Pub Date : 2023-01-01DOI: 10.27658/d.cnki.gzzyy.2023.000075
李金璞
{"title":"基于能量开关AMPK/PGC1α通路探讨加减缓衰方在CKD合并CVD中的作用和机制研究","authors":"李金璞","doi":"10.27658/d.cnki.gzzyy.2023.000075","DOIUrl":"https://doi.org/10.27658/d.cnki.gzzyy.2023.000075","url":null,"abstract":"","PeriodicalId":35615,"journal":{"name":"Chinese Medical Sciences Journal","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142028220","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.27658/d.cnki.gzzyy.2023.000247
汪麟双
{"title":"参芪益智颗粒治疗阿尔茨海默病的作用机制研究","authors":"汪麟双","doi":"10.27658/d.cnki.gzzyy.2023.000247","DOIUrl":"https://doi.org/10.27658/d.cnki.gzzyy.2023.000247","url":null,"abstract":"","PeriodicalId":35615,"journal":{"name":"Chinese Medical Sciences Journal","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142031010","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.27658/d.cnki.gzzyy.2023.000023
虞慕瑶
{"title":"三七瘤状突起的形成机制及其对品质的影响研究","authors":"虞慕瑶","doi":"10.27658/d.cnki.gzzyy.2023.000023","DOIUrl":"https://doi.org/10.27658/d.cnki.gzzyy.2023.000023","url":null,"abstract":"","PeriodicalId":35615,"journal":{"name":"Chinese Medical Sciences Journal","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142027986","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Lulu Ma , Xuerong Yu , Xisheng Weng , Jin Lin , Jin Jin , Wenwei Qian , Yuguang Huang
Objective
Total knee arthroplasty is one of the most common orthopedic surgeries. Readmission due to severe complications after total knee arthroplasty is a grave concern to surgeons. In this study, we evaluated the risk factors for severe complications after primary total knee arthroplasty.
Methods
We retrospectively collected clinical data of 2,974 patients who underwent primary total knee arthroplasty from July 2013 to June 2019 in our hospital. Postoperative complication > grade IE was defined as severe complication according to Clavien-Dindo classification system. Binary logistic regression was used to identify the predictive risk factors for severe complications.
Results
The complication rate after primary total knee arthroplasty was 6.8% and severe complication rate was 2.5%. Male (OR = 2.178, 95%CI: 1.324-3.585, P = 0.002), individuals above 75 years old (OR = 1.936, 95%CI: 1.155-3.244, P = 0.012), arrhythmia (OR = 2.913, 95%CI: 1.350-6.285, P = 0.006) and cerebrovascular disease (OR = 2.804, 95%CI: 1.432-5.489, P = 0.003) were predictive risk factors for severe complications after primary total knee arthroplasty.
Conclusion
Advanced age, male, arrhythmia, and cerebrovascular disease might be patients-related risk factors for postoperative severe complications after primary total knee arthroplasty. Special attention should be paid to patients with risk factors.
目的全膝关节置换术是最常见的骨科手术之一。全膝关节置换术后由于严重并发症导致的再入院是外科医生严重关注的问题。在这项研究中,我们评估了原发性全膝关节置换术后严重并发症的危险因素。方法回顾性收集2013年7月至2019年6月在我院行一期全膝关节置换术的2974例患者的临床资料。术后并发症>根据Clavien-Dindo分级系统,重度并发症为IE级。采用二元逻辑回归确定严重并发症的预测危险因素。结果原发性全膝关节置换术后并发症发生率为6.8%,严重并发症发生率为2.5%。男性(OR = 2.178, 95%CI: 1.324 ~ 3.585, P = 0.002)、75岁以上(OR = 1.936, 95%CI: 1.155 ~ 3.244, P = 0.012)、心律失常(OR = 2.913, 95%CI: 1.350 ~ 6.285, P = 0.006)、脑血管疾病(OR = 2.804, 95%CI: 1.432 ~ 5.489, P = 0.003)是原发性全膝关节置换术后严重并发症的预测危险因素。结论高龄、男性、心律失常、脑血管疾病可能是原发性全膝关节置换术后严重并发症的危险因素。应特别注意有危险因素的患者。
{"title":"Possible Risk Factors for Severe Complications Occurring after Primary Total Knee Arthroplasty","authors":"Lulu Ma , Xuerong Yu , Xisheng Weng , Jin Lin , Jin Jin , Wenwei Qian , Yuguang Huang","doi":"10.24920/003938","DOIUrl":"10.24920/003938","url":null,"abstract":"<div><h3>Objective</h3><p>Total knee arthroplasty is one of the most common orthopedic surgeries. Readmission due to severe complications after total knee arthroplasty is a grave concern to surgeons. In this study, we evaluated the risk factors for severe complications after primary total knee arthroplasty.</p></div><div><h3>Methods</h3><p>We retrospectively collected clinical data of 2,974 patients who underwent primary total knee arthroplasty from July 2013 to June 2019 in our hospital. Postoperative complication > grade IE was defined as severe complication according to Clavien-Dindo classification system. Binary logistic regression was used to identify the predictive risk factors for severe complications.</p></div><div><h3>Results</h3><p>The complication rate after primary total knee arthroplasty was 6.8% and severe complication rate was 2.5%. Male (<em>OR =</em> 2.178, 95%<em>CI</em>: 1.324-3.585, <em>P</em> = 0.002), individuals above 75 years old (<em>OR =</em> 1.936, 95%<em>CI</em>: 1.155-3.244, <em>P</em> = 0.012), arrhythmia (<em>OR =</em> 2.913, 95%<em>CI</em>: 1.350-6.285, <em>P</em> = 0.006) and cerebrovascular disease (<em>OR</em> = 2.804, 95%<em>CI</em>: 1.432-5.489, <em>P</em> = 0.003) were predictive risk factors for severe complications after primary total knee arthroplasty.</p></div><div><h3>Conclusion</h3><p>Advanced age, male, arrhythmia, and cerebrovascular disease might be patients-related risk factors for postoperative severe complications after primary total knee arthroplasty. Special attention should be paid to patients with risk factors.</p></div>","PeriodicalId":35615,"journal":{"name":"Chinese Medical Sciences Journal","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9272337","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Xin He , Jianping Ning , Hui Xu , Gong Xiao , Huixiang Yang , Weiyuan Wang , Xiaoying Wu , Hongling Yin , Xiaozhao Li
Renal amyloidosis secondary to anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is extremely rare. Here, we reported a 77-year-old woman with ANCA-associated vasculitis. Renal biopsy with Masson trichrome staining showed pauci-immune crescentic glomerulonephritis, and electron microscopy showed amyloid deposition in the mesangial area. Immunofluorescence revealed kappa light chain and lambda light chain negative. Bone marrow biopsy revealed no clonal plasma cell. Finally, she was diagnosed as ANCA-associated vasculitis with secondary renal amyloid A amyloidosis.
{"title":"Renal Amyloidosis Secondary to ANCA-Associated Vasculitis: A Case Report","authors":"Xin He , Jianping Ning , Hui Xu , Gong Xiao , Huixiang Yang , Weiyuan Wang , Xiaoying Wu , Hongling Yin , Xiaozhao Li","doi":"10.24920/003999","DOIUrl":"10.24920/003999","url":null,"abstract":"<div><p>Renal amyloidosis secondary to anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is extremely rare. Here, we reported a 77-year-old woman with ANCA-associated vasculitis. Renal biopsy with Masson trichrome staining showed pauci-immune crescentic glomerulonephritis, and electron microscopy showed amyloid deposition in the mesangial area. Immunofluorescence revealed kappa light chain and lambda light chain negative. Bone marrow biopsy revealed no clonal plasma cell. Finally, she was diagnosed as ANCA-associated vasculitis with secondary renal amyloid A amyloidosis.</p></div>","PeriodicalId":35615,"journal":{"name":"Chinese Medical Sciences Journal","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9278396","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Jin Lu , Shaoguang An , Junjie Ma , Yue Yang , Lei Zhang , Peng Yu , Heng Tao , Yunfan Chen , Haoxuan Zhang
Objective
To investigate the expression of topoisomerase II α (TOP2α) in hepatocellular carcinoma (HCC) and its role in predicting prognosis of HCC patients.
Methods
We used HCC-related datasets in UALCAN, HCCDB, and cBioPortal databases to analyze the expression and mutation of TOP2α and its co-expressed genes in HCC tissues. GO function and KEGG pathway enrichment of TOP2α and its co-expressed genes were identified. The TIMER database was used to analyze infiltration levels of immune cells in HCC. The impacts of TOP2α and its co-expression genes and the infiltrated immune cells on the survival of HCC patients were assayed by Kaplan-Meier plotter analysis.
Results
TOP2α and its co-expression genes were highly expressed in HCC (P < 0.001) and detrimental to overall survival of HCC patients (P < 0.001). TOP2α and its co-expression genes were mainly involved in cell mitosis and proliferation, and cell cycle pathway (ID: hsa04110, P = 0.00194S). TOP2α and its co-expression genes were mutated in HCC and the mutations were significantly detrimental to overall survival (P = 0.0247) and disease-free survival (P = 0.026S) of HCC patients. High TOP2α expression was positively correlated with the infiltration of B cell (r = 0.4S9, P < 0.01), CD8+T cell (r = 0.312, P < 0.01), CD4+T cell (r = 0.370, P < 0.01), macrophage (r = 0.459, P < 0.01), neutrophil (r = 0.405, P < 0.01), and dendritic cell (r = 0.473, P < 0.01) in HCC. The CD8+T cell infiltration significantly prolonged the 3- and 5-year survival of HCC patients (all P < 0.05), and CD4+T cell infiltration significantly shortened the 3-, 5-, and 10-year survival of HCC patients (all P < 0.05)
Conclusion
TOP2α may be an oncogene, which was associated with poor prognosis of HCC patients and could be used as a biomarker for the prognostic prediction of HCC.
目的探讨拓扑异构酶II α (TOP2α)在肝细胞癌(HCC)组织中的表达及其对预后的预测作用。方法利用UALCAN、HCCDB和cBioPortal数据库中的HCC相关数据集,分析TOP2α及其共表达基因在HCC组织中的表达和突变。鉴定了TOP2α及其共表达基因的GO功能和KEGG通路富集。使用TIMER数据库分析HCC中免疫细胞的浸润水平。应用Kaplan-Meier绘图仪分析TOP2α及其共表达基因及浸润免疫细胞对肝癌患者生存的影响。结果stop2 α及其共表达基因在HCC中高表达(P <0.001),对HCC患者的总生存期不利(P <0.001)。TOP2α及其共表达基因主要参与细胞有丝分裂、增殖和细胞周期通路(ID: hsa04110, P = 0.00194S)。TOP2α及其共表达基因在HCC中发生突变,对HCC患者的总生存期(P = 0.0247)和无病生存期(P = 0.026S)均有显著影响。高表达的TOP2α与B细胞浸润呈正相关(r = 0.4S9, P <0.01), CD8+T细胞(r = 0.312, P <0.01), CD4+T细胞(r = 0.370, P <0.01),巨噬细胞(r = 0.459, P <0.01),中性粒细胞(r = 0.405, P <0.01),树突状细胞(r = 0.473, P <0.01)。CD8+T细胞浸润显著延长HCC患者的3年和5年生存率(P <CD4+T细胞浸润显著缩短HCC患者3、5、10年生存率(P <0.05)结论top2 α可能是一种致癌基因,与HCC患者预后不良相关,可作为HCC预后预测的生物标志物。
{"title":"Topoisomerase II α Gene as a Marker for Prognostic Prediction of Hepatocellular Carcinoma: A Bioinformatics Analysis","authors":"Jin Lu , Shaoguang An , Junjie Ma , Yue Yang , Lei Zhang , Peng Yu , Heng Tao , Yunfan Chen , Haoxuan Zhang","doi":"10.24920/004006","DOIUrl":"https://doi.org/10.24920/004006","url":null,"abstract":"<div><h3>Objective</h3><p>To investigate the expression of <em>topoisomerase II α</em> (<em>TOP2α</em>) in hepatocellular carcinoma (HCC) and its role in predicting prognosis of HCC patients.</p></div><div><h3>Methods</h3><p>We used HCC-related datasets in UALCAN, HCCDB, and cBioPortal databases to analyze the expression and mutation of <em>TOP2α</em> and its co-expressed genes in HCC tissues. GO function and KEGG pathway enrichment of <em>TOP2α</em> and its co-expressed genes were identified. The TIMER database was used to analyze infiltration levels of immune cells in HCC. The impacts of <em>TOP2α</em> and its co-expression genes and the infiltrated immune cells on the survival of HCC patients were assayed by <em>Kaplan-Meier</em> plotter analysis.</p></div><div><h3>Results</h3><p><em>TOP2α</em> and its co-expression genes were highly expressed in HCC (<em>P</em> < 0.001) and detrimental to overall survival of HCC patients (<em>P</em> < 0.001). <em>TOP2α</em> and its co-expression genes were mainly involved in cell mitosis and proliferation, and cell cycle pathway (ID: hsa04110, <em>P</em> = 0.00194S). <em>TOP2α</em> and its co-expression genes were mutated in HCC and the mutations were significantly detrimental to overall survival (<em>P</em> = 0.0247) and disease-free survival (<em>P</em> = 0.026S) of HCC patients. High <em>TOP2α</em> expression was positively correlated with the infiltration of B cell (<em>r</em> = 0.4S9, <em>P</em> < 0.01), CD8<sup>+</sup>T cell (r = 0.312, <em>P</em> < 0.01), CD4<sup>+</sup>T cell (<em>r</em> = 0.370, <em>P</em> < 0.01), macrophage (<em>r</em> = 0.459, <em>P</em> < 0.01), neutrophil (<em>r</em> = 0.405, <em>P</em> < 0.01), and dendritic cell (<em>r</em> = 0.473, <em>P</em> < 0.01) in HCC. The CD8<sup>+</sup>T cell infiltration significantly prolonged the 3- and 5-year survival of HCC patients (all <em>P</em> < 0.05), and CD4<sup>+</sup>T cell infiltration significantly shortened the 3-, 5-, and 10-year survival of HCC patients (all P < 0.05)</p></div><div><h3>Conclusion</h3><p><em>TOP2α</em> may be an oncogene, which was associated with poor prognosis of HCC patients and could be used as a biomarker for the prognostic prediction of HCC.</p></div>","PeriodicalId":35615,"journal":{"name":"Chinese Medical Sciences Journal","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"91969987","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ran Li , Zhanyun Lv , Yanxin Li , Wei Li , Yanlei Hao
Objective
To study the effects of TYRO protein kinase-binding protein (TYROBP) deficiency on learning behavior, glia activation and pro-inflammatory cycokines, andTau phosphorylation of a new Alzheimer’s disease (AD) mouse model carrying a PSEN1 p.G378E mutation.
Methods
A new AD mouse model carrying PSEN1 p.G378E mutation was built based on our previously found AD family which might be ascribed to the PSEN1 mutation, and then crossed with TYROBP deficient mice to produce the heterozygous hybrid mice (PSEN1G378E/WT; Tyrobp+/–) and the homozygous hybrid mice (PSEN1G378E/G378E; Tyrobp–/–). Water maze test was used to detect spatial learning and memory ability of mice. After the mice were sacrificed, the hippocampus was excised for further analysis. Immunofluorescence was used to identify the cell that expresses TYROBP and the number of microglia and astrocyte. Western blot was used to detect the expression levels of Tau and phosphorylatedTau (p-Tau), and ELISA to measure the levels of pro-inflammatory cytokines.
Results
Our results showed that TYROBP specifically expressed in the microglia of mouse hippocampus. Absence of TYROBP in PSEN1G378E mutation mouse model prevented the deterioration of learning behavior, decreased the numbers of microglia and astrocytes, and the levels of interleukin-6, interleukin-1β and tumor necrosis factor-α in the hippocampus (all P < 0.05). The ratios of AT8/Tau5, PHF1/Tau5, pT181/Tau5, pT231/ Tau5 and p-ERK/ERK were all higher in homozygous hybrid mice (PSEN1G378E/G378E; Tyrobp–/– mice) compared with PSEN1G378E/G378E mice (all P < 0.05).
Conclusions
TYROBP deficiency might play a protective role in the modulation of neuroinflammation of AD. However, the relationship between neuroinflammation processes involving microglia and astrocyte activation, and release of pro-inflammatory cytokines, and p-Tau pathology needs further study.
{"title":"Effects of TYROBP Deficiency on Neuroinflammation of a Alzheimer’s Disease Mouse Model Carrying a PSEN1 p.G378E Mutation","authors":"Ran Li , Zhanyun Lv , Yanxin Li , Wei Li , Yanlei Hao","doi":"10.24920/004059","DOIUrl":"https://doi.org/10.24920/004059","url":null,"abstract":"<div><h3>Objective</h3><p>To study the effects of TYRO protein kinase-binding protein (TYROBP) deficiency on learning behavior, glia activation and pro-inflammatory cycokines, andTau phosphorylation of a new Alzheimer’s disease (AD) mouse model carrying a <em>PSEN1</em> p.G378E mutation.</p></div><div><h3>Methods</h3><p>A new AD mouse model carrying <em>PSEN1</em> p.G378E mutation was built based on our previously found AD family which might be ascribed to the <em>PSEN1</em> mutation, and then crossed with TYROBP deficient mice to produce the heterozygous hybrid mice (<em>PSEN1<sup>G378E/WT</sup></em>; <em>Tyrobp<sup>+/–</sup></em>) and the homozygous hybrid mice (<em>PSEN1<sup>G378E/G378E</sup></em>; <em>Tyrobp<sup>–/–</sup></em>). Water maze test was used to detect spatial learning and memory ability of mice. After the mice were sacrificed, the hippocampus was excised for further analysis. Immunofluorescence was used to identify the cell that expresses TYROBP and the number of microglia and astrocyte. Western blot was used to detect the expression levels of Tau and phosphorylatedTau (p-Tau), and ELISA to measure the levels of pro-inflammatory cytokines.</p></div><div><h3>Results</h3><p>Our results showed that TYROBP specifically expressed in the microglia of mouse hippocampus. Absence of TYROBP in <em>PSEN1<sup>G378E</sup></em> mutation mouse model prevented the deterioration of learning behavior, decreased the numbers of microglia and astrocytes, and the levels of interleukin-6, interleukin-1β and tumor necrosis factor-<em>α</em> in the hippocampus (all <em>P <</em> 0.05). The ratios of AT8/Tau5, PHF1/Tau5, pT181/Tau5, pT231/ Tau5 and p-ERK/ERK were all higher in homozygous hybrid mice (<em>PSEN1<sup>G378E/G378E</sup></em>; <em>Tyrobp<sup>–/–</sup></em> mice) compared with <em>PSEN1<sup>G378E/G378E</sup></em> mice (all <em>P <</em> 0.05).</p></div><div><h3>Conclusions</h3><p>TYROBP deficiency might play a protective role in the modulation of neuroinflammation of AD. However, the relationship between neuroinflammation processes involving microglia and astrocyte activation, and release of pro-inflammatory cytokines, and p-Tau pathology needs further study.</p></div>","PeriodicalId":35615,"journal":{"name":"Chinese Medical Sciences Journal","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"92118368","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
To evaluate the clinical characteristics and prognostic predictors of patients with diffuse alveolar hemorrhage (DAH) and/or interstitial lung disease (ILD) secondary to microscopic polyangiitis (MPA) in a Chinese general hospital.
Methods
We retrospectively reviewed the medical records of MPA patients admitted to internal medicine departments between the year 2002 and 2012. The patients were divided into the ILD, DAH, DAH combined with ILD (DAHILD), and no pulmonary involvement (NPI) groups according to pulmonary involvement patterns. The clinical characteristics at diagnosis were analyzed. The risk factors associated with short-term death and long-term death were identified with Logistic regression and Cox analysis.
Results
Of 193 newly diagnosed MPA patients, 181 patients were enrolled in the research, of which 19 had DAH alone, 96 had ILD alone, 18 had DAH and DAH concurrently, and 48 had NPI. The median of serum creatine level in the DAH group was 449 µmol/L, significantly higher than that in the ILD group (123 µmol/L, Nemenyi = -35.215, P = 0.045) and DAHILD group (359 µmol/L, Nemenji = -43.609, P = 0.007). The median follow-up time was 67 (range: 1-199) months. Patients in the ILD group were older than those in the DAH group (median: 69 years vs. 57 years, Nemenji = 43.853, P = 0.005). The patients with both DAH and ILD had combined features of the two subtypes, and the highest mortality (72.2% at the end of follow-up). The elevated white blood cell count was a risk factor for short-term death (OR = 1.103, 95%CI: 1.008-1.207, P = 0.032 for one month; OR = 1.103, 95%CI: 1.026-1.186, P = 0.008 for one year). Old age (HR = 1.044, 95%C7: 1.023-1.066, P < 0.001), cardiovascular system involvement (HR = 2.093, 95%CI: 1.195-3.665, P = 0.010), poor renal function (HR = 1.001, 95%CI: 1.000-1.002, P = 0.032) were risk factors for long-term death. Pulmonary infections (38/54) were the leading causes of death, especially for the patients with ILD. Besides, 49 patients suffered from pulmonary infections in the first year after diagnosis.
Conclusions
MPA patients who presented with different pulmonary involvement patterns have completely different clinical features. These subtypes probably have different pathogenesis and should be studied separately.
目的探讨某综合医院弥漫性肺泡出血(DAH)和/或间质性肺疾病(ILD)继发于显微多血管炎(MPA)的临床特点及预后预测因素。方法回顾性分析2002 ~ 2012年内科收治的MPA患者的病历资料。根据肺受累情况将患者分为ILD组、DAH组、DAH合并ILD组(DAHILD)和无肺受累组(NPI)。分析诊断时的临床特点。通过Logistic回归和Cox分析确定与短期死亡和长期死亡相关的危险因素。结果193例新诊断的MPA患者中,181例纳入研究,其中单独DAH 19例,单独ILD 96例,DAH合并DAH 18例,NPI 48例。DAH组血清肌酸水平中位数为449µmol/L,显著高于ILD组(123µmol/L, Nemenyi = -35.215, P = 0.045)和DAHILD组(359µmol/L, Nemenyi = -43.609, P = 0.007)。中位随访时间为67个月(范围:1-199个月)。ILD组患者年龄大于DAH组(中位数:69岁vs. 57岁,Nemenji = 43.853, P = 0.005)。DAH和ILD患者具有两种亚型的综合特征,死亡率最高(随访结束时为72.2%)。白细胞计数升高是短期死亡的危险因素(OR = 1.103, 95%CI: 1.008-1.207, P = 0.032);OR = 1.103, 95%CI: 1.026-1.186, 1年P = 0.008)。老年组(HR = 1.044, 95%C7: 1.023-1.066, P <0.001)、心血管系统受累(HR = 2.093, 95%CI: 1.195 ~ 3.665, P = 0.010)、肾功能不佳(HR = 1.001, 95%CI: 1.000 ~ 1.002, P = 0.032)是长期死亡的危险因素。肺部感染(38/54)是主要死亡原因,尤其是ILD患者。49例患者在确诊后一年内出现肺部感染。结论不同肺部受累类型的smpa患者具有完全不同的临床特征。这些亚型可能有不同的发病机制,应分别研究。
{"title":"Characteristics and Prognosis of Microscopic Polyangiitis Patients with Diffuse Alveolar Hemorrhage and Interstitial Lung Disease","authors":"Yu Gu, Ting Zhang, Min Peng, Juhong Shi","doi":"10.24920/004067","DOIUrl":"10.24920/004067","url":null,"abstract":"<div><h3>Objective</h3><p>To evaluate the clinical characteristics and prognostic predictors of patients with diffuse alveolar hemorrhage (DAH) and/or interstitial lung disease (ILD) secondary to microscopic polyangiitis (MPA) in a Chinese general hospital.</p></div><div><h3>Methods</h3><p>We retrospectively reviewed the medical records of MPA patients admitted to internal medicine departments between the year 2002 and 2012. The patients were divided into the ILD, DAH, DAH combined with ILD (DAHILD), and no pulmonary involvement (NPI) groups according to pulmonary involvement patterns. The clinical characteristics at diagnosis were analyzed. The risk factors associated with short-term death and long-term death were identified with Logistic regression and Cox analysis.</p></div><div><h3>Results</h3><p>Of 193 newly diagnosed MPA patients, 181 patients were enrolled in the research, of which 19 had DAH alone, 96 had ILD alone, 18 had DAH and DAH concurrently, and 48 had NPI. The median of serum creatine level in the DAH group was 449 µmol/L, significantly higher than that in the ILD group (123 µmol/L, <em>Nemenyi</em> = -35.215, P = 0.045) and DAHILD group (359 µmol/L, <em>Nemenji</em> = -43.609, <em>P</em> = 0.007). The median follow-up time was 67 (range: 1-199) months. Patients in the ILD group were older than those in the DAH group (median: 69 years <em>vs.</em> 57 years, <em>Nemenji</em> = 43.853, <em>P</em> = 0.005). The patients with both DAH and ILD had combined features of the two subtypes, and the highest mortality (72.2% at the end of follow-up). The elevated white blood cell count was a risk factor for short-term death (<em>OR</em> = 1.103, 95%<em>CI</em>: 1.008-1.207, <em>P</em> = 0.032 for one month; <em>OR</em> = 1.103, 95%<em>CI</em>: 1.026-1.186, <em>P</em> = 0.008 for one year). Old age (<em>HR</em> = 1.044, 95%C7: 1.023-1.066, <em>P</em> < 0.001), cardiovascular system involvement (<em>HR</em> = 2.093, 95%<em>CI</em>: 1.195-3.665, <em>P</em> = 0.010), poor renal function (<em>HR</em> = 1.001, 95%<em>CI</em>: 1.000-1.002, <em>P</em> = 0.032) were risk factors for long-term death. Pulmonary infections (38/54) were the leading causes of death, especially for the patients with ILD. Besides, 49 patients suffered from pulmonary infections in the first year after diagnosis.</p></div><div><h3>Conclusions</h3><p>MPA patients who presented with different pulmonary involvement patterns have completely different clinical features. These subtypes probably have different pathogenesis and should be studied separately.</p></div>","PeriodicalId":35615,"journal":{"name":"Chinese Medical Sciences Journal","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9632720","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Complex coronary heart disease (CHD) has become a hot spot in medicine due to its complex coronary anatomy, variable clinical factors, difficult hemodynamic reconstruction, and limited effect of conservative drug treatment. Identifying complex CHD and selecting optimal treatment methods have become more scientific as revascularization technology has improved, and coronary risk stratification scores have been introduced. SYNTAX and its derivative scores are decision-making tools that quantitatively describe the characteristics of coronary lesions in patients based on their complexity and severity. The SYNTAX and its derivative scores could assist clinicians in rationalizing the selection of hemodynamic reconstruction treatment strategies, and have demonstrated outstanding value in evaluating the prognosis of patients with complex CHD undergoing revascularization treatment. The authors in this article summary the practical application of SYNTAX and its derivative scores in complex CHD in order to deepen the understanding of the relationship between the choice of different revascularization strategies and SYNTAX and its derived scores in complex CHD and provide a further reference for clinical treatment of complex CHD.
{"title":"Application of SYNTAX and its Derivative Scores in the Selection of Revascularization Strategies for Complex Coronary Heart Disease","authors":"Yuxu Zhang , Rongruo Zeng , Ye Yang , Yin Shen","doi":"10.24920/004085","DOIUrl":"10.24920/004085","url":null,"abstract":"<div><p>Complex coronary heart disease (CHD) has become a hot spot in medicine due to its complex coronary anatomy, variable clinical factors, difficult hemodynamic reconstruction, and limited effect of conservative drug treatment. Identifying complex CHD and selecting optimal treatment methods have become more scientific as revascularization technology has improved, and coronary risk stratification scores have been introduced. SYNTAX and its derivative scores are decision-making tools that quantitatively describe the characteristics of coronary lesions in patients based on their complexity and severity. The SYNTAX and its derivative scores could assist clinicians in rationalizing the selection of hemodynamic reconstruction treatment strategies, and have demonstrated outstanding value in evaluating the prognosis of patients with complex CHD undergoing revascularization treatment. The authors in this article summary the practical application of SYNTAX and its derivative scores in complex CHD in order to deepen the understanding of the relationship between the choice of different revascularization strategies and SYNTAX and its derived scores in complex CHD and provide a further reference for clinical treatment of complex CHD.</p></div>","PeriodicalId":35615,"journal":{"name":"Chinese Medical Sciences Journal","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9647483","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Han Li , Lijuan Qian , Nan Xu , Li Huang , Lixing Qiao
Autosomal recessive congenital ichthyosis (ARCI) is characterized by being born as collodion babies, hyperkeratosis, and skin scaling. We described a collodion baby at birth with mild ectropion, eclabium, and syndactyly. Whole exome sequencing showed a compound heterozygous variant c.[S6C>A], p.(Ser19X) and c.[100G>A], p.(Ala34Thr) in the PNPLA1 gene [NM_001145717; exon 1]. The protein encoded by PNPLA1 acts as a unique transacylase that specifically transfers linoleic acid from triglyceride to ω-hydroxy fatty acid in ceramide, thus giving rise to ω-O-acylceramide, a particular class of sphingolipids that is essential for skin barrier function. The variant was located in the patatin core domain of PNPLA1 and resulted in a truncated protein which could disrupt the function of the protein. This case report highhghts a novel compound heterozygous mutation in PNPLA1 identified in a Chinese child.
常染色体隐性先天性鱼鳞病(ARCI)的特点是出生时为胶体婴儿,角化过度和皮肤脱屑。我们描述了一个出生时患有轻度外翻、外翻和并指的胶体婴儿。全外显子组测序结果显示,PNPLA1基因[NM_001145717;外显子1]。由PNPLA1编码的蛋白质作为一种独特的转酰基酶,特异性地将亚油酸从甘油三酯转移到神经酰胺中的ω-羟基脂肪酸,从而产生ω- o -酰基神经酰胺,这是一类特殊的鞘脂,对皮肤屏障功能至关重要。该变异位于PNPLA1的patatin核心区域,导致一个截断的蛋白,可能破坏该蛋白的功能。本病例报告强调了在一名中国儿童中发现的一种新的PNPLA1复合杂合突变。
{"title":"Novel Pathogenic Mutation of PNPLA1 Identified in Autosomal Recessive Congenital Ichthyosis: A Case Report","authors":"Han Li , Lijuan Qian , Nan Xu , Li Huang , Lixing Qiao","doi":"10.24920/004009","DOIUrl":"https://doi.org/10.24920/004009","url":null,"abstract":"<div><p>Autosomal recessive congenital ichthyosis (ARCI) is characterized by being born as collodion babies, hyperkeratosis, and skin scaling. We described a collodion baby at birth with mild ectropion, eclabium, and syndactyly. Whole exome sequencing showed a compound heterozygous variant c.[S6C>A], p.(Ser19X) and c.[100G>A], p.(Ala34Thr) in the <em>PNPLA1</em> gene [NM_001145717; exon 1]. The protein encoded by <em>PNPLA1</em> acts as a unique transacylase that specifically transfers linoleic acid from triglyceride to ω-hydroxy fatty acid in ceramide, thus giving rise to ω-O-acylceramide, a particular class of sphingolipids that is essential for skin barrier function. The variant was located in the patatin core domain of <em>PNPLA1</em> and resulted in a truncated protein which could disrupt the function of the protein. This case report highhghts a novel compound heterozygous mutation in <em>PNPLA1</em> identified in a Chinese child.</p></div>","PeriodicalId":35615,"journal":{"name":"Chinese Medical Sciences Journal","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"92074451","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}