawayimixike Honey Paste （养心达瓦依米西克蜜膏， YDMHP） in the treatment of stable angina pectoris （SAP） of qi stagnation and blood stasis syndrome. MethodsA randomized ， double-blind， positive-controlled，multi-center clinical trial was conducted， in which 370 patients with SAP of qi stagnation and blood stasis syndrome were randomly divided into treatment group（279 cases）and control group（91cases）at a ratio of 3∶1. The treatment group was orally administered with YDMHP， 3 g each time， and placebo of Xuefu Zhuyu Capsule （血府逐瘀胶囊）， 2.4 g each time， while the control group was treated with Xuefu Zhuyu Capsule， 2.4 g each time， and placebo of YDMHP， 3 g each time， both twice a day for a course of 12 weeks. The primary outcome was the effect of angina pectoris symptom. The secondary outcomes include single angina symptom scores such as number of attacks， duration of attacks， pain intensity and usae of nitroglycerin scores， the total angina symptom score before and after the treatment， the usage of nitroglycerin， the exercise duration in treadmill exercise test （TET） and the Duck treadmill score among patients，the scores of Seattle Angina Questionnaire （SAQ） on five dimensions including physical limitations， anginal stability， anginal frequency， treatment satisfaction， and disease perception， and efficacy of TCM syndrome and of each single TCM symptom after treatment. The safety were evaluated by examine blood routine， urine routine， liver and kidney function， fasting blood sugar， electrocardiogram， adverse events. ResultsThe total effective rate of angina symptom in the treatment group was 71.69% （200/279）， significantly higher than 51.64% （47/91） in the control group （P＜0.01）. The curative and markedly effective rate of TCM syndrome in the treatment group was 53.05% （148/279）， which was significantly higher than 25.27% （23/91） in the control group （P＜0.01）. After treatment， scores of the number as well as duration of angina attacks and pain severity， the total score of angina symptoms， and the usage of nitroglycerin significantly decreased in both groups， and more changes were seen in the treatment group than in the control group； the scores of physical limitations， anginal stability， anginal frequency， treatment satisfaction， and disease perception in both groups significantly increased， and more improvement were shown in the experimental group regarding the anginal stability， anginal frequency and treatment satisfaction （P＜0.05 or P＜0.01）. The effects of chest pain， chest tightness， palpitation， shortness of breath and fatigue in experimental group were significantly higher than those in control group （P＜0.05 or P＜0.01）. There was no significant difference in the exercise duration of treadmill test and Duke score among patients between the two groups either before or after treatment （P＞0.05）. Adverse events occurred in 66 cases （23.66%） of the experimental group and 16 cases （17.58%） of the control group， with no statistical significance between the two groups （P＞0.05）. ConclusionThe Uyghur medicine YDMHP can effectively improve symptoms of angina pectoris， reduce the number， duration， and intensity of attacks， decrease the dosage of nitrogly-cerin and improve the individual TCM symptoms and has good safety in the treatment of SAP patients of qi stagnation and blood stasis.

Objective: To evaluate the safety and long-term clinical efficacy of percutaneous coronary intervention (PCI) in patients with in-stent chronic total occlusion (IS-CTO) lesions. Metheds: This is a retrospective analysis. Patients with IS-CTO who underwent PCI in Fuwai hospital from January 2010 to December 2013 were enrolled. A total of 212 patients who met the inclusion criteria were included in the IS-CTO group, 212 matched patients with primary CTO lesions were included in the de novo CTO group. The incidence of complications and the success rate of PCI were compared between the two groups. Successful PCI was defined as successfully implantation of stent(s) at target CTO lesions. The primary endpoint was defined as a composite event of cardiac death and myocardial infarction (MI). Secondary endpoints including PCI success, all-cause death, cardiac death, MI, target vessel related MI, revascularization, target vessel revascularization, heart failure for rehospitalization. The patients were followed up for 5 years after PCI. Results: A total of 424 cases were included. The mean age was (57.8±10.5) years, there were 364 males in this cohort. The left ventricular ejection fraction was significantly lower ((58.7±9.2)% vs. (61.0±7.7)%, P=0.01) and the SYNTAX scores was significantly higher (19.4±8.3 vs. 15.3±10.0, P<0.01) in IS-CTO group than that in de novo CTO group. The proportion of patients with target CTO lesions in left anterior descending artery was significantly higher (42.9% (50/212) vs. 23.6% (91/212), P<0.01) in IS-CTO group than that in de novo CTO group. The rate of successful PCI (71.7% (152/212) vs. 69.8% (148/212), P=0.70) and complication (40.6% (86/212) vs. 36.3% (77/212), P=0.37) was similar between the two groups. The incidence of primary endpoint at 5 years was significantly higher in IS-CTO group (10.8% (23/212) vs. 4.7% (10/212), P=0.02), which was driven by higher incidence of MI (9.0% (19/212) vs. 4.2% (9/212), P=0.05). There were a trend of higher secondary endpoints in IS-CTO group (all P>0.05). Conclusion: The safety and effectiveness of PCI are acceptable in patients with IS-CTO, but the risk of long-term cardiac death and MI is higher among patients with IS-CTO as compared to patients with primary CTO lesions.

Objective: To evaluate the 4-year clinical outcomes of patients following Firesorb bioresorbable scaffold (BRS) implantation. Methods: The study reported the 4-year follow-up results of the FUTURE I study. FUTURE I was a prospective, single-center, open-label, first-in-man study which evaluated the feasibility, preliminary safety, and efficacy of Firesorb stent in the treatment of coronary artery stenosis. A total of 45 patients with single de novo lesions in native coronary arteries ,who hospitalized in Fuwai Hospital from January to March 2016 were enrolled. After successfully stent implantation these patients were randomized in a 2∶1 ratio into cohort 1 (n=30) or cohort 2 (n=15). The patients in cohort 1 underwent angiographic, IVUS or OCT examination at 6 months and 2 years; and cohort 2 underwent angiographic, IVUS or OCT at 1 and 3 years. All patients underwent clinical follow-up at 1, 6 months and 1 year and annually thereafter up to 5 years. The primary endpoint was target lesion failure (TLF, including cardiac death, target vessel myocardial infarction, and ischemia-driven target lesion revascularization). Secondary endpoints included patient-oriented composite endpoint (PoCE, defined as composite of all death, all miocardial infarction, or any revascularization). Results: A total of 45 patients were enrolled and implanted with Firesorb BRS, including 35 males (77.8%), and the age was (54.4±9.3) years. At 4 years, 10 patients in cohort 1 were reexamined by coronary angiography and OCT examination. Among them, 2 patients' stents were completely degraded and absorbed. Compared with the OCT images of the other 8 patients in cohort 2 at 3 years, the degree of stent degradation was increased, and no stent adherence was found. The 4-year clinical follow-up rate was 100%. In 4-year clinical following up, 2 patients suffered PoCE (4.4%): 1 patient underwent non-target vessel revascularization the day after index procedure and target vessel revascularization (Non-target lesion revascularization) at 2-year imaging follow-up; the other patient underwent target lesion revascularization during imaging follow-up at 4 years but not due to ischemic driven. There was no scaffold thrombosis or TLF events through 4 years. Conclusions: Four years after the implantation, complete degradation and absorption of the Firsorb stent are evidenced in some patients. Firesorb stent is feasible and effective in the treatment of patients with non-complex coronary lesions.
Absorbable Implants ; Cardiovascular Agents ; Coronary Artery Disease/surgery* ; Drug-Eluting Stents ; Humans ; Male ; Middle Aged ; Percutaneous Coronary Intervention ; Prospective Studies ; Sirolimus ; Treatment Outcome

Objective: To investigate the predictive value of SYNTAX-Ⅱ score on long term prognosis of patients diagnosed with chronic total occlusion (CTO) and received percutaneous coronary intervention (PCI). Methods: Patients undergoing CTO-PCI in Fuwai hospital from January 2010 to December 2013 were enrolled in this retrospective analysis. The SYNTAX-Ⅱ score of the patients was calculated. According to SYNTAX-Ⅱ score tertiles, patients were stratified as follows: SYNTAX-Ⅱ≤20, 2027. Primary endpoint was major adverse cardiac events (MACCE), including all-cause death, myocardial infarction, stroke and any revascularization. Secondary endpoints included stent thrombosis, heart failure and target lesion failure (TLF). Patients were followed up by outpatient visit or telephone call at 1 month, 6 months and 1 year after PCI, and annually up to 5 years. Multivariate Cox regression model was used to analyze the independent risk factors of all-cause death in patients undergoing CTO-PCI. The predictive value of SYNTAX score with SYNTAX-Ⅱ score for all-cause death was evaluated by the receiver operating characteristic (ROC) curve and the area under the curve (AUC). Results: A total of 2 391 patients with CTO and received PCI were enrolled in this study. The mean age was (57.0±10.5) years, 1 994 (83.40%) patients were male. There were 802 patients in lower tertile group (SYNTAX-Ⅱ≤20), 798 patients in intermediate group (2027). At the end of 5-year follow-up, the loss to follow-up rate of the three groups was 9.10%(73/802), 10.78%(86/798)and 8.85%(70/791), respectively. The rate of all-cause mortality (1.78% (13/729) vs. 3.65% (26/712) vs. 9.02% (65/721), P<0.001), cardiac death (1.37% (10/729) vs. 2.11% (15/712) vs. 4.85% (35/721), P<0.001), target vessel myocardial infarctions (4.25% (31/729) vs. 4.49% (32/712) vs. 7.07% (51/721), P=0.03), probable stent thrombosis (1.51% (11/729) vs. 2.81% (20/712) vs. 3.61% (26/721), P=0.04) and heart failure (1.78% (13/729) vs. 1.97% (14/712) vs. 5.41% (39/721), P<0.001) increased in proportion to increasing SYNTAX-Ⅱ score (all P<0.05). Multivariable Cox regression analysis indicated that female (HR=2.05, 95%CI 1.12-3.73, P=0.01), left ventricular ejection fraction (HR=0.97, 95%CI 0.95-1.00, P=0.05) and SYNTAX-Ⅱ score (HR=1.07, 95%CI 1.02-1.11,P=0.01) were independent predictors for all-cause mortality in patients undergoing CTO-PCI. The predicted value of the SYNTAX-Ⅱ score for all-cause death was significantly higher than the SYNTAX score (AUC 0.71 vs. 0.60, P=0.003). Conclusion: For CTO patients who underwent percutaneous coronary intervention, SYNTAX-Ⅱ score is an independent predictor for 5-year all-cause death, and SYNTAX-Ⅱ serves as an important predictor for all-cause death in these patients.
Humans ; Male ; Female ; Middle Aged ; Aged ; Percutaneous Coronary Intervention ; Coronary Artery Disease ; Retrospective Studies ; Stroke Volume ; Treatment Outcome ; Ventricular Function, Left ; Myocardial Infarction ; Prognosis ; Risk Factors ; Heart Failure ; Coronary Occlusion/surgery*

Objective: To explore the heterogeneity and correlation of clinical phenotypes and genotypes in children with disorders of sex development (DSD). Methods: A retrospective study of 1 235 patients with clinically proposed DSD in 36 pediatric medical institutions across the country from January 2017 to May 2021. After capturing 277 DSD-related candidate genes, second-generation sequencing was performed to analyzed the heterogeneity and correlation combined with clinical phenotypes. Results: Among 1 235 children with clinically proposed DSD, 980 were males and 255 were females of social gender at the time of initial diagnosis with the age ranged from 1 day of age to 17.92 years. A total of 443 children with pathogenic variants were detected through molecular genetic studies, with a positive detection rate of 35.9%. The most common clinical phenotypes were micropenis (455 cases), hypospadias (321 cases), and cryptorchidism (172 cases) and common mutations detected were in SRD5A2 gene (80 cases), AR gene (53 cases) and CYP21A2 gene (44 cases). Among them, the SRD5A2 mutation is the most common in children with simple micropenis and simple hypospadias, while the AMH mutation is the most common in children with simple cryptorchidism. Conclusions: The SRD5A2 mutation is the most common genetic variant in Chinese children with DSD, and micropenis, cryptorchidism, and hypospadias are the most common clinical phenotypes. Molecular diagnosis can provide clues about the biological basis of DSD, and can also guide clinicians to perform specific clinical examinations. Target sequence capture probes and next-generation sequencing technology can provide effective and economical genetic diagnosis for children with DSD.
3-Oxo-5-alpha-Steroid 4-Dehydrogenase/genetics* ; Child ; China/epidemiology* ; Cryptorchidism/genetics* ; Disorders of Sex Development/genetics* ; Female ; Genital Diseases, Male ; Genotype ; Humans ; Hypospadias/genetics* ; Male ; Membrane Proteins/genetics* ; Penis/abnormalities* ; Phenotype ; Retrospective Studies ; Steroid 21-Hydroxylase/genetics*