| Part ⅠClinical characteristics and risk factors of nosocomial death of sporadic thoracic aortic dissectionObjective: To analyze the clinical characteristics and independent risk factors of hospital death in patients with sporadic acute thoracic aortic dissection through a retrospective study of acute thoracic aortic dissection without family history.Methods: The clinical data of 220 patients with sporadic acute thoracic aortic dissection diagnosed in the affiliated Hospital of Jining Medical College from January 1,2019 to December 31,2021 were collected retrospectively,including age,sex,time of onset,past history,vital signs and auxiliary examination.According to the discharge condition,the patients were divided into survival group and death group.The potential risk factors were determined by univariate analysis,and the independent risk factors of hospital death were determined by multivariate Logistic analysis.Results: 1.The average age of STAAD patients was 55.5±13.4 years old,and the male to female ratio was about 3.3:1.The mean age of onset was 53.1±13.2 in males and 62.47±11.4 in females.The age range of 50 to 59 years old is the most common age for STAAD patients.180 patients(81.8%)had a history of hypertension,130 patients(59.1%)had a history of smoking,27 patients(12.3%)had a history of coronary heart disease,127 patients(57.5%)had aortic reflux,80 patients(36.4%)had pericardial effusion,and 58 patients(26.4%)had pleural effusion.164patients(74.5%)underwent surgery,and 45 patients(20.5%)died in hospital.2.It is common in November,December and January in winter,accounting for 38.6%,among which January has the highest incidence,accounting for 14.1% of the total number of the year.3.Stanford A and Stanford B intersections had fewer thrombines than men,had a low smoking history,had high thrombintime white blood cell count,low Plasmafibrinogen on admission,and were prone to complications.Longer mechanical ventilation is required and hospital mortality is higher.4.Age,type A dissection,complicated acute kidney injury,and absence of surgery were independent risk factors for in-hospital death.Conclusion: 1.Sporadic acute thoracic aortic dissection is common in elderly males and later in females,especially in winter and spring.The patients were well complicated with a history of hyperemia and smoking,with a high level of white blood cell count at admission,often complicated with various complications and a high mortality.2.Stanford type A dissection has more white blood cell count and prothrombin time than Stanford type B dissection,and is more likely to be complicated with aortic valve regurgitation,pericardial effusion and pleural effusion,easy to involve coronary sinus,longer mechanical ventilation and higher mortality.3.Age,type A dissection,acute renal injury and non-operation were the independent risk factors of hospital death.Part Ⅱ:Analysis of the results of exon gene screening for sporadic thoracic aortic dissectionObjective: Through to the part in our hospital diagnosed as sporadic acute thoracic aortic dissection(Sporadicacutethoracicaorticdissection,STAAD)on patients with all exons gene sequencing and advanced analysis,analysis of its pathogenic gene and its related pathways.Methods: 39 patients diagnosed with STAAD in Affiliated Hospital of Jining Medical College were sequenced by whole exon gene sequencing(WES)and the enrichment pathway was analyzed.Results: 1.74 mutations were identified as pathogenic by the mutation classification system of ACMG.2.Screen out MAP2K3,C17orf103,PRIM2,PRSS2,PRSS3P2,PRSS1,HCG4 B,PRSS3,HCG9,HLA-A,LILRA4,HLA-DRB1,HLA-DRB6,LILRA5,MIR570,ZNF141,HS6.There were 20 pathogenic genes of STAAD,including ST1,SDHAP2,LILRB5 and MUC3 A.3.In terms of BP,GO enrichment analysis mainly concentrated on the O-glycosylation process and the production and regulation of interleukin-6.CC is mainly enriched on the trans-Golgi network membrane.At MF level,the lubrication activity of extracellular matrix components was mainly enriched.4.KEGG enrichment analysis was mainly concentrated in B cell receptor signaling pathway and osteoclast differentiation.5.PPI network analysis showed that HLA-A had close interaction with HLA-DRB1,HLA-DRB5,LILRA2 and LILRA3.Conclusion: The top 20 P-value genes with the most significant mutations: MAP2K3,C17orf103,PRIM2,PRSS2,PRSS3P2,PRSS1,HCG4 B,PRSS3,HCG9,HLA-A,LILRA4,HLA-DRB1,HLA-DRB6,LILRA5,MIR570,ZNF141,HS6ST1,SDHAP2,LILRB5,MUC3 A,among which HLA-A may play an important role in the occurrence of STAAD,providing a basis for further experimental verification. |