Uncertainty is an important factor that needs to be considered while analyzing the performance of any engineering system.In order to quantify uncertainty,fuzzy set theory is frequently used by most of researchers,incl...Uncertainty is an important factor that needs to be considered while analyzing the performance of any engineering system.In order to quantify uncertainty,fuzzy set theory is frequently used by most of researchers,including energy system experts.According to the classical reliability theory,component lifetimes have crisp parameters,but due to uncertainty and inaccuracy in data,it is sometimes very difficult to determine the exact values of these parameters in real-world systems.To overcome this difficulty in the current research,failure and repair rates were taken as triangular fuzzy numbers to determine the fuzzy availability of a system undergoing calendar-based periodic inspection subject to multiple failure modes(FMs).It was assumed that each component in the system had an exponential failure rate and repair rate with fuzzy parameters.System FMs were explicitly taken into account when a functional state of the system was considered.Each FM had a random failure time.On the occurrence of any failure,a random time was selected for the relevant corrective repair work.The proposed research was studied for one of the major sources of green energy,namely a wind turbine system wherein all the derived propositions have been implemented on it.展开更多
BACKGROUND The introduction of pegaspargase has greatly advanced the treatment of acute lymphoblastic leukemia(ALL).In the literature,only one case of pegaspargaseinduced multiple organ failure has been reported,and t...BACKGROUND The introduction of pegaspargase has greatly advanced the treatment of acute lymphoblastic leukemia(ALL).In the literature,only one case of pegaspargaseinduced multiple organ failure has been reported,and the patient died due to multiple organ failure.CASE SUMMARY Herein,we present a rare case of a 40-year-old man with ALL who developed multiple organ failure after treatment with pegaspargase.The patient had two rare phenomena reflecting poor prognosis,including the discrepancy between clinical manifestations and liver function and persistently low alpha-fetoprotein(AFP)levels from subacute liver failure.However,the patient was successfully treated using a multidisciplinary team approach.CONCLUSION This is the first case report of successful treatment of pegaspargase-induced multiple organ failure.The findings emphasize the importance of a multidisciplinary team approach in treating pegaspargase-induced multiple organ failure.展开更多
In this work,heat treatment experiments at 1050 and 1100℃were carried out on single-crystal su-peralloy specimens coated with the electron beam physical vapor deposition(EB-PVD)thermal barrier coating(TBC)system.Furt...In this work,heat treatment experiments at 1050 and 1100℃were carried out on single-crystal su-peralloy specimens coated with the electron beam physical vapor deposition(EB-PVD)thermal barrier coating(TBC)system.Furthermore,the evolution of microstructural characteristics and sintering-induced mechanical properties were separately obtained by the field emission scanning electron microscope(FE-SEM)and nanoindenter,providing inputs for damage modeling.Meanwhile,the critical compressive strain of TBCs at room temperature was acquired using 3D digital image correlation(3D-DIC)technology to characterize the interfacial damage combined with the experimentally observed buckling modes.The re-sults demonstrate that not only does oxidative damage exist in the TBCs system due to thermally grown oxide(TGO)growth,but additional damage is generated by thermal cycling and sintering behavior,re-spectively.Then,a nonlinear cumulative interfacial damage model considering multiple failure factors is developed to predict TBCs’life.The error between the measured damage and calculated damage is less than 15%,showing good prediction accuracy.展开更多
BACKGROUND Multiple acyl-CoA dehydrogenase deficiency(MADD)is a disease of rare autosomal recessive disorder.There are three types of MADD.Type I is a neonatalonset form with congenital anomalies.Type II is a neonatal...BACKGROUND Multiple acyl-CoA dehydrogenase deficiency(MADD)is a disease of rare autosomal recessive disorder.There are three types of MADD.Type I is a neonatalonset form with congenital anomalies.Type II is a neonatal-onset form without congenital anomalies.Type III is considered to a milder form and usually responds to riboflavin.However,late-onset form could also be fatal and not responsive to treatments.CASE SUMMARY We report a severe case of a young man with onset type III MADD induced by drugs and strenuous exercise characterized by rhabdomyolysis and liver dysfunction.Urine analysis indicated 12 out of 70 kinds of organic acids like glutaric acid-2 were detected.Serum analysis in genetic metabolic diseases revealed 24 out of 43 tested items were abnormal,revealing the elevation of several acylcarnitines and the reduction of carnitine in the patient.By next generation sequencing technology for gene sequencing related to fatty acid oxidation and carnitine cycle defects,a rare ETFDH gene variant was identified:NM_004453:4:C.1448C>T(p.Pro483 Leu).The patient was diagnosed with lateonset GAII.He was not responsive to riboflavin and progressively worsened into multiple organ failure that finally led to death.CONCLUSION Type III MADD can also be fatal and not responsive to treatments.展开更多
Combining the advantages of the stratified sampling and the importance sampling, a stratified importance sampling method (SISM) is presented to analyze the reliability sensitivity for structure with multiple failure...Combining the advantages of the stratified sampling and the importance sampling, a stratified importance sampling method (SISM) is presented to analyze the reliability sensitivity for structure with multiple failure modes. In the presented method, the variable space is divided into several disjoint subspace by n-dimensional coordinate planes at the mean point of the random vec- tor, and the importance sampling functions in the subspaces are constructed by keeping the sampling center at the mean point and augmenting the standard deviation by a factor of 2. The sample size generated from the importance sampling function in each subspace is determined by the contribution of the subspace to the reliability sensitivity, which can be estimated by iterative simulation in the sampling process. The formulae of the reliability sensitivity estimation, the variance and the coefficient of variation are derived for the presented SISM. Comparing with the Monte Carlo method, the stratified sampling method and the importance sampling method, the presented SISM has wider applicability and higher calculation efficiency, which is demonstrated by numerical examples. Finally, the reliability sensitivity analysis of flap structure is illustrated that the SISM can be applied to engineering structure.展开更多
The effects of early hemofiltration on the serum levels of cytokines, pro- and anti-inflammatory balance and organ function in pigs with severe acute pancreatits (SAP) were studied. SAP pig model was induced by retrog...The effects of early hemofiltration on the serum levels of cytokines, pro- and anti-inflammatory balance and organ function in pigs with severe acute pancreatits (SAP) were studied. SAP pig model was induced by retrograde injection of artificial bile into the pancreatic duct. The pigs were randomly divided into SAP hemofiltration treatment group (HF group, n=8) and SAP non-hemofiltration treatment group (NHF group, n=8). In the HF group, the animals were subjected to high-volume and zero-balance hemofiltration therapy. The results showed that as compared with NHF group, MAP, CVP and PaO 2/FiO 2 were significantly increased (P<0.01), while HR, urinary protein content, serum ALT level, pulmonary coefficient and lung wet/dry ratio obviously decreased (P<0.05) in HF group. Under a light microscope, the pulmonary histologic scoring was lower that in HF group (P<0.01) and the lesions of renal and liver tissues were milder. However, there was no significant difference in the pancreatic histologic scoring between the two groups. Six h after establishment of the model, the serum levels of TNF-α, IL-1β were lower, while the IL-10/TNF-α ratio was higher in HF group (all P<0.05). It was suggested that early hemofiltration could effectively remove the serum cytokines TNF-α and IL-1β in SAP pigs, elevate the ratio of IL-10/TNF-α, improve hemodynamics and alleviate the lesions of lung, kidney and liver tissues.展开更多
This paper presents a procedure to develop fragility curves of structures equipped with TMD considering multiple failure functions.The failure criteria considered are maximum inter-story drift ratio as a safety criter...This paper presents a procedure to develop fragility curves of structures equipped with TMD considering multiple failure functions.The failure criteria considered are maximum inter-story drift ratio as a safety criterion,maximum absolute acceleration as a convenience criterion and TMD stroke length.The relationship between intensity measure and responses of the structure was assumed to follow the power-law model,and a regression analysis was used to estimate its properties.A nonlinear eight-story shear building subjected to near-fault earthquakes was used for the numerical studies.Fragility curves using multiple and single failure functions for an uncontrolled structure and a structure equipped with optimal TMDs were developed.Numerical analysis showed that using multiple failure functions led to increasing the fragility when compared with using the single failure function for both the uncontrolled and controlled structures.However,TMDs slightly reduced the seismic fragility and have the capability to improve the reliability of the structure.Also,it was found that the fragility was significantly influenced by the values of the capacity thresholds of both the acceleration of the structure and TMD stroke length,which should be selected by considering the target performance and application of the structure and control device.展开更多
TanshinoneⅡA(TanⅡA)is a noteworthy lipophilic diterpene compound derived from the dried roots of the Traditional Chinese Medicine Danshen(Radix Salviae Miltiorrhizae)that has various pharmacological properties,inclu...TanshinoneⅡA(TanⅡA)is a noteworthy lipophilic diterpene compound derived from the dried roots of the Traditional Chinese Medicine Danshen(Radix Salviae Miltiorrhizae)that has various pharmacological properties,including anti-inflammatory,antibacterial,and antioxidative effects.Sepsis is a life-threatening organ dysfunction induced by a dysregulated host response to infection.Recently,increasing attention has been paid to sepsis-induced dysfunction of the intestine,cardiovascular system,lungs,kidneys,liver,and other organs.Experimental studies have shown that TanⅡA has therapeutic potential for sepsis-induced organ dysfunction owing to its anti-inflammatory,anti-apoptotic and regulatory effects on multiple signalling pathways.The purpose of this article is to evaluate the potential multiorgan protective effects of TanⅡA in sepsis.展开更多
BACKGROUND Adult-onset Still’s disease(AOSD)is a rare systemic inflammatory disease,which is characterized by daily fever and arthritis,with an evanescent rash and neutrophilic leukocytosis.To date,there has been no ...BACKGROUND Adult-onset Still’s disease(AOSD)is a rare systemic inflammatory disease,which is characterized by daily fever and arthritis,with an evanescent rash and neutrophilic leukocytosis.To date,there has been no definite laboratory or imaging test available for diagnosing AOSD;the diagnosis is one of exclusion,which can be very challenging.In particular,AOSD patients may experience different complications affecting their clinical picture,management,and prognosis.The treatment of AOSD remains largely empirical and involves therapeutic agents.CASE SUMMARY We report the case of a 36-year-old woman who presented with fever,red rash,arthralgia,and sore throat.Her serum ferritin level and white blood cell count were markedly elevated,and the first diagnosis 22 years prior was"juvenile rheumatoid arthritis of systemic type".The patient was treated with prednisone,sulfasalazine,methotrexate,and leflunomide.After remission of her symptoms,the patient stopped taking the medications,and the disease recurred.Ultimately,the patient was diagnosed with adult-onset Still's disease.Relapse occurred several times due to self-medication withdrawal,and an interleukin-6 antagonist(tocilizumab/Actemra)was administered to control the disease.Recently,she was hospitalized because an incision did not heal,and the patient suddenly developed high fever and diarrhea during hospitalization.The patient's disease progressed violently and quickly developed into macrophage activation syndrome,disseminated intravascular coagulation,shock,and multiple organ failure.The patient had sudden cardiac arrest,and she died despite emergency rescue efforts.CONCLUSION AOSD patients need regular follow-up in the long-term treatment process,and must press formulary standard medication,and do not voluntarily withdraw or reduce the dose.Otherwise it may cause disease back-and-forth or serious lifethreatening complications.Meanwhile,strict management of trauma,infections,tumors,and other diseases may contribute to improved outcomes in patients with complications.展开更多
With control using redundant multiple control surface arrangement and large-deflection drag rudders,a combat flying wing has a higher probability for control surface failures.Therefore,its flight control system must b...With control using redundant multiple control surface arrangement and large-deflection drag rudders,a combat flying wing has a higher probability for control surface failures.Therefore,its flight control system must be able to reconfigure after such failures.Considering three types of typical control surface failures(lock-in-place(LIP),loss-of-effectiveness(LOE) and float),flight control reconfiguration characteristic and capability of such aircraft types are analyzed.Because of the control surface redundancy,the aircraft using the dynamic inversion flight control law already has a control allocation block.In this paper,its flight control configuration during the above failures is achieved by modifying this block.It is shown that such a reconfigurable flight control design is valid,through numerical simulations of flight attitude control task.Results indicate that,in the circumstances of control surface failures with limited degree and the degradation of the flying quality level,a combat flying wing adopting this flight control reconfiguration approach based on control allocation could guarantee its flight safety and perform some flight combat missions.展开更多
BACKGROUND We report a rare case of full neurological recovery from severe nonexertional heat stroke in a 67-year-old woman with an initial Glasgow Coma Scale of 3.This report raises awareness among doctors that when ...BACKGROUND We report a rare case of full neurological recovery from severe nonexertional heat stroke in a 67-year-old woman with an initial Glasgow Coma Scale of 3.This report raises awareness among doctors that when heatstroke is diagnosed,comprehensive treatment should be implemented as soon as possible.Moreover,targeted temperature management,combination therapy with hemodialysis and hemoperfusion,and hyperbaric oxygen therapy may alleviate multiorgan failure and prevent neurological sequelae caused by heatstroke.CASE SUMMARY A previously healthy 67-year-old woman with an initial Glasgow Coma Scale of 3 was found lying prone on the road at noon on a summer day.Laboratory tests revealed multiorgan failure.As soon as heatstroke was diagnosed,comprehensive treatment was implemented.On hospital Day 3,the patient was extubated.Her initial Sequential Organ Failure Assessment score at hospitalization was 14 and decreased to 2 on hospital Day 4.On the seventh day following hospital admission,as the patient’s general condition improved,the levels of laboratory test findings decreased rapidly.Finally,the patient gradually recovered with no other neurological symptoms(the Glasgow Coma Scale at discharge was 15,and her ability to walk independently was restored).CONCLUSION This case demonstrated that targeted temperature management,combination therapy with hemodialysis and hemoperfusion,and hyperbaric oxygen therapy may alleviate multiorgan failure and prevent neurological sequelae caused by heatstroke.展开更多
Objective:To analyze the clinical and follow-up data of patients with multiple organ failure of melioidosis pneumonia,to strengthen the understanding of melioidosis,and to provide references for the diagnosis and trea...Objective:To analyze the clinical and follow-up data of patients with multiple organ failure of melioidosis pneumonia,to strengthen the understanding of melioidosis,and to provide references for the diagnosis and treatment of clinicians,especially doctors in Hai nan province with high incidence,so as to reduce misdiagnosis,improve the diagnosis rate and reduce the mortality.Methods:The clinical symptoms,signs,laboratory examination and follow-up of patients with multiple organ failure were analyzed retrospectively.Results:Multiple organ failure with melioidosis pneumonia was easily misdiagnosed,Treatment cycle is long,easy to relapse.Conclusion:It is very important to analyze and differentiate the anthrax with multiple organ failure,especially in Hai nan province,which can improve the diagnosis rate and reduce the mortality.展开更多
Objective: the effect of high quality nursing on patients with acute renal failure and multiple organ failure treated with continuous renal replacement therapy. Methods: 20 patients in our hospital from January 2021 t...Objective: the effect of high quality nursing on patients with acute renal failure and multiple organ failure treated with continuous renal replacement therapy. Methods: 20 patients in our hospital from January 2021 to January 2022 were randomly divided into two groups, with 10 patients in each group. The observation group received high-quality nursing and the control group received routine nursing. Results: the nursing satisfaction of the observation group was higher than that of the control group (P < 0.05);complications were lower than those in the control group (P < 0.05). The level of inflammatory factors was lower than that of the control group (P < 0.05). The index level of renal function was lower than that of the control group (P < 0.05). The quality of life was higher than that of the control group (P < 0.05). Conclusion: high-quality nursing intervention for patients with acute renal failure during continuous renal replacement therapy can effectively improve the treatment effect, and it is recommended to be popularized.展开更多
Critical illness polyneuropathy and critical illness myopathy are frequent complications of severe illness that involve sensorimotor axons and skeletal muscles, respectively. Clinically, they manifest as limb and resp...Critical illness polyneuropathy and critical illness myopathy are frequent complications of severe illness that involve sensorimotor axons and skeletal muscles, respectively. Clinically, they manifest as limb and respiratory muscle weakness. Critical illness polyneuropathy/myopathy in isolation or combination increases intensive care unit morbidity via the inability or difficulty in weaning these patients off mechanical ventilation. Many patients continue to suffer from decreased exercise capacity and compromised quality of life for months to years after the acute event. Substantial progress has been made lately in the understanding of the pathophysiology of critical illness polyneuropathy and myopathy. Clinical and ancillary test results should be carefully interpreted to differentiate critical illness polyneuropathy/myopathy from similar weaknesses in this patient population. The present review is aimed at providing the latest knowledge concerning the pathophysiology of critical illness polyneuropathy/myopathy along with relevant clinical, diagnostic, differentiating, and treatment information for this debilitat- ing neurological disease.展开更多
Abdominal compartment syndrome(ACS)develops when organ failure arises secondary to an increase in intraabdominal pressure.The abdominal pressure is determined by multiple factors such as blood pressure,abdominal compl...Abdominal compartment syndrome(ACS)develops when organ failure arises secondary to an increase in intraabdominal pressure.The abdominal pressure is determined by multiple factors such as blood pressure,abdominal compliance,and other factors that exert a constant pressure within the abdominal cavity.Several conditions in the critically ill may increase abdominal pressure compromising organ perfusion that may lead to renal and respiratory dysfunction.Among surgical and trauma patients,aggressive fluid resuscitation is the most commonly reported risk factor to develop ACS.Other conditions that have also been identified as risk factors are ascites,hemoperitoneum,bowel distention,and large tumors.All patients with abdominal trauma possess a higher risk of developing intra-abdominal hypertension(IAH).Certain surgical interventions are reported to have a higher risk to develop IAH such as damage control surgery,abdominal aortic aneurysm repair,and liver transplantation among others.Close monitoring of organ function and intra-abdominal pressure(IAP)allows clinicians to diagnose ACS rapidly and intervene with target-specific management to reduce IAP.Surgical decompression followed by temporary abdominal closure should be considered in all patients with signs of organ dysfunction.There is still a great need for more studies to determine the adequate timing for interventions to improve patient outcomes.展开更多
Elevated intra-abdominal pressure(IAP)is a known cause of increased morbidity and mortality among critically ill patients.Intra-abdominal hypertension(IAH)and abdominal compartment syndrome can lead to rapid deteriora...Elevated intra-abdominal pressure(IAP)is a known cause of increased morbidity and mortality among critically ill patients.Intra-abdominal hypertension(IAH)and abdominal compartment syndrome can lead to rapid deterioration of organ function and the development of multiple organ failure.Raised IAP affects every system and main organ in the human body.Even marginally sustained IAH results in malperfusion and may disrupt the process of recovery.Yet,despite being so common,this potentially lethal condition often goes unnoticed.In 2004,the World Society of the Abdominal Compartment Syndrome,an international multidisciplinary consensus group,was formed to provide unified definitions,improve understanding and promote research in this field.Simple,reliable and nearly costless standardized methods of non-invasive measurement and monitoring of bladder pressure allow early recognition of IAH and timely optimized management.The correct,structured approach to treatment can have a striking effect and fully restore homeostasis.In recent years,significant progress has been made in this area with the contribution of surgeons,internal medicine specialists and anesthesiologists.Our review focuses on recent advances in order to present the complex underlying pathophysiology and guidelines concerning diagnosis,monitoring and treatment of this life-threatening condition.展开更多
BACKGROUND Visceral disseminated varicella-zoster virus(VZV)infection is a rare but lifethreatening disease.In transplant recipients with VZV infection,visceral dissemination may develop without skin eruptions,which l...BACKGROUND Visceral disseminated varicella-zoster virus(VZV)infection is a rare but lifethreatening disease.In transplant recipients with VZV infection,visceral dissemination may develop without skin eruptions,which leads to the failure of early diagnosis.CASE SUMMARY The patient was a 33-year-old male renal recipient who was referred to our hospital with severe upper abdominal pain of 3-d duration.On admission,the patient rapidly developed septic shock and multiple organ dysfunction syndrome with liver dysfunction and acute kidney injury.Next-generation sequencing of peripheral blood yielded 39224 sequence reads of VZV,and real-time polymerase chain reaction for VZV was positive,with 1.2×10^(7) copies/mL.The final diagnosis was visceral disseminated VZV infection.Acyclovir and supportive therapy were started,but the patient died of severe visceral organ damage 16 h after admission.CONCLUSION Visceral disseminated VZV infection is possible in renal transplant recipients presenting abdominal pain and rapidly-evolving organ damage without skin involvement.展开更多
In this paper, a new method is developed to model dependent failure behavior among failure mechanisms. Unlike the existing methods, the developed method models the root cause of the dependency explicitly, so that a de...In this paper, a new method is developed to model dependent failure behavior among failure mechanisms. Unlike the existing methods, the developed method models the root cause of the dependency explicitly, so that a deterministic model, rather than a probabilistic one, can be established. Three steps comprise the developed method. First, physics-of-failure(PoF) models are utilized to model each failure mechanism. Then, interactions among failure mechanisms are modeled as a combination of three basic relations, competition, superposition and coupling. This is the reason why the method is referred to as "compositional method". Finally, the PoF models and the interaction model are combined to develop a deterministic model of the dependent failure behavior. As a demonstration, the method is applied on an actual spool and the developed failure behavior model is validated by a wear test. The result demonstrates that the compositional method is an effective way to model dependent failure behavior.展开更多
We read with interest the review paper by Tao et al. on thetopic of early severe acute pancreatifis (EASP, defined as severeacute pancreatitis according to the Altanta criteria, with organdysfunction within 72 h after...We read with interest the review paper by Tao et al. on thetopic of early severe acute pancreatifis (EASP, defined as severeacute pancreatitis according to the Altanta criteria, with organdysfunction within 72 h after the start of symptoms) in a recentissue of the World Journal of Gastroenterology. It addressesan important problem in patients with severe acute pancreatitis,namely early organ dysfunction and its effect on outcomes.展开更多
文摘Uncertainty is an important factor that needs to be considered while analyzing the performance of any engineering system.In order to quantify uncertainty,fuzzy set theory is frequently used by most of researchers,including energy system experts.According to the classical reliability theory,component lifetimes have crisp parameters,but due to uncertainty and inaccuracy in data,it is sometimes very difficult to determine the exact values of these parameters in real-world systems.To overcome this difficulty in the current research,failure and repair rates were taken as triangular fuzzy numbers to determine the fuzzy availability of a system undergoing calendar-based periodic inspection subject to multiple failure modes(FMs).It was assumed that each component in the system had an exponential failure rate and repair rate with fuzzy parameters.System FMs were explicitly taken into account when a functional state of the system was considered.Each FM had a random failure time.On the occurrence of any failure,a random time was selected for the relevant corrective repair work.The proposed research was studied for one of the major sources of green energy,namely a wind turbine system wherein all the derived propositions have been implemented on it.
基金Supported by National Natural Science Foundation of China,No.82100625Zhejiang Provincial natural Science Foundation of China,No.Q20H030013.
文摘BACKGROUND The introduction of pegaspargase has greatly advanced the treatment of acute lymphoblastic leukemia(ALL).In the literature,only one case of pegaspargaseinduced multiple organ failure has been reported,and the patient died due to multiple organ failure.CASE SUMMARY Herein,we present a rare case of a 40-year-old man with ALL who developed multiple organ failure after treatment with pegaspargase.The patient had two rare phenomena reflecting poor prognosis,including the discrepancy between clinical manifestations and liver function and persistently low alpha-fetoprotein(AFP)levels from subacute liver failure.However,the patient was successfully treated using a multidisciplinary team approach.CONCLUSION This is the first case report of successful treatment of pegaspargase-induced multiple organ failure.The findings emphasize the importance of a multidisciplinary team approach in treating pegaspargase-induced multiple organ failure.
基金supported by the National Natural Science Foundation of China(Grant Nos.12202028,52022007,51905510)the National Science and Technology Major Project(Grant Nos.J2019-IV-0009-0077,J2019-IV-0006-0074,J2019-IV-0016-0084).
文摘In this work,heat treatment experiments at 1050 and 1100℃were carried out on single-crystal su-peralloy specimens coated with the electron beam physical vapor deposition(EB-PVD)thermal barrier coating(TBC)system.Furthermore,the evolution of microstructural characteristics and sintering-induced mechanical properties were separately obtained by the field emission scanning electron microscope(FE-SEM)and nanoindenter,providing inputs for damage modeling.Meanwhile,the critical compressive strain of TBCs at room temperature was acquired using 3D digital image correlation(3D-DIC)technology to characterize the interfacial damage combined with the experimentally observed buckling modes.The re-sults demonstrate that not only does oxidative damage exist in the TBCs system due to thermally grown oxide(TGO)growth,but additional damage is generated by thermal cycling and sintering behavior,re-spectively.Then,a nonlinear cumulative interfacial damage model considering multiple failure factors is developed to predict TBCs’life.The error between the measured damage and calculated damage is less than 15%,showing good prediction accuracy.
文摘BACKGROUND Multiple acyl-CoA dehydrogenase deficiency(MADD)is a disease of rare autosomal recessive disorder.There are three types of MADD.Type I is a neonatalonset form with congenital anomalies.Type II is a neonatal-onset form without congenital anomalies.Type III is considered to a milder form and usually responds to riboflavin.However,late-onset form could also be fatal and not responsive to treatments.CASE SUMMARY We report a severe case of a young man with onset type III MADD induced by drugs and strenuous exercise characterized by rhabdomyolysis and liver dysfunction.Urine analysis indicated 12 out of 70 kinds of organic acids like glutaric acid-2 were detected.Serum analysis in genetic metabolic diseases revealed 24 out of 43 tested items were abnormal,revealing the elevation of several acylcarnitines and the reduction of carnitine in the patient.By next generation sequencing technology for gene sequencing related to fatty acid oxidation and carnitine cycle defects,a rare ETFDH gene variant was identified:NM_004453:4:C.1448C>T(p.Pro483 Leu).The patient was diagnosed with lateonset GAII.He was not responsive to riboflavin and progressively worsened into multiple organ failure that finally led to death.CONCLUSION Type III MADD can also be fatal and not responsive to treatments.
基金National Natural Science Foundation of China (10572117,10802063,50875213)Aeronautical Science Foundation of China (2007ZA53012)+1 种基金New Century Program For Excellent Talents of Ministry of Education of China (NCET-05-0868)National High-tech Research and Development Program (2007AA04Z401)
文摘Combining the advantages of the stratified sampling and the importance sampling, a stratified importance sampling method (SISM) is presented to analyze the reliability sensitivity for structure with multiple failure modes. In the presented method, the variable space is divided into several disjoint subspace by n-dimensional coordinate planes at the mean point of the random vec- tor, and the importance sampling functions in the subspaces are constructed by keeping the sampling center at the mean point and augmenting the standard deviation by a factor of 2. The sample size generated from the importance sampling function in each subspace is determined by the contribution of the subspace to the reliability sensitivity, which can be estimated by iterative simulation in the sampling process. The formulae of the reliability sensitivity estimation, the variance and the coefficient of variation are derived for the presented SISM. Comparing with the Monte Carlo method, the stratified sampling method and the importance sampling method, the presented SISM has wider applicability and higher calculation efficiency, which is demonstrated by numerical examples. Finally, the reliability sensitivity analysis of flap structure is illustrated that the SISM can be applied to engineering structure.
文摘The effects of early hemofiltration on the serum levels of cytokines, pro- and anti-inflammatory balance and organ function in pigs with severe acute pancreatits (SAP) were studied. SAP pig model was induced by retrograde injection of artificial bile into the pancreatic duct. The pigs were randomly divided into SAP hemofiltration treatment group (HF group, n=8) and SAP non-hemofiltration treatment group (NHF group, n=8). In the HF group, the animals were subjected to high-volume and zero-balance hemofiltration therapy. The results showed that as compared with NHF group, MAP, CVP and PaO 2/FiO 2 were significantly increased (P<0.01), while HR, urinary protein content, serum ALT level, pulmonary coefficient and lung wet/dry ratio obviously decreased (P<0.05) in HF group. Under a light microscope, the pulmonary histologic scoring was lower that in HF group (P<0.01) and the lesions of renal and liver tissues were milder. However, there was no significant difference in the pancreatic histologic scoring between the two groups. Six h after establishment of the model, the serum levels of TNF-α, IL-1β were lower, while the IL-10/TNF-α ratio was higher in HF group (all P<0.05). It was suggested that early hemofiltration could effectively remove the serum cytokines TNF-α and IL-1β in SAP pigs, elevate the ratio of IL-10/TNF-α, improve hemodynamics and alleviate the lesions of lung, kidney and liver tissues.
文摘This paper presents a procedure to develop fragility curves of structures equipped with TMD considering multiple failure functions.The failure criteria considered are maximum inter-story drift ratio as a safety criterion,maximum absolute acceleration as a convenience criterion and TMD stroke length.The relationship between intensity measure and responses of the structure was assumed to follow the power-law model,and a regression analysis was used to estimate its properties.A nonlinear eight-story shear building subjected to near-fault earthquakes was used for the numerical studies.Fragility curves using multiple and single failure functions for an uncontrolled structure and a structure equipped with optimal TMDs were developed.Numerical analysis showed that using multiple failure functions led to increasing the fragility when compared with using the single failure function for both the uncontrolled and controlled structures.However,TMDs slightly reduced the seismic fragility and have the capability to improve the reliability of the structure.Also,it was found that the fragility was significantly influenced by the values of the capacity thresholds of both the acceleration of the structure and TMD stroke length,which should be selected by considering the target performance and application of the structure and control device.
基金Natural Science Foundation of Hubei Province:Mechanism of Degenerative Aortic Valve Calcification Induced by Inflammatory Responses in Valvular Interstitial Cells(No.2020CFB577)。
文摘TanshinoneⅡA(TanⅡA)is a noteworthy lipophilic diterpene compound derived from the dried roots of the Traditional Chinese Medicine Danshen(Radix Salviae Miltiorrhizae)that has various pharmacological properties,including anti-inflammatory,antibacterial,and antioxidative effects.Sepsis is a life-threatening organ dysfunction induced by a dysregulated host response to infection.Recently,increasing attention has been paid to sepsis-induced dysfunction of the intestine,cardiovascular system,lungs,kidneys,liver,and other organs.Experimental studies have shown that TanⅡA has therapeutic potential for sepsis-induced organ dysfunction owing to its anti-inflammatory,anti-apoptotic and regulatory effects on multiple signalling pathways.The purpose of this article is to evaluate the potential multiorgan protective effects of TanⅡA in sepsis.
文摘BACKGROUND Adult-onset Still’s disease(AOSD)is a rare systemic inflammatory disease,which is characterized by daily fever and arthritis,with an evanescent rash and neutrophilic leukocytosis.To date,there has been no definite laboratory or imaging test available for diagnosing AOSD;the diagnosis is one of exclusion,which can be very challenging.In particular,AOSD patients may experience different complications affecting their clinical picture,management,and prognosis.The treatment of AOSD remains largely empirical and involves therapeutic agents.CASE SUMMARY We report the case of a 36-year-old woman who presented with fever,red rash,arthralgia,and sore throat.Her serum ferritin level and white blood cell count were markedly elevated,and the first diagnosis 22 years prior was"juvenile rheumatoid arthritis of systemic type".The patient was treated with prednisone,sulfasalazine,methotrexate,and leflunomide.After remission of her symptoms,the patient stopped taking the medications,and the disease recurred.Ultimately,the patient was diagnosed with adult-onset Still's disease.Relapse occurred several times due to self-medication withdrawal,and an interleukin-6 antagonist(tocilizumab/Actemra)was administered to control the disease.Recently,she was hospitalized because an incision did not heal,and the patient suddenly developed high fever and diarrhea during hospitalization.The patient's disease progressed violently and quickly developed into macrophage activation syndrome,disseminated intravascular coagulation,shock,and multiple organ failure.The patient had sudden cardiac arrest,and she died despite emergency rescue efforts.CONCLUSION AOSD patients need regular follow-up in the long-term treatment process,and must press formulary standard medication,and do not voluntarily withdraw or reduce the dose.Otherwise it may cause disease back-and-forth or serious lifethreatening complications.Meanwhile,strict management of trauma,infections,tumors,and other diseases may contribute to improved outcomes in patients with complications.
文摘With control using redundant multiple control surface arrangement and large-deflection drag rudders,a combat flying wing has a higher probability for control surface failures.Therefore,its flight control system must be able to reconfigure after such failures.Considering three types of typical control surface failures(lock-in-place(LIP),loss-of-effectiveness(LOE) and float),flight control reconfiguration characteristic and capability of such aircraft types are analyzed.Because of the control surface redundancy,the aircraft using the dynamic inversion flight control law already has a control allocation block.In this paper,its flight control configuration during the above failures is achieved by modifying this block.It is shown that such a reconfigurable flight control design is valid,through numerical simulations of flight attitude control task.Results indicate that,in the circumstances of control surface failures with limited degree and the degradation of the flying quality level,a combat flying wing adopting this flight control reconfiguration approach based on control allocation could guarantee its flight safety and perform some flight combat missions.
文摘BACKGROUND We report a rare case of full neurological recovery from severe nonexertional heat stroke in a 67-year-old woman with an initial Glasgow Coma Scale of 3.This report raises awareness among doctors that when heatstroke is diagnosed,comprehensive treatment should be implemented as soon as possible.Moreover,targeted temperature management,combination therapy with hemodialysis and hemoperfusion,and hyperbaric oxygen therapy may alleviate multiorgan failure and prevent neurological sequelae caused by heatstroke.CASE SUMMARY A previously healthy 67-year-old woman with an initial Glasgow Coma Scale of 3 was found lying prone on the road at noon on a summer day.Laboratory tests revealed multiorgan failure.As soon as heatstroke was diagnosed,comprehensive treatment was implemented.On hospital Day 3,the patient was extubated.Her initial Sequential Organ Failure Assessment score at hospitalization was 14 and decreased to 2 on hospital Day 4.On the seventh day following hospital admission,as the patient’s general condition improved,the levels of laboratory test findings decreased rapidly.Finally,the patient gradually recovered with no other neurological symptoms(the Glasgow Coma Scale at discharge was 15,and her ability to walk independently was restored).CONCLUSION This case demonstrated that targeted temperature management,combination therapy with hemodialysis and hemoperfusion,and hyperbaric oxygen therapy may alleviate multiorgan failure and prevent neurological sequelae caused by heatstroke.
基金National Natural Science Foundation of China(No.81660010,31660329,8191101552)
文摘Objective:To analyze the clinical and follow-up data of patients with multiple organ failure of melioidosis pneumonia,to strengthen the understanding of melioidosis,and to provide references for the diagnosis and treatment of clinicians,especially doctors in Hai nan province with high incidence,so as to reduce misdiagnosis,improve the diagnosis rate and reduce the mortality.Methods:The clinical symptoms,signs,laboratory examination and follow-up of patients with multiple organ failure were analyzed retrospectively.Results:Multiple organ failure with melioidosis pneumonia was easily misdiagnosed,Treatment cycle is long,easy to relapse.Conclusion:It is very important to analyze and differentiate the anthrax with multiple organ failure,especially in Hai nan province,which can improve the diagnosis rate and reduce the mortality.
文摘Objective: the effect of high quality nursing on patients with acute renal failure and multiple organ failure treated with continuous renal replacement therapy. Methods: 20 patients in our hospital from January 2021 to January 2022 were randomly divided into two groups, with 10 patients in each group. The observation group received high-quality nursing and the control group received routine nursing. Results: the nursing satisfaction of the observation group was higher than that of the control group (P < 0.05);complications were lower than those in the control group (P < 0.05). The level of inflammatory factors was lower than that of the control group (P < 0.05). The index level of renal function was lower than that of the control group (P < 0.05). The quality of life was higher than that of the control group (P < 0.05). Conclusion: high-quality nursing intervention for patients with acute renal failure during continuous renal replacement therapy can effectively improve the treatment effect, and it is recommended to be popularized.
基金supported by grants from China Scholarship Council,No.2008102056the National Natural Science Foundation of China,No.81241147
文摘Critical illness polyneuropathy and critical illness myopathy are frequent complications of severe illness that involve sensorimotor axons and skeletal muscles, respectively. Clinically, they manifest as limb and respiratory muscle weakness. Critical illness polyneuropathy/myopathy in isolation or combination increases intensive care unit morbidity via the inability or difficulty in weaning these patients off mechanical ventilation. Many patients continue to suffer from decreased exercise capacity and compromised quality of life for months to years after the acute event. Substantial progress has been made lately in the understanding of the pathophysiology of critical illness polyneuropathy and myopathy. Clinical and ancillary test results should be carefully interpreted to differentiate critical illness polyneuropathy/myopathy from similar weaknesses in this patient population. The present review is aimed at providing the latest knowledge concerning the pathophysiology of critical illness polyneuropathy/myopathy along with relevant clinical, diagnostic, differentiating, and treatment information for this debilitat- ing neurological disease.
文摘Abdominal compartment syndrome(ACS)develops when organ failure arises secondary to an increase in intraabdominal pressure.The abdominal pressure is determined by multiple factors such as blood pressure,abdominal compliance,and other factors that exert a constant pressure within the abdominal cavity.Several conditions in the critically ill may increase abdominal pressure compromising organ perfusion that may lead to renal and respiratory dysfunction.Among surgical and trauma patients,aggressive fluid resuscitation is the most commonly reported risk factor to develop ACS.Other conditions that have also been identified as risk factors are ascites,hemoperitoneum,bowel distention,and large tumors.All patients with abdominal trauma possess a higher risk of developing intra-abdominal hypertension(IAH).Certain surgical interventions are reported to have a higher risk to develop IAH such as damage control surgery,abdominal aortic aneurysm repair,and liver transplantation among others.Close monitoring of organ function and intra-abdominal pressure(IAP)allows clinicians to diagnose ACS rapidly and intervene with target-specific management to reduce IAP.Surgical decompression followed by temporary abdominal closure should be considered in all patients with signs of organ dysfunction.There is still a great need for more studies to determine the adequate timing for interventions to improve patient outcomes.
文摘Elevated intra-abdominal pressure(IAP)is a known cause of increased morbidity and mortality among critically ill patients.Intra-abdominal hypertension(IAH)and abdominal compartment syndrome can lead to rapid deterioration of organ function and the development of multiple organ failure.Raised IAP affects every system and main organ in the human body.Even marginally sustained IAH results in malperfusion and may disrupt the process of recovery.Yet,despite being so common,this potentially lethal condition often goes unnoticed.In 2004,the World Society of the Abdominal Compartment Syndrome,an international multidisciplinary consensus group,was formed to provide unified definitions,improve understanding and promote research in this field.Simple,reliable and nearly costless standardized methods of non-invasive measurement and monitoring of bladder pressure allow early recognition of IAH and timely optimized management.The correct,structured approach to treatment can have a striking effect and fully restore homeostasis.In recent years,significant progress has been made in this area with the contribution of surgeons,internal medicine specialists and anesthesiologists.Our review focuses on recent advances in order to present the complex underlying pathophysiology and guidelines concerning diagnosis,monitoring and treatment of this life-threatening condition.
文摘BACKGROUND Visceral disseminated varicella-zoster virus(VZV)infection is a rare but lifethreatening disease.In transplant recipients with VZV infection,visceral dissemination may develop without skin eruptions,which leads to the failure of early diagnosis.CASE SUMMARY The patient was a 33-year-old male renal recipient who was referred to our hospital with severe upper abdominal pain of 3-d duration.On admission,the patient rapidly developed septic shock and multiple organ dysfunction syndrome with liver dysfunction and acute kidney injury.Next-generation sequencing of peripheral blood yielded 39224 sequence reads of VZV,and real-time polymerase chain reaction for VZV was positive,with 1.2×10^(7) copies/mL.The final diagnosis was visceral disseminated VZV infection.Acyclovir and supportive therapy were started,but the patient died of severe visceral organ damage 16 h after admission.CONCLUSION Visceral disseminated VZV infection is possible in renal transplant recipients presenting abdominal pain and rapidly-evolving organ damage without skin involvement.
基金supported by the National Natural Science Foundation of China (No. 71671009)supported by the National Natural Science Foundation of China (No. 61573043)supported by the National Natural Science Foundation of China (No. 51675025)
文摘In this paper, a new method is developed to model dependent failure behavior among failure mechanisms. Unlike the existing methods, the developed method models the root cause of the dependency explicitly, so that a deterministic model, rather than a probabilistic one, can be established. Three steps comprise the developed method. First, physics-of-failure(PoF) models are utilized to model each failure mechanism. Then, interactions among failure mechanisms are modeled as a combination of three basic relations, competition, superposition and coupling. This is the reason why the method is referred to as "compositional method". Finally, the PoF models and the interaction model are combined to develop a deterministic model of the dependent failure behavior. As a demonstration, the method is applied on an actual spool and the developed failure behavior model is validated by a wear test. The result demonstrates that the compositional method is an effective way to model dependent failure behavior.
基金supported by the Defense Industrial Technology Development Program (Grant Nos.JCKY2018601B001,JCKY2019209C004,and JCKY2019205A006)the National Natural Science Foundation of China (Grant Nos.11432002,11772026,and 12002015)+1 种基金the Aeronautical Science Foundation of China (Grant No.20182951014)the Beijing Municipal Science and Technology Commission (Grant No.Z191100004619006).
文摘We read with interest the review paper by Tao et al. on thetopic of early severe acute pancreatifis (EASP, defined as severeacute pancreatitis according to the Altanta criteria, with organdysfunction within 72 h after the start of symptoms) in a recentissue of the World Journal of Gastroenterology. It addressesan important problem in patients with severe acute pancreatitis,namely early organ dysfunction and its effect on outcomes.