Since the advent of transcatheter aortic valve replacement(TAVR)in 2002,it has now become the default interventional strategy for symptomatic patients presenting with severe aortic stenosis,particularly in intermediat...Since the advent of transcatheter aortic valve replacement(TAVR)in 2002,it has now become the default interventional strategy for symptomatic patients presenting with severe aortic stenosis,particularly in intermediate to highsurgical risk patients.In 2019,the United States Food and Drug Administration approved TAVR in low-risk patients based on two randomized trials.However,these breakthrough trials excluded patients with certain unfavorable anatomies and odd profiles.While currently there is no randomized study of TAVR in young patients,it may be preferred by the young population given the benefits of early discharge,shorter hospital stay,and expedite recovery.Nonetheless,it is important to ruminate various factors including lifetime expectancy,risk of pacemaker implantation,and the need for future valve or coronary interventions in young cohorts before considering TAVR in these patients.Furthermore,the data on long-term durability(>10 years)of TAVR is still unknown given most of the procedures were initially performed in the high or prohibitive surgical risk population.Thus,this editorial aims to highlight the importance of considering an individualized approach in young patients with consideration of various factors including lifetime expectancy while choosing TAVR against surgical aortic valve replacement.展开更多
A notable portion of cachelines in real-world workloads exhibits inner non-uniform access behaviors.However,modern cache management rarely considers this fine-grained feature,which impacts the effective cache capacity...A notable portion of cachelines in real-world workloads exhibits inner non-uniform access behaviors.However,modern cache management rarely considers this fine-grained feature,which impacts the effective cache capacity of contemporary high-performance spacecraft processors.To harness these non-uniform access behaviors,an efficient cache replacement framework featuring an auxiliary cache specifically designed to retain evicted hot data was proposed.This framework reconstructs the cache replacement policy,facilitating data migration between the main cache and the auxiliary cache.Unlike traditional cacheline-granularity policies,the approach excels at identifying and evicting infrequently used data,thereby optimizing cache utilization.The evaluation shows impressive performance improvement,especially on workloads with irregular access patterns.Benefiting from fine granularity,the proposal achieves superior storage efficiency compared with commonly used cache management schemes,providing a potential optimization opportunity for modern resource-constrained processors,such as spacecraft processors.Furthermore,the framework complements existing modern cache replacement policies and can be seamlessly integrated with minimal modifications,enhancing their overall efficacy.展开更多
Mutations in mitochondrial DNA(mtDNA)are maternally inherited and have the potential to cause severe disorders.Mitochondrial replacement therapies,including spindle,polar body,and pronuclear transfers,are promising st...Mutations in mitochondrial DNA(mtDNA)are maternally inherited and have the potential to cause severe disorders.Mitochondrial replacement therapies,including spindle,polar body,and pronuclear transfers,are promising strategies for preventing the hereditary transmission of mtDNA diseases.While pronuclear transfer has been used to generate mitochondrial replacement mouse models and human embryos,its application in non-human primates has not been previously reported.In this study,we successfully generated four healthy cynomolgus monkeys(Macaca fascicularis)via female pronuclear transfer.These individuals all survived for more than two years and exhibited minimal mtDNA carryover(3.8%–6.7%),as well as relatively stable mtDNA heteroplasmy dynamics during development.The successful establishment of this nonhuman primate model highlights the considerable potential of pronuclear transfer in reducing the risk of inherited mtDNA diseases and provides a valuable preclinical research model for advancing mitochondrial replacement therapies in humans.展开更多
Transcatheter aortic valve replacement(TAVR)has emerged as a formidable treatment option for severe symptomatic aortic stenosis ahead of surgical aortic valve replacement.The encouraging results from large randomized ...Transcatheter aortic valve replacement(TAVR)has emerged as a formidable treatment option for severe symptomatic aortic stenosis ahead of surgical aortic valve replacement.The encouraging results from large randomized controlled trials has resulted in an exponential rise in the use of TAVR even in the low-risk patients.However,this is not without challenges.Need for permanent pacemaker(PPM)post-TAVR remains the most frequent and clinically relevant challenge.Naturally,identifying risk factors which predispose an individual to develop high grade conduction block post-TAVR is important.Various demographic factors,electrocardiographic features,anatomic factors and procedural characteristics have all been linked to the development of advanced conduction block and need for PPM following TAVR.Amongst these electrophysiological variables,most notably a prolonged QRS>120 ms regardless of the type of conduction block seems to be one of the strongest predictors on logistic regression models.The index study by Nwaedozie et al highlights that patients requiring PPM post-TAVR had higher odds of having a baseline QRS>120 ms and were more likely to be having diabetes mellitus that those who did not require PPM.展开更多
In this editorial,we comprehensively summarized the preoperative risk factors of early permanent pacemaker implantation after transcatheter aortic valve replacement(TAVR)among patients with severe aortic stenosis from...In this editorial,we comprehensively summarized the preoperative risk factors of early permanent pacemaker implantation after transcatheter aortic valve replacement(TAVR)among patients with severe aortic stenosis from several renowned clinical studies and focused on the primary prevention of managing the modifiable factors,e.g.,paroxysmal atrial fibrillation before the TAVR.展开更多
Pacemaker post-transcatheter aortic valve replacement is related to multifactorial risk.Nwaedozie et al brought to the body of evidence electrocardiogram and clinical findings.However,procedural characteristics have a...Pacemaker post-transcatheter aortic valve replacement is related to multifactorial risk.Nwaedozie et al brought to the body of evidence electrocardiogram and clinical findings.However,procedural characteristics have at least as much impact on the final need for a permanent pacemaker and potentially on the pacing rate.In this regard,long-term follow-up and understanding of the impact of long-term stimulation is of utmost importance.展开更多
BACKGROUND Osteonecrosis or avascular necrosis(AVN)of the hip was one of the dreaded complications of coronavirus disease 2019(COVID-19),which emerged in patients who received steroid therapy.Corticosteroids have been...BACKGROUND Osteonecrosis or avascular necrosis(AVN)of the hip was one of the dreaded complications of coronavirus disease 2019(COVID-19),which emerged in patients who received steroid therapy.Corticosteroids have been a mainstay in the treatment protocol of COVID-19 patients.Popular corticosteroid drugs used in patients suffering from COVID-19 were intravenous(IV)or oral dexamethasone,methylprednisolone or hydrocortisone.The use of such high doses of corticost-eroids has shown very positive results and has been lifesaving in many cases.Still,long-term consequences were drug-induced diabetes,osteoporosis,Cushing syndrome,muscle wasting,peripheral fat mobilization,AVN,hirsutism,sleep disturbances and poor wound healing.A significant number of young patients were admitted for bilateral total hip replacements(THR)secondary to AVN following steroid use for COVID-19 treatment.AIM To assess the efficacy of bilateral pericapsular end nerve group(PENG)blocks in patients posted for bilateral THR post-steroid therapy after COVID-19 infection and assess the time taken to first ambulate after surgery.METHODS This prospective observational study was conducted between January 2023 and August 2023 at Care Hospitals,Hyderabad,India.Twenty young patients 30-35 years of age who underwent bilateral THR were studied after due consent over 8 months.All the patients received spinal anaesthesia for surgery and bilateral PENG blocks for postoperative analgesia.RESULTS The duration of surgery was 2.5 h on average.Seventeen out of twenty patients(85%)had a Visual Analog Score(VAS)of less than 2 and did not require any supplementation.One patient was removed from the study,as he required re-exploration.The remaining two patients had a VAS of more than 8 and received IV morphine post-operatively as a rescue analgesic drug.Fifteen out of seventeen patients(88.2%)could be mobilized 12 h after the procedure.CONCLUSION Osteonecrosis or AVN of the hip was one of the dreaded complications of COVID-19,which surfaced in patients who received steroid therapy requiring surgical intervention.Bilateral PENG block is an effective technique to provide post-operative analgesia resulting in early mobilization and enhanced recovery after surgery.展开更多
BACKGROUND Hemophilia A is a rare inherited bleeding disorder caused by mutations in the factor Ⅷ gene. This clotting factor plays an intrinsic role in the blood coagulation pathway. Patients with hemophilia may deve...BACKGROUND Hemophilia A is a rare inherited bleeding disorder caused by mutations in the factor Ⅷ gene. This clotting factor plays an intrinsic role in the blood coagulation pathway. Patients with hemophilia may develop orthopedic manifestations such as hemarthrosis, but multiple malunion of fractures over the knee is rare and difficult to treat.CASE SUMMARY We report a patient with hemophilia A who developed severe knee osteoarthritis along with fracture malunion and nonunion. Total knee replacement was performed using a custom-made modular hinged knee prosthesis(cemented) equipped with extended distal and proximal stems. At 3 years’ follow-up, the patient exhibited excellent clinical function and remained satisfied with the surgical outcome. Surgical intervention was accompanied by rigorous coagulation factor replacement.CONCLUSION This case highlights various unique scenarios specific to individuals with hemophilia and fracture deformity.展开更多
BACKGROUND Liver transplant(LT)is becoming increasingly common with improved life expectancy.Joint replacement is usually a safe procedure;however,its safety in LT recipients remains understudied.AIM To evaluate the m...BACKGROUND Liver transplant(LT)is becoming increasingly common with improved life expectancy.Joint replacement is usually a safe procedure;however,its safety in LT recipients remains understudied.AIM To evaluate the mortality,outcome,and 90-d readmission rate in LT patients undergoing hip and knee replacement surgery.METHODS Patients with history of LT who underwent hip and knee replacement surgery between 2016 and 2019 were identified using the National Readmission Database.RESULTS A total of 5046119 hip and knee replacement surgeries were identified.3219 patients had prior LT.Mean age of patients with no history of LT was 67.51[95%confidence interval(CI):67.44-67.58],while it was 64.05(95%CI:63.55-64.54)in patients with LT.Patients with history of LT were more likely to have prolonged length of hospital stay(17.1%vs 8.4%,P<0.001).The mortality rate for patients with no history of LT was 0.22%,while it was 0.24%for patients with LT(P=0.792).Patients with history of LT were more likely to have re-admissions within 90 d of initial hospitalization:11.4%as compared to 6.2%in patients without history of LT(P<0.001).The mortality rate between both groups during readmission was not statistically different(1.9%vs 2%,P=0.871)respectively.CONCLUSION Hip and knee replacements in patients with history of LT are not associated with increased mortality;increased readmissions were more frequent in this cohort of patients.Chronic kidney disease and congestive heart failure appear to predict higher risk of readmission.展开更多
With the rapid development of DNA technologies, high throughput genomic data have become a powerful leverage to locate desirable genetic loci associated with traits of importance in various crop species. However, curr...With the rapid development of DNA technologies, high throughput genomic data have become a powerful leverage to locate desirable genetic loci associated with traits of importance in various crop species. However, current genetic association mapping analyses are focused on identifying individual QTLs. This study aimed to identify a set of QTLs or genetic markers, which can capture genetic variability for marker-assisted selection. Selecting a set with k loci that can maximize genetic variation out of high throughput genomic data is a challenging issue. In this study, we proposed an adaptive sequential replacement (ASR) method, which is considered a variant of the sequential replacement (SR) method. Through Monte Carlo simulation and comparing with four other selection methods: exhaustive, SR method, forward, and backward methods we found that the ASR method sustains consistent and repeatable results comparable to the exhaustive method with much reduced computational intensity.展开更多
Background: The Buechel-Pappas (BP) meniscal bearing total ankle replace-ment was initially developed as a “shallow-sulcus” talar component device using cobalt-chromium-molybdenum alloy, in 1978, and later, modified...Background: The Buechel-Pappas (BP) meniscal bearing total ankle replace-ment was initially developed as a “shallow-sulcus” talar component device using cobalt-chromium-molybdenum alloy, in 1978, and later, modified to a “deep-sulcus” talar component device using titanium nitride (TiN) ceramic and porous coating in 1989. Wear related osteolytic cysts were noted in the tibia and talus surrounding these devices that compromised long term fixation and stability when using standard ultra-high molecular weight polyethylene (UHMWPe) as a bearing material. This study explores the use of highly cross-linked UHMWPe (HXLPe) to minimize osteolysis by replacing standard UHMWPe with this more wear-resistant material. Methods: There were 12 primary and 8 revision total ankle replacements followed for 13 to 15 years. HXLPe was used in all meniscal bearings, either as primary or revision implants. All stable metallic tibial and talar components were retained in revision cases. Osteolytic cysts greater than 10 mm in diameter were bone grafted with homologous morselized banked bone through cortical windows in the tibia or talus. No adjuvant screw fixation was used to stabilize any metallic implant. Results: No HXLPe bearings failed in this study, and no re-revisions were performed. No primary total ankle replacement failed in this study, and there were no substantial osteolytic cysts (>2 mm) observed in primary total ankle replacements on plain X-rays. All bone grafted osteolytic cysts in revision ankle replacements remained stable, even though partial resorption of the grafted material was identified in most of the ankles. No loosening of porous coated and TiN coated tibial and talar components were seen;the longest surviving metal components in the revision group was 24 years with the revised bearing at 15 years. Conclusions: HXLPe has greatly improved wear resistance in meniscal-bearing BP total ankle replacements in both primary and revision arthroplasties. Osteolytic cysts can be successfully bone grafted during bearing exchange revisions. Primary and revision, cementless BP metallic total ankle components have remained well-fixed to bone in the long term (greater than 20 years), without the use of adjuvant screw fixation.展开更多
Continuous renal replacement therapy(CRRT)is widely used for treating critically-ill patients in the emergency department in China.Anticoagulant therapy is needed to prevent clotting in the extracorporeal circulation ...Continuous renal replacement therapy(CRRT)is widely used for treating critically-ill patients in the emergency department in China.Anticoagulant therapy is needed to prevent clotting in the extracorporeal circulation during CRRT.Regional citrate anticoagulation(RCA)has been shown to potentially be safer and more effective,and is now recommended as the preferred anticoagulant method for CRRT.However,there is still a lack of unified standards for RCA management in the world,and there are many problems in using this method in clinical practice.The Emergency Medical Doctor Branch of the Chinese Medical Doctor Association(CMDA)organized a panel of domestic emergency medicine experts and international experts of CRRT to discuss RCA-related issues,including the advantages and disadvantages of RCA in CRRT anticoagulation,the principle of RCA,parameter settings for RCA,monitoring of RCA(mainly metabolic acid-base disorders),and special issues during RCA.Based on the latest available research evidence as well as the paneled experts'clinical experience,considering the generalizability,suitability,and potential resource utilization,while also balancing clinical advantages and disadvantages,a total of 16 guideline recommendations were formed from the experts'consensus.展开更多
BACKGROUND Conduction and rhythm abnormalities requiring permanent pacemakers(PPM)are short-term complications following transcatheter aortic valve replacement(TAVR),and their clinical outcomes remain conflicting.Pote...BACKGROUND Conduction and rhythm abnormalities requiring permanent pacemakers(PPM)are short-term complications following transcatheter aortic valve replacement(TAVR),and their clinical outcomes remain conflicting.Potential novel predictors of post-TAVR PPM,like QRS duration,QTc prolongation,and supraventricular arrhythmias,have been poorly studied.AIM To evaluate the effects of baseline nonspecific interventricular conduction delay and supraventricular arrhythmia on post-TAVR PPM requirement and determine the impact of PPM implantation on clinical outcomes.METHODS RESULTS Out of the 357 patients that met inclusion criteria,the mean age was 80 years,188(52.7%)were male,and 57(16%)had a PPM implantation.Baseline demographics,valve type,and cardiovascular risk factors were similar except for type II diabetes mellitus(DM),which was more prevalent in the PPM cohort(59.6%vs 40.7%;P=0.009).The PPM cohort had a significantly higher rate of pre-procedure right bundle branch block,prolonged QRS>120 ms,prolonged QTc>470 ms,and supraventricular arrhythmias.There was a consistently significant increase in the odds ratio(OR)of PPM implantation for every 20 ms increase in the QRS duration above 100 ms:QRS 101-120[OR:2.44;confidence intervals(CI):1.14-5.25;P=0.022],QRS 121-140(OR:3.25;CI:1.32-7.98;P=0.010),QRS 141-160(OR:6.98;CI:3.10-15.61;P<0.001).After model adjustment for baseline risk factors,the OR remained significant for type II DM(aOR:2.16;CI:1.18-3.94;P=0.012),QRS>120(aOR:2.18;CI:1.02-4.66;P=0.045)and marginally significant for supraventricular arrhythmias(aOR:1.82;CI:0.97-3.42;P=0.062).The PPM cohort had a higher adjusted OR of heart failure(HF)hospitalization(aOR:2.2;CI:1.1-4.3;P=0.022)and nonfatal myocardial infarction(MI)(aOR:3.9;CI:1.1-14;P=0.031)without any difference in mortality(aOR:1.1;CI:0.5-2.7;P=0.796)at one year.CONCLUSION Pre-TAVR type II DM and QRS duration>120,regardless of the presence of bundle branch blocks,are predictors of post-TAVR PPM.At 1-year post-TAVR,patients with PPM have higher odds of HF hospitalization and MI.展开更多
BACKGROUND:This study aims to evaluate the eff ect of continuous renal replacement therapy(CRRT)on inflammation-related anemia,iron metabolism,and the prognosis in sepsis patients with acute kidney injury(AKI).METHODS...BACKGROUND:This study aims to evaluate the eff ect of continuous renal replacement therapy(CRRT)on inflammation-related anemia,iron metabolism,and the prognosis in sepsis patients with acute kidney injury(AKI).METHODS:Sepsis patients with AKI were prospectively enrolled and randomized into the CRRT and control groups.The clinical and laboratory data on days 1,3 and 7 after intensive care unit(ICU)admission were collected.The serum interleukin(IL)-6,hepcidin,erythropoietin,ferritin,and soluble transferrin receptor(sTfR)were determined by enzyme-linked immunosorbent assay.The Sequential Organ Failure Assessment(SOFA)score and 28-day mortality were recorded.Data were analyzed using Pearson’s Chi-square test or Fisher’s exact test(categorical variables),and Mann-Whitney U-test or t-test(continuous variables).RESULTS:The hemoglobin and serum erythropoietin levels did not signifi cantly diff er between the CRRT and control groups though gradually decreased within the first week of ICU admission.On days 3 and 7,the serum IL-6,hepcidin,ferritin,and red blood cell distribution width significantly decreased in the CRRT group compared to the control group(all P<0.05).On day 7,the serum iron was significantly elevated in the CRRT group compared to the control group(P<0.05).However,the serum sTfR did not signifi cantly diff er between the groups over time.In addition,the SOFA scores were signifi cantly lower in the CRRT group compared to the control group on day 7.The 28-day mortality did not signifi cantly diff er between the control and CRRT groups(38.0%vs.28.2%,P=0.332).CONCLUSION:CRRT might have beneficial effects on the improvement in inflammationrelated iron metabolism and disease severity during the fi rst week of ICU admission but not anemia and 28-day mortality in sepsis patients with AKI.展开更多
BACKGROUND A femoral neck fracture is a common and frequently reported issue in orthopedics, with a greater rate of incidence among the elderly. Due to their advanced age and the presence of some primary diseases, bot...BACKGROUND A femoral neck fracture is a common and frequently reported issue in orthopedics, with a greater rate of incidence among the elderly. Due to their advanced age and the presence of some primary diseases, both anesthesia and surgery are increasingly difficult in elderly patients with a femoral neck fractures. In fact,general anesthesia can easily induce complications such as cognitive dysfunction,which is not conducive to postoperative recovery.AIM To analyze the efficacy of dexmedetomidine in inducing anesthesia for elderly patients undergoing hip replacement surgery.METHODS A total of 98 elderly patients undergoing hip replacement in our hospital from June 2020 to June 2021 were randomly divided into control group(49 cases) and observation group(49 cases). The control group was given general anesthesia, and the observation group was combined with dexmedetomidine for anesthesia on the basis of the control group. Both groups were observed until the patients were discharged. The vital signs, serum inflammatory factors and renal function indexes of the two groups were compared before, during and 6 h after operation.The postoperative recovery and adverse events of the two groups were statistically analyzed.RESULTS Compared with the mean arterial pressure of the two groups, the intraoperative and postoperative 6 h was higher than that before the operation, the intraoperative was lower than the postoperative 6 h(P < 0.05);the blood oxygen saturation of the two groups was higher than that before operation and 6 h after operation, and the observation group was higher than the control group 6 h after operation(P < 0.05). The heart rate of the two groups was lower during and 6 h after operation than that before operation, and higher at 6 h after operation than that during operation(P < 0.05).The levels of serum C-reactive protein, tumor necrosis factor-α, interleukin-1β and kidney injury molecule-1 in the two groups were higher during operation and 6 h after operation than those before operation(P < 0.05). The level of serum urea nitrogen in the two groups was higher than that before operation, and that in the observation group was lower than that in the control group(P < 0.05). During hospitalization, the first time of getting out of bed, recovery time of grade Ⅱ muscle strength, recovery time of grade Ⅲ muscle strength and hospitalization time in the observation group were shorter than those in the control group(P < 0.05).CONCLUSION Dexmedetomidine can effectively improve the vital signs of elderly patients undergoing hip replacement surgery, reduce the body’s inflammatory response and renal function damage, and promote postoperative recovery. Meanwhile, dexmedetomidine showcased a good safety profile and a good anesthetic outcome.展开更多
BACKGROUND The pathophysiology of Fabry disease(FD)-induced progressive vital organ damage is irreversible.Disease progression can be delayed using enzyme replacement therapy(ERT).In patients with classic FD,sporadic ...BACKGROUND The pathophysiology of Fabry disease(FD)-induced progressive vital organ damage is irreversible.Disease progression can be delayed using enzyme replacement therapy(ERT).In patients with classic FD,sporadic accumulation of globotriaosylceramide(GL-3)in the heart and kidney begins in utero;however,until childhood,GL-3 accumulation is mild and reversible and can be restored by ERT.The current consensus is that ERT initiation during early childhood is paramount.Nonetheless,complete recovery of organs in patients with advanced FD is challenging.CASE SUMMARY Two related male patients,an uncle(patient 1)and nephew(patient 2),presented with classic FD.Both patients were treated by us.Patient 1 was in his 50s,and ERT was initiated following end-organ damage;this was subsequently ineffective.He developed cerebral infarction and died of sudden cardiac arrest.Patient 2 was in his mid-30s,and ERT was initiated when the patient was diagnosed with FD,during which the damage to vital organs was not overtly apparent.Although he had left ventricular hypertrophy at the beginning of this treatment,the degree of hypertrophy progression was limited to a minimal range after>18 years of ERT.CONCLUSION We obtained discouraging ERT outcomes for older patients but encouraging outcomes for younger adults with classic FD.展开更多
BACKGROUND Endoprosthetic distal femoral replacement(DFR)is a well-established salvage procedure following resection of malignant tumors within the distal femur.Use of an all-polyethylene tibial(APT)component is cost-...BACKGROUND Endoprosthetic distal femoral replacement(DFR)is a well-established salvage procedure following resection of malignant tumors within the distal femur.Use of an all-polyethylene tibial(APT)component is cost-effective and avoids failure due to locking-mechanism issues and backside wear,but limits modularity and the option for late liner exchange.Due to a paucity of literature we sought to answer three questions:(1)What are the most common modes of implant failure for patients undergoing cemented DFR with APT for oncologic indications?(2)What is the survivorship,rate of all-cause reoperation,and rate of revision for aseptic loosening of these implants?And(3)Is there a difference in implant survivorship or patient demographics between cemented DFRs with APT performed as a primary reconstruction vs those performed as a revision procedure?AIM To assess outcomes of cemented DFRs with APT components used for oncologic indications.METHODS After Institutional Review Board approval,a retrospective review of consecutive patients who underwent DFR between December 2000 to September 2020 was performed using a single-institutional database.Inclusion criteria consisted of all patients who underwent DFR with a GMRS®(Global Modular Replacement System,Stryker,Kalamazoo,MI,United States)cemented distal femoral endoprosthesis and APT component for an oncologic indication.Patients undergoing DFR for non-oncologic indications and patients with metal-backed tibial components were excluded.Implant failure was recorded using Henderson's classification and survivorship was reported using a competing risks analysis.RESULTS 55 DFRs(55 patients)with an average age of 50.9±20.7 years and average body mass index of 29.7±8.3 kg/m2 were followed for 38.8±54.9 mo(range 0.2-208.4).Of these,60.0%were female and 52.7%were white.The majority of DFRs with APT in this cohort were indicated for oncologic diagnoses of osteogenic sarcoma(n=22,40.0%),giant cell tumor(n=9,16.4%),and metastatic carcinoma(n=8,14.6%).DFR with APT implantation was performed as a primary procedure in 29 patients(52.7%)and a revision procedure in 26 patients(47.3%).Overall,twenty patients(36.4%)experienced a postoperative complication requiring reoperation.The primary modes of implant failure included Henderson Type 1(soft tissue failure,n=6,10.9%),Type 2(aseptic loosening,n=5,9.1%),and Type 4(infection,n=6,10.9%).There were no significant differences in patient demographics or rates of postoperative complications between the primary procedure and revision procedure subgroups.In total,12 patients(21.8%)required a revision while 20 patients(36.4%)required a reoperation,resulting in three-year cumulative incidences of 24.0%(95%CI 9.9%-41.4%)and 47.2%(95%CI 27.5%-64.5%),respectively.CONCLUSION This study demonstrates modest short-term survivorship following cemented DFR with APT components for oncologic indications.Soft tissue failure and endoprosthetic infection were the most common postoperative complications in our cohort.展开更多
Currently,the decision of aortic valve replacement surgery time for asymptomatic patients with moderate-to-severe aortic stenosis(AS)is made by healthcare professionals based on the patient’s clinical biometric recor...Currently,the decision of aortic valve replacement surgery time for asymptomatic patients with moderate-to-severe aortic stenosis(AS)is made by healthcare professionals based on the patient’s clinical biometric records.A delay in surgical aortic valve replacement(SAVR)can potentially affect patients’quality of life.By using ML algorithms,this study aims to predict the optimal SAVR timing and determine the enhancement in moderate-to-severe AS patient survival following surgery.This study represents a novel approach that has the potential to improve decision-making and,ultimately,improve patient outcomes.We analyze data from 176 patients with moderate-to-severe aortic stenosis who had undergone or were indicated for SAVR.We divide the data into two groups:those who died within the first year after SAVR and those who survived for more than one year or were still alive at the last follow-up.We then use six different ML algorithms,Support Vector Machine(SVM),Classification and Regression Tree(C and R tree),Generalized Linear(GL),Chi-Square Automatic Interaction Detector(CHAID),Artificial Neural Net-work(ANN),and Linear Regression(LR),to generate predictions for the best timing for SAVR.The results showed that the SVM algorithm is the best model for predicting the optimal timing for SAVR and for predicting the post-surgery survival period.By optimizing the timing of SAVR surgery using the SVM algorithm,we observed a significant improvement in the survival period after SAVR.Our study demonstrates that ML algorithms generate reliable models for predicting the optimal timing of SAVR in asymptomatic patients with moderate-to-severe AS.展开更多
BACKGROUND Mitral regurgitation(MR)is commonly seen in patients with severe aortic stenosis(AS)undergoing aortic valve replacement(AVR).But the long-term implications of MR in AS are unknown.AIM To investigate MR’s i...BACKGROUND Mitral regurgitation(MR)is commonly seen in patients with severe aortic stenosis(AS)undergoing aortic valve replacement(AVR).But the long-term implications of MR in AS are unknown.AIM To investigate MR’s impact on survival of patients undergoing surgical AVR for severe AS.METHODS Of the 740 consecutive patients with severe AS evaluated between 1993 and 2003,287 underwent AVR forming the study cohort.They were followed up to death or till the end of 2019.Chart reviews were performed for clinical,echocardiographic,and therapeutic data.MR was graded on a 1-4 scale.Mortality data was obtained from chart review and the Social Security Death Index.Survival was analyzed as a function of degree of MR.RESULTS The mean age of the severe AS patients who had AVR(n=287)was 72±13 years,46%women.Over up to 26 years of follow up,there were 201(70%)deaths,giving deep insights into the determinants of survival of severe AS who had AVR.The 5,10 and 20 years survival rates were 75%,45%and 25%respectively.Presence of MR was associated with higher mortality in a graded fashion(P=0.0003).MR was significantly associated with lower left ventricular(LV)ejection fraction and larger LV size.Impact of MR on mortality was partially mediated through lower LV ejection fraction and larger LV size.By Cox regression,MR,lower ejection fraction(EF)and larger LV end-systolic dimension were independent predictors of higher mortality(χ^(2)=33.2).CONCLUSION Presence of greater than 2+MR in patients with severe AS is independently associated with reduced survival in surgically managed patients,an effect incremental to reduced EF and larger LV size.We suggest that aortic valve intervention should be considered in severe AS patients when>2+MR occurs irrespective of EF or symptoms.展开更多
BACKGROUND Atrioventricular block requiring permanent pacemaker(PPM)implantation is an important complication of transcatheter aortic valve replacement(TAVR).Application of machine learning could potentially be used t...BACKGROUND Atrioventricular block requiring permanent pacemaker(PPM)implantation is an important complication of transcatheter aortic valve replacement(TAVR).Application of machine learning could potentially be used to predict preprocedural risk for PPM.AIM To apply machine learning to be used to predict pre-procedural risk for PPM.METHODS A retrospective study of 1200 patients who underwent TAVR(January 2014-December 2017)was performed.964 patients without prior PPM were included for a 30-d analysis and 657 patients without PPM requirement through 30 d were included for a 1-year analysis.After the exclusion of variables with near-zero variance or≥50%missing data,167 variables were included in the random forest gradient boosting algorithm(GBM)optimized using 5-fold cross-validations repeated 10 times.The receiver operator curve(ROC)for the GBM model and PPM risk score models were calculated to predict the risk of PPM at 30 d and 1 year.RESULTS Of 964 patients included in the 30-d analysis without prior PPM,19.6%required PPM post-TAVR.The mean age of patients was 80.9±8.7 years.42.1%were female.Of 657 patients included in the 1-year analysis,the mean age of the patients was 80.7±8.2.Of those,42.6%of patients were female and 26.7%required PPM at 1-year post-TAVR.The area under ROC to predict 30-d and 1-year risk of PPM for the GBM model(0.66 and 0.72)was superior to that of the PPM risk score(0.55 and 0.54)with a P value<0.001.CONCLUSION The GBM model has good discrimination and calibration in identifying patients at high risk of PPM post-TAVR.展开更多
文摘Since the advent of transcatheter aortic valve replacement(TAVR)in 2002,it has now become the default interventional strategy for symptomatic patients presenting with severe aortic stenosis,particularly in intermediate to highsurgical risk patients.In 2019,the United States Food and Drug Administration approved TAVR in low-risk patients based on two randomized trials.However,these breakthrough trials excluded patients with certain unfavorable anatomies and odd profiles.While currently there is no randomized study of TAVR in young patients,it may be preferred by the young population given the benefits of early discharge,shorter hospital stay,and expedite recovery.Nonetheless,it is important to ruminate various factors including lifetime expectancy,risk of pacemaker implantation,and the need for future valve or coronary interventions in young cohorts before considering TAVR in these patients.Furthermore,the data on long-term durability(>10 years)of TAVR is still unknown given most of the procedures were initially performed in the high or prohibitive surgical risk population.Thus,this editorial aims to highlight the importance of considering an individualized approach in young patients with consideration of various factors including lifetime expectancy while choosing TAVR against surgical aortic valve replacement.
文摘A notable portion of cachelines in real-world workloads exhibits inner non-uniform access behaviors.However,modern cache management rarely considers this fine-grained feature,which impacts the effective cache capacity of contemporary high-performance spacecraft processors.To harness these non-uniform access behaviors,an efficient cache replacement framework featuring an auxiliary cache specifically designed to retain evicted hot data was proposed.This framework reconstructs the cache replacement policy,facilitating data migration between the main cache and the auxiliary cache.Unlike traditional cacheline-granularity policies,the approach excels at identifying and evicting infrequently used data,thereby optimizing cache utilization.The evaluation shows impressive performance improvement,especially on workloads with irregular access patterns.Benefiting from fine granularity,the proposal achieves superior storage efficiency compared with commonly used cache management schemes,providing a potential optimization opportunity for modern resource-constrained processors,such as spacecraft processors.Furthermore,the framework complements existing modern cache replacement policies and can be seamlessly integrated with minimal modifications,enhancing their overall efficacy.
基金supported by the National Natural Science Foundation of China (82021001,31825018)National Key Research and Development Program of China (2022YFF0710901)+3 种基金Shanghai Municipal Science and Technology Major Project (2018SHZDZX05)Strategic Priority Research Program of the Chinese Academy of Sciences (XDB32060100)Biological Resources Program of Chinese Academy of Sciences (KFJ-BRP-005)National Science and Technology Innovation 2030 Major Program 2021ZD0200900。
文摘Mutations in mitochondrial DNA(mtDNA)are maternally inherited and have the potential to cause severe disorders.Mitochondrial replacement therapies,including spindle,polar body,and pronuclear transfers,are promising strategies for preventing the hereditary transmission of mtDNA diseases.While pronuclear transfer has been used to generate mitochondrial replacement mouse models and human embryos,its application in non-human primates has not been previously reported.In this study,we successfully generated four healthy cynomolgus monkeys(Macaca fascicularis)via female pronuclear transfer.These individuals all survived for more than two years and exhibited minimal mtDNA carryover(3.8%–6.7%),as well as relatively stable mtDNA heteroplasmy dynamics during development.The successful establishment of this nonhuman primate model highlights the considerable potential of pronuclear transfer in reducing the risk of inherited mtDNA diseases and provides a valuable preclinical research model for advancing mitochondrial replacement therapies in humans.
文摘Transcatheter aortic valve replacement(TAVR)has emerged as a formidable treatment option for severe symptomatic aortic stenosis ahead of surgical aortic valve replacement.The encouraging results from large randomized controlled trials has resulted in an exponential rise in the use of TAVR even in the low-risk patients.However,this is not without challenges.Need for permanent pacemaker(PPM)post-TAVR remains the most frequent and clinically relevant challenge.Naturally,identifying risk factors which predispose an individual to develop high grade conduction block post-TAVR is important.Various demographic factors,electrocardiographic features,anatomic factors and procedural characteristics have all been linked to the development of advanced conduction block and need for PPM following TAVR.Amongst these electrophysiological variables,most notably a prolonged QRS>120 ms regardless of the type of conduction block seems to be one of the strongest predictors on logistic regression models.The index study by Nwaedozie et al highlights that patients requiring PPM post-TAVR had higher odds of having a baseline QRS>120 ms and were more likely to be having diabetes mellitus that those who did not require PPM.
文摘In this editorial,we comprehensively summarized the preoperative risk factors of early permanent pacemaker implantation after transcatheter aortic valve replacement(TAVR)among patients with severe aortic stenosis from several renowned clinical studies and focused on the primary prevention of managing the modifiable factors,e.g.,paroxysmal atrial fibrillation before the TAVR.
文摘Pacemaker post-transcatheter aortic valve replacement is related to multifactorial risk.Nwaedozie et al brought to the body of evidence electrocardiogram and clinical findings.However,procedural characteristics have at least as much impact on the final need for a permanent pacemaker and potentially on the pacing rate.In this regard,long-term follow-up and understanding of the impact of long-term stimulation is of utmost importance.
文摘BACKGROUND Osteonecrosis or avascular necrosis(AVN)of the hip was one of the dreaded complications of coronavirus disease 2019(COVID-19),which emerged in patients who received steroid therapy.Corticosteroids have been a mainstay in the treatment protocol of COVID-19 patients.Popular corticosteroid drugs used in patients suffering from COVID-19 were intravenous(IV)or oral dexamethasone,methylprednisolone or hydrocortisone.The use of such high doses of corticost-eroids has shown very positive results and has been lifesaving in many cases.Still,long-term consequences were drug-induced diabetes,osteoporosis,Cushing syndrome,muscle wasting,peripheral fat mobilization,AVN,hirsutism,sleep disturbances and poor wound healing.A significant number of young patients were admitted for bilateral total hip replacements(THR)secondary to AVN following steroid use for COVID-19 treatment.AIM To assess the efficacy of bilateral pericapsular end nerve group(PENG)blocks in patients posted for bilateral THR post-steroid therapy after COVID-19 infection and assess the time taken to first ambulate after surgery.METHODS This prospective observational study was conducted between January 2023 and August 2023 at Care Hospitals,Hyderabad,India.Twenty young patients 30-35 years of age who underwent bilateral THR were studied after due consent over 8 months.All the patients received spinal anaesthesia for surgery and bilateral PENG blocks for postoperative analgesia.RESULTS The duration of surgery was 2.5 h on average.Seventeen out of twenty patients(85%)had a Visual Analog Score(VAS)of less than 2 and did not require any supplementation.One patient was removed from the study,as he required re-exploration.The remaining two patients had a VAS of more than 8 and received IV morphine post-operatively as a rescue analgesic drug.Fifteen out of seventeen patients(88.2%)could be mobilized 12 h after the procedure.CONCLUSION Osteonecrosis or AVN of the hip was one of the dreaded complications of COVID-19,which surfaced in patients who received steroid therapy requiring surgical intervention.Bilateral PENG block is an effective technique to provide post-operative analgesia resulting in early mobilization and enhanced recovery after surgery.
文摘BACKGROUND Hemophilia A is a rare inherited bleeding disorder caused by mutations in the factor Ⅷ gene. This clotting factor plays an intrinsic role in the blood coagulation pathway. Patients with hemophilia may develop orthopedic manifestations such as hemarthrosis, but multiple malunion of fractures over the knee is rare and difficult to treat.CASE SUMMARY We report a patient with hemophilia A who developed severe knee osteoarthritis along with fracture malunion and nonunion. Total knee replacement was performed using a custom-made modular hinged knee prosthesis(cemented) equipped with extended distal and proximal stems. At 3 years’ follow-up, the patient exhibited excellent clinical function and remained satisfied with the surgical outcome. Surgical intervention was accompanied by rigorous coagulation factor replacement.CONCLUSION This case highlights various unique scenarios specific to individuals with hemophilia and fracture deformity.
文摘BACKGROUND Liver transplant(LT)is becoming increasingly common with improved life expectancy.Joint replacement is usually a safe procedure;however,its safety in LT recipients remains understudied.AIM To evaluate the mortality,outcome,and 90-d readmission rate in LT patients undergoing hip and knee replacement surgery.METHODS Patients with history of LT who underwent hip and knee replacement surgery between 2016 and 2019 were identified using the National Readmission Database.RESULTS A total of 5046119 hip and knee replacement surgeries were identified.3219 patients had prior LT.Mean age of patients with no history of LT was 67.51[95%confidence interval(CI):67.44-67.58],while it was 64.05(95%CI:63.55-64.54)in patients with LT.Patients with history of LT were more likely to have prolonged length of hospital stay(17.1%vs 8.4%,P<0.001).The mortality rate for patients with no history of LT was 0.22%,while it was 0.24%for patients with LT(P=0.792).Patients with history of LT were more likely to have re-admissions within 90 d of initial hospitalization:11.4%as compared to 6.2%in patients without history of LT(P<0.001).The mortality rate between both groups during readmission was not statistically different(1.9%vs 2%,P=0.871)respectively.CONCLUSION Hip and knee replacements in patients with history of LT are not associated with increased mortality;increased readmissions were more frequent in this cohort of patients.Chronic kidney disease and congestive heart failure appear to predict higher risk of readmission.
文摘With the rapid development of DNA technologies, high throughput genomic data have become a powerful leverage to locate desirable genetic loci associated with traits of importance in various crop species. However, current genetic association mapping analyses are focused on identifying individual QTLs. This study aimed to identify a set of QTLs or genetic markers, which can capture genetic variability for marker-assisted selection. Selecting a set with k loci that can maximize genetic variation out of high throughput genomic data is a challenging issue. In this study, we proposed an adaptive sequential replacement (ASR) method, which is considered a variant of the sequential replacement (SR) method. Through Monte Carlo simulation and comparing with four other selection methods: exhaustive, SR method, forward, and backward methods we found that the ASR method sustains consistent and repeatable results comparable to the exhaustive method with much reduced computational intensity.
文摘Background: The Buechel-Pappas (BP) meniscal bearing total ankle replace-ment was initially developed as a “shallow-sulcus” talar component device using cobalt-chromium-molybdenum alloy, in 1978, and later, modified to a “deep-sulcus” talar component device using titanium nitride (TiN) ceramic and porous coating in 1989. Wear related osteolytic cysts were noted in the tibia and talus surrounding these devices that compromised long term fixation and stability when using standard ultra-high molecular weight polyethylene (UHMWPe) as a bearing material. This study explores the use of highly cross-linked UHMWPe (HXLPe) to minimize osteolysis by replacing standard UHMWPe with this more wear-resistant material. Methods: There were 12 primary and 8 revision total ankle replacements followed for 13 to 15 years. HXLPe was used in all meniscal bearings, either as primary or revision implants. All stable metallic tibial and talar components were retained in revision cases. Osteolytic cysts greater than 10 mm in diameter were bone grafted with homologous morselized banked bone through cortical windows in the tibia or talus. No adjuvant screw fixation was used to stabilize any metallic implant. Results: No HXLPe bearings failed in this study, and no re-revisions were performed. No primary total ankle replacement failed in this study, and there were no substantial osteolytic cysts (>2 mm) observed in primary total ankle replacements on plain X-rays. All bone grafted osteolytic cysts in revision ankle replacements remained stable, even though partial resorption of the grafted material was identified in most of the ankles. No loosening of porous coated and TiN coated tibial and talar components were seen;the longest surviving metal components in the revision group was 24 years with the revised bearing at 15 years. Conclusions: HXLPe has greatly improved wear resistance in meniscal-bearing BP total ankle replacements in both primary and revision arthroplasties. Osteolytic cysts can be successfully bone grafted during bearing exchange revisions. Primary and revision, cementless BP metallic total ankle components have remained well-fixed to bone in the long term (greater than 20 years), without the use of adjuvant screw fixation.
文摘Continuous renal replacement therapy(CRRT)is widely used for treating critically-ill patients in the emergency department in China.Anticoagulant therapy is needed to prevent clotting in the extracorporeal circulation during CRRT.Regional citrate anticoagulation(RCA)has been shown to potentially be safer and more effective,and is now recommended as the preferred anticoagulant method for CRRT.However,there is still a lack of unified standards for RCA management in the world,and there are many problems in using this method in clinical practice.The Emergency Medical Doctor Branch of the Chinese Medical Doctor Association(CMDA)organized a panel of domestic emergency medicine experts and international experts of CRRT to discuss RCA-related issues,including the advantages and disadvantages of RCA in CRRT anticoagulation,the principle of RCA,parameter settings for RCA,monitoring of RCA(mainly metabolic acid-base disorders),and special issues during RCA.Based on the latest available research evidence as well as the paneled experts'clinical experience,considering the generalizability,suitability,and potential resource utilization,while also balancing clinical advantages and disadvantages,a total of 16 guideline recommendations were formed from the experts'consensus.
文摘BACKGROUND Conduction and rhythm abnormalities requiring permanent pacemakers(PPM)are short-term complications following transcatheter aortic valve replacement(TAVR),and their clinical outcomes remain conflicting.Potential novel predictors of post-TAVR PPM,like QRS duration,QTc prolongation,and supraventricular arrhythmias,have been poorly studied.AIM To evaluate the effects of baseline nonspecific interventricular conduction delay and supraventricular arrhythmia on post-TAVR PPM requirement and determine the impact of PPM implantation on clinical outcomes.METHODS RESULTS Out of the 357 patients that met inclusion criteria,the mean age was 80 years,188(52.7%)were male,and 57(16%)had a PPM implantation.Baseline demographics,valve type,and cardiovascular risk factors were similar except for type II diabetes mellitus(DM),which was more prevalent in the PPM cohort(59.6%vs 40.7%;P=0.009).The PPM cohort had a significantly higher rate of pre-procedure right bundle branch block,prolonged QRS>120 ms,prolonged QTc>470 ms,and supraventricular arrhythmias.There was a consistently significant increase in the odds ratio(OR)of PPM implantation for every 20 ms increase in the QRS duration above 100 ms:QRS 101-120[OR:2.44;confidence intervals(CI):1.14-5.25;P=0.022],QRS 121-140(OR:3.25;CI:1.32-7.98;P=0.010),QRS 141-160(OR:6.98;CI:3.10-15.61;P<0.001).After model adjustment for baseline risk factors,the OR remained significant for type II DM(aOR:2.16;CI:1.18-3.94;P=0.012),QRS>120(aOR:2.18;CI:1.02-4.66;P=0.045)and marginally significant for supraventricular arrhythmias(aOR:1.82;CI:0.97-3.42;P=0.062).The PPM cohort had a higher adjusted OR of heart failure(HF)hospitalization(aOR:2.2;CI:1.1-4.3;P=0.022)and nonfatal myocardial infarction(MI)(aOR:3.9;CI:1.1-14;P=0.031)without any difference in mortality(aOR:1.1;CI:0.5-2.7;P=0.796)at one year.CONCLUSION Pre-TAVR type II DM and QRS duration>120,regardless of the presence of bundle branch blocks,are predictors of post-TAVR PPM.At 1-year post-TAVR,patients with PPM have higher odds of HF hospitalization and MI.
基金funded by the Shenzhen Key Medical Discipline Construction Fund(S ZXK046)the National Nature Science Foundation of China(81571869).
文摘BACKGROUND:This study aims to evaluate the eff ect of continuous renal replacement therapy(CRRT)on inflammation-related anemia,iron metabolism,and the prognosis in sepsis patients with acute kidney injury(AKI).METHODS:Sepsis patients with AKI were prospectively enrolled and randomized into the CRRT and control groups.The clinical and laboratory data on days 1,3 and 7 after intensive care unit(ICU)admission were collected.The serum interleukin(IL)-6,hepcidin,erythropoietin,ferritin,and soluble transferrin receptor(sTfR)were determined by enzyme-linked immunosorbent assay.The Sequential Organ Failure Assessment(SOFA)score and 28-day mortality were recorded.Data were analyzed using Pearson’s Chi-square test or Fisher’s exact test(categorical variables),and Mann-Whitney U-test or t-test(continuous variables).RESULTS:The hemoglobin and serum erythropoietin levels did not signifi cantly diff er between the CRRT and control groups though gradually decreased within the first week of ICU admission.On days 3 and 7,the serum IL-6,hepcidin,ferritin,and red blood cell distribution width significantly decreased in the CRRT group compared to the control group(all P<0.05).On day 7,the serum iron was significantly elevated in the CRRT group compared to the control group(P<0.05).However,the serum sTfR did not signifi cantly diff er between the groups over time.In addition,the SOFA scores were signifi cantly lower in the CRRT group compared to the control group on day 7.The 28-day mortality did not signifi cantly diff er between the control and CRRT groups(38.0%vs.28.2%,P=0.332).CONCLUSION:CRRT might have beneficial effects on the improvement in inflammationrelated iron metabolism and disease severity during the fi rst week of ICU admission but not anemia and 28-day mortality in sepsis patients with AKI.
文摘BACKGROUND A femoral neck fracture is a common and frequently reported issue in orthopedics, with a greater rate of incidence among the elderly. Due to their advanced age and the presence of some primary diseases, both anesthesia and surgery are increasingly difficult in elderly patients with a femoral neck fractures. In fact,general anesthesia can easily induce complications such as cognitive dysfunction,which is not conducive to postoperative recovery.AIM To analyze the efficacy of dexmedetomidine in inducing anesthesia for elderly patients undergoing hip replacement surgery.METHODS A total of 98 elderly patients undergoing hip replacement in our hospital from June 2020 to June 2021 were randomly divided into control group(49 cases) and observation group(49 cases). The control group was given general anesthesia, and the observation group was combined with dexmedetomidine for anesthesia on the basis of the control group. Both groups were observed until the patients were discharged. The vital signs, serum inflammatory factors and renal function indexes of the two groups were compared before, during and 6 h after operation.The postoperative recovery and adverse events of the two groups were statistically analyzed.RESULTS Compared with the mean arterial pressure of the two groups, the intraoperative and postoperative 6 h was higher than that before the operation, the intraoperative was lower than the postoperative 6 h(P < 0.05);the blood oxygen saturation of the two groups was higher than that before operation and 6 h after operation, and the observation group was higher than the control group 6 h after operation(P < 0.05). The heart rate of the two groups was lower during and 6 h after operation than that before operation, and higher at 6 h after operation than that during operation(P < 0.05).The levels of serum C-reactive protein, tumor necrosis factor-α, interleukin-1β and kidney injury molecule-1 in the two groups were higher during operation and 6 h after operation than those before operation(P < 0.05). The level of serum urea nitrogen in the two groups was higher than that before operation, and that in the observation group was lower than that in the control group(P < 0.05). During hospitalization, the first time of getting out of bed, recovery time of grade Ⅱ muscle strength, recovery time of grade Ⅲ muscle strength and hospitalization time in the observation group were shorter than those in the control group(P < 0.05).CONCLUSION Dexmedetomidine can effectively improve the vital signs of elderly patients undergoing hip replacement surgery, reduce the body’s inflammatory response and renal function damage, and promote postoperative recovery. Meanwhile, dexmedetomidine showcased a good safety profile and a good anesthetic outcome.
基金Supported by the Red Cross Hospital Research and Training Fund,Fukushima R.C.Hosp.No.57.
文摘BACKGROUND The pathophysiology of Fabry disease(FD)-induced progressive vital organ damage is irreversible.Disease progression can be delayed using enzyme replacement therapy(ERT).In patients with classic FD,sporadic accumulation of globotriaosylceramide(GL-3)in the heart and kidney begins in utero;however,until childhood,GL-3 accumulation is mild and reversible and can be restored by ERT.The current consensus is that ERT initiation during early childhood is paramount.Nonetheless,complete recovery of organs in patients with advanced FD is challenging.CASE SUMMARY Two related male patients,an uncle(patient 1)and nephew(patient 2),presented with classic FD.Both patients were treated by us.Patient 1 was in his 50s,and ERT was initiated following end-organ damage;this was subsequently ineffective.He developed cerebral infarction and died of sudden cardiac arrest.Patient 2 was in his mid-30s,and ERT was initiated when the patient was diagnosed with FD,during which the damage to vital organs was not overtly apparent.Although he had left ventricular hypertrophy at the beginning of this treatment,the degree of hypertrophy progression was limited to a minimal range after>18 years of ERT.CONCLUSION We obtained discouraging ERT outcomes for older patients but encouraging outcomes for younger adults with classic FD.
文摘BACKGROUND Endoprosthetic distal femoral replacement(DFR)is a well-established salvage procedure following resection of malignant tumors within the distal femur.Use of an all-polyethylene tibial(APT)component is cost-effective and avoids failure due to locking-mechanism issues and backside wear,but limits modularity and the option for late liner exchange.Due to a paucity of literature we sought to answer three questions:(1)What are the most common modes of implant failure for patients undergoing cemented DFR with APT for oncologic indications?(2)What is the survivorship,rate of all-cause reoperation,and rate of revision for aseptic loosening of these implants?And(3)Is there a difference in implant survivorship or patient demographics between cemented DFRs with APT performed as a primary reconstruction vs those performed as a revision procedure?AIM To assess outcomes of cemented DFRs with APT components used for oncologic indications.METHODS After Institutional Review Board approval,a retrospective review of consecutive patients who underwent DFR between December 2000 to September 2020 was performed using a single-institutional database.Inclusion criteria consisted of all patients who underwent DFR with a GMRS®(Global Modular Replacement System,Stryker,Kalamazoo,MI,United States)cemented distal femoral endoprosthesis and APT component for an oncologic indication.Patients undergoing DFR for non-oncologic indications and patients with metal-backed tibial components were excluded.Implant failure was recorded using Henderson's classification and survivorship was reported using a competing risks analysis.RESULTS 55 DFRs(55 patients)with an average age of 50.9±20.7 years and average body mass index of 29.7±8.3 kg/m2 were followed for 38.8±54.9 mo(range 0.2-208.4).Of these,60.0%were female and 52.7%were white.The majority of DFRs with APT in this cohort were indicated for oncologic diagnoses of osteogenic sarcoma(n=22,40.0%),giant cell tumor(n=9,16.4%),and metastatic carcinoma(n=8,14.6%).DFR with APT implantation was performed as a primary procedure in 29 patients(52.7%)and a revision procedure in 26 patients(47.3%).Overall,twenty patients(36.4%)experienced a postoperative complication requiring reoperation.The primary modes of implant failure included Henderson Type 1(soft tissue failure,n=6,10.9%),Type 2(aseptic loosening,n=5,9.1%),and Type 4(infection,n=6,10.9%).There were no significant differences in patient demographics or rates of postoperative complications between the primary procedure and revision procedure subgroups.In total,12 patients(21.8%)required a revision while 20 patients(36.4%)required a reoperation,resulting in three-year cumulative incidences of 24.0%(95%CI 9.9%-41.4%)and 47.2%(95%CI 27.5%-64.5%),respectively.CONCLUSION This study demonstrates modest short-term survivorship following cemented DFR with APT components for oncologic indications.Soft tissue failure and endoprosthetic infection were the most common postoperative complications in our cohort.
文摘Currently,the decision of aortic valve replacement surgery time for asymptomatic patients with moderate-to-severe aortic stenosis(AS)is made by healthcare professionals based on the patient’s clinical biometric records.A delay in surgical aortic valve replacement(SAVR)can potentially affect patients’quality of life.By using ML algorithms,this study aims to predict the optimal SAVR timing and determine the enhancement in moderate-to-severe AS patient survival following surgery.This study represents a novel approach that has the potential to improve decision-making and,ultimately,improve patient outcomes.We analyze data from 176 patients with moderate-to-severe aortic stenosis who had undergone or were indicated for SAVR.We divide the data into two groups:those who died within the first year after SAVR and those who survived for more than one year or were still alive at the last follow-up.We then use six different ML algorithms,Support Vector Machine(SVM),Classification and Regression Tree(C and R tree),Generalized Linear(GL),Chi-Square Automatic Interaction Detector(CHAID),Artificial Neural Net-work(ANN),and Linear Regression(LR),to generate predictions for the best timing for SAVR.The results showed that the SVM algorithm is the best model for predicting the optimal timing for SAVR and for predicting the post-surgery survival period.By optimizing the timing of SAVR surgery using the SVM algorithm,we observed a significant improvement in the survival period after SAVR.Our study demonstrates that ML algorithms generate reliable models for predicting the optimal timing of SAVR in asymptomatic patients with moderate-to-severe AS.
文摘BACKGROUND Mitral regurgitation(MR)is commonly seen in patients with severe aortic stenosis(AS)undergoing aortic valve replacement(AVR).But the long-term implications of MR in AS are unknown.AIM To investigate MR’s impact on survival of patients undergoing surgical AVR for severe AS.METHODS Of the 740 consecutive patients with severe AS evaluated between 1993 and 2003,287 underwent AVR forming the study cohort.They were followed up to death or till the end of 2019.Chart reviews were performed for clinical,echocardiographic,and therapeutic data.MR was graded on a 1-4 scale.Mortality data was obtained from chart review and the Social Security Death Index.Survival was analyzed as a function of degree of MR.RESULTS The mean age of the severe AS patients who had AVR(n=287)was 72±13 years,46%women.Over up to 26 years of follow up,there were 201(70%)deaths,giving deep insights into the determinants of survival of severe AS who had AVR.The 5,10 and 20 years survival rates were 75%,45%and 25%respectively.Presence of MR was associated with higher mortality in a graded fashion(P=0.0003).MR was significantly associated with lower left ventricular(LV)ejection fraction and larger LV size.Impact of MR on mortality was partially mediated through lower LV ejection fraction and larger LV size.By Cox regression,MR,lower ejection fraction(EF)and larger LV end-systolic dimension were independent predictors of higher mortality(χ^(2)=33.2).CONCLUSION Presence of greater than 2+MR in patients with severe AS is independently associated with reduced survival in surgically managed patients,an effect incremental to reduced EF and larger LV size.We suggest that aortic valve intervention should be considered in severe AS patients when>2+MR occurs irrespective of EF or symptoms.
基金funded by Mayo Clinic Arizona Cardiovascular Clinical Research Center (MCA CV CRC)
文摘BACKGROUND Atrioventricular block requiring permanent pacemaker(PPM)implantation is an important complication of transcatheter aortic valve replacement(TAVR).Application of machine learning could potentially be used to predict preprocedural risk for PPM.AIM To apply machine learning to be used to predict pre-procedural risk for PPM.METHODS A retrospective study of 1200 patients who underwent TAVR(January 2014-December 2017)was performed.964 patients without prior PPM were included for a 30-d analysis and 657 patients without PPM requirement through 30 d were included for a 1-year analysis.After the exclusion of variables with near-zero variance or≥50%missing data,167 variables were included in the random forest gradient boosting algorithm(GBM)optimized using 5-fold cross-validations repeated 10 times.The receiver operator curve(ROC)for the GBM model and PPM risk score models were calculated to predict the risk of PPM at 30 d and 1 year.RESULTS Of 964 patients included in the 30-d analysis without prior PPM,19.6%required PPM post-TAVR.The mean age of patients was 80.9±8.7 years.42.1%were female.Of 657 patients included in the 1-year analysis,the mean age of the patients was 80.7±8.2.Of those,42.6%of patients were female and 26.7%required PPM at 1-year post-TAVR.The area under ROC to predict 30-d and 1-year risk of PPM for the GBM model(0.66 and 0.72)was superior to that of the PPM risk score(0.55 and 0.54)with a P value<0.001.CONCLUSION The GBM model has good discrimination and calibration in identifying patients at high risk of PPM post-TAVR.
