An analytical model for chatter aero-engine casings is presented in this paper stability prediction in bull-nose end milling of And the mechanics and dynamics variations due to the complex cutter and workpiece geomet...An analytical model for chatter aero-engine casings is presented in this paper stability prediction in bull-nose end milling of And the mechanics and dynamics variations due to the complex cutter and workpiece geometry are considered by analyzing the effects of the lead angle on the milling process. Firstly, the tool-workpiece engagement region is obtained by using a previously developed method and divided into several disk elements along the tool-axis direction. Secondly, a 3D dynamic model for stability limit calculation is developed and simplified into a 1D model in normal direction considering only the dominant mode of the workpiece. Then the cutting force coefficients, the start and exit angles corresponding to each disk element are determined. And the total stability lobe diagram is calculated using an iterative algorithm. Finally, several experimental tests are carried out to validate the feasibility and effectiveness of the proposed ~rediction approach.展开更多
In order to satisfy the machining requirements of aero-engine casing in modern aviation industry, this paper investigates three main issues during the design and development process of a five-axis machine tool with hi...In order to satisfy the machining requirements of aero-engine casing in modern aviation industry, this paper investigates three main issues during the design and development process of a five-axis machine tool with high accuracy, stiffness and efficiency, including whole structure design,key components design, and supporting stiffness design. First, an appropriate structure of five-axis machine tool is determined considering the processing characteristics of aero-engine casing. Then, a dual drive swing head and a compact motorized spindle are designed with enough drive capability and stiffness, and related structure, assembly method, cooling technology, and performance simulation are given in detail. Next, a design method of supporting stiffness of guide is proposed through the deformation prediction of the spindle end. Based on above work, a prototype of machine tool is developed, and some experiments are carried out, including performance tests of swing head and motorized spindle, and machining of a simulated workpiece of aero-engine casing. All experimental results show that the machine tool has satisfactory accuracy, stiffness and efficiency, which meets the machining requirements of aero-engine casing. The main work can be used as references for engineers and technicians, which are meaningful in practice.展开更多
The original monitoring data from aero-engines possess characteristics such as high dimen-sionality,strong noise,and imbalance,which present substantial challenges to traditional anomalydetection methods.In response,t...The original monitoring data from aero-engines possess characteristics such as high dimen-sionality,strong noise,and imbalance,which present substantial challenges to traditional anomalydetection methods.In response,this paper proposes a method based on Fuzzy Fusion of variablesand Discriminant mapping of features for Clustering(FFD-Clustering)to detect anomalies in originalmonitoring data from Aircraft Communication Addressing and Reporting System(ACARS).Firstly,associated variables are fuzzily grouped to extract the underlying distribution characteristics and trendsfrom the data.Secondly,a multi-layer contrastive denoising-based feature Fusion Encoding Network(FEN)is designed for each variable group,which can construct representative features for each variablegroup through eliminating strong noise and complex interrelations between variables.Thirdly,a featureDiscriminative Mapping Network(DMN)based on reconstruction difference re-clustering is designed,which can distinguish dissimilar feature vectors when mapping representative features to a unified fea-ture space.Finally,the K-means clustering is used to detect the abnormal feature vectors in the unifiedfeature space.Additionally,the algorithm is capable of reconstructing identified abnormal vectors,thereby locating the abnormal variable groups.The performance of this algorithm was tested ontwo public datasets and real original monitoring data from four aero-engines'ACARS,demonstratingits superiority and application potential in aero-engine anomaly detection.展开更多
By using fatigue crack propagation testing and microstructural characterization,the crack fracture and propagation mechanisms of K4169 superalloy under various loads were investigated.The results demonstrate that the ...By using fatigue crack propagation testing and microstructural characterization,the crack fracture and propagation mechanisms of K4169 superalloy under various loads were investigated.The results demonstrate that the grain sizes of K4169 superalloy significantly increase,and the precipitation of the needle-likeδphase and the Laves phase is observed.Voids and microcracks form at location of Laves phase enrichment,creating conditions for crack propagation.By the a−N(a is the crack length,and N is the number of cycles)relationship curve,the change in the fatigue crack growth rate with the increasing number of cycles progresses through three separate stages.The fracture process of K4169 superalloy under low-stress cyclic loading(3 kN)exhibits the ductile fracture.Subsequently,the fracture process starts to change from the ductile fracture to the brittle fracture as the stress increases to 4.5 kN.In the microstructures of fractures in both stress states,intergranular propagation is the mechanism responsible for crack propagation.Moreover,the Laves phase exists near the fracture crack,which is in line with the post-service structural phenomenon.展开更多
Precise and quantitative measurement of soot particle emission plays an essential role in accurately assessing the combustion performance of aero-engine combustors and infrared signature levels in aircraft exhausts.Am...Precise and quantitative measurement of soot particle emission plays an essential role in accurately assessing the combustion performance of aero-engine combustors and infrared signature levels in aircraft exhausts.Among various intrusive or non-intrusive approaches for soot diagnostics,Laser-Induced Incandescence(LⅡ)technique has been increasingly applied for soot concentration measurement in various combustion environments such as laminar flames and internal combustion engines due to its high spatial resolution and sensitivity.As for LⅡmeasurement in aero-engine combustors,however,it normally suffers from very limited optical accesses and often faces mandatory requirements of oblique imaging from a small backward angle.In this work,we demonstrate a Two-Color(2C)LⅡsystem that simultaneously captures LⅡsignal images at two distinct wavelengths using a Scheimpflug imaging configuration.A projective transformation algorithm and image overlapping procedures were employed to spatially correct the raw Scheimpflug LⅡimages.Performance validation of the developed 2C-Scheimpflug LⅡsystem was first conducted under specified conditions in a laminar C_(2)H_(4)/air McKenna flame.The obtained Soot Volume Fraction(SVF)level and its spatial distribution are in consistent with previous studies under identical flame conditions reported by other research groups.Finally,as a demonstration of engineering benchmark application,we applied the developed 2C-Scheimpflug LⅡsystem to measure SVF distribution in the cross-section plane perpendicular to the direction of flame propagation at the exhaust of a single-sector dual-swirl aero-engine model combustor.Transient soot production events were observed and characteristics of the SVF distribution were investigated.These experimental results suggest the feasibility of the 2C-Scheimpflug LⅡtechnique developed in this work for precise and quantitative measurements of soot concentration in practical environments.展开更多
This paper analyzes the nonlinear dynamic characteristics and stability of Aero-Engine Dual-Rotor(AEDR)systems under high-frequency excitation,based on the Adaptive Harmonic Balance with the Asymptotic Harmonic Select...This paper analyzes the nonlinear dynamic characteristics and stability of Aero-Engine Dual-Rotor(AEDR)systems under high-frequency excitation,based on the Adaptive Harmonic Balance with the Asymptotic Harmonic Selection(AHB-AHS)method.A finite element dynamic equation for the AEDR system is introduced,considering complex nonlinearities of the intershaft bearing,unbalanced excitations,and high-frequency excitation.A solving strategy combining the AHB-AHS method and improved arclength continuation method is proposed to solve highdimensional dynamic equations containing complex nonlinearities and to track periodic solutions with parameter variations.The Floquet theory is used to analyze the types of bifurcation points in the system and the stability of periodic motions.The results indicate that high-frequency excitation can couple high-order and low-order modes,especially when the system undergoes superharmonic resonance.High-frequency excitation leads to more combination frequency harmonics,among which N_(f)ω_(1)-2ω_(2)dominates.Furthermore,changing the parameters(amplitude and frequency)of high-frequency excitation widens or shifts the unstable regions of the system.These findings contribute to understanding the mechanism of high-frequency excitation on aero-engines and demonstrate that the proposed AHB-AHS method is a powerful tool for analyzing highdimensional complex nonlinear dynamic systems under multi-frequency excitation.展开更多
Quantitative measurement of Soot Volume Fraction (SVF) is an essential prerequisite for controlling soot particle emissions from aero-engine combustors. As an in-situ and non-intrusive optical diagnostic technique, La...Quantitative measurement of Soot Volume Fraction (SVF) is an essential prerequisite for controlling soot particle emissions from aero-engine combustors. As an in-situ and non-intrusive optical diagnostic technique, Laser-Induced Incandescence (LII) has been increasingly applied for soot concentration quantification in various combustion environments such as laminar flame, vehicle exhaust, internal combustion chamber as well as aero-engine combustor. In this work, we experimentally measured the spatial and temporal distribution of SVF using two-color LII technique at the outlet of a single-sector dual-swirl aero-engine model combustor. The effect of inlet pressure and air preheat temperature on the SVF distribution was separately investigated within a pressure range of 241–425 kPa and a temperature range of 292–500 K. The results show that soot production increases with the inlet pressure but generally decreases with the air preheat temperature. Qualitative analysis was provided to explain the above results of parametric studies. The LII experiments were also conducted under 3 designed conditions to evaluate soot emission under practical operations. Particularly, weak soot emission was detected at the outlet under the idle condition. Our experimental results provide a valuable benchmark for evaluating soot emission in the exhaust plume of this aero-engine combustor during practical operations.展开更多
The spatiotemporal distribution of soot concentration in aero-engine combustor is important for assessing its combustion performance.Here,we report experimental measurements of soot concentration in terms of Soot Volu...The spatiotemporal distribution of soot concentration in aero-engine combustor is important for assessing its combustion performance.Here,we report experimental measurements of soot concentration in terms of Soot Volume Fraction(SVF)and its spatiotemporal distribution in a single-sector dual-swirl aero-engine combustor using Two-Color Laser-Induced Incandescence(2C-LII).It is shown that soot predominantly forms in the symmetrical vortices of the primary combustion zone,exhibiting a V-type distribution with higher concentration in the lower half of the zone than the upper half,with a small amount distributed in the secondary recirculation zone.Soot emissions at the combustor outlet are relatively low under three typical operating conditions by LII experiments,which is aligned with Smoke Number(SN)from gas analysis.The effect of inlet air temperature on SVF distribution and dynamics in the primary combustion zone is studied,which suggests that the SVF level in the primary combustion zone monotonically increases with the temperature.Meanwhile,the SVF distribution becomes more symmetrical as the inlet temperature increases,although the overall SVF level in the lower half of the zone is still higher.We also investigate the influence of the inlet air pressure on the SVF distribution at the combustor outlet.The soot concentration at the combustor outlet increases with inlet pressure,mainly distributed irregularly across both sides and the center.On both sides,the distribution is continuous,while the center exhibits dot-like and linear patterns.Numerical simulations correlated SVF distribution with the flow field in the primary combustion zone,qualitatively explaining the observed SVF distribution behavior.These results under various conditions can provide valuable insights for improving the performance of this specific combustor and designing high-temperature-rise combustors in the future.展开更多
BACKGROUND Ganglioneuroma is a rare,well-differentiated,slow-growing benign tumor of the peripheral nerves,with surgical resection being the only curative treatment.Surgical resection of ganglioneuromas encasing major...BACKGROUND Ganglioneuroma is a rare,well-differentiated,slow-growing benign tumor of the peripheral nerves,with surgical resection being the only curative treatment.Surgical resection of ganglioneuromas encasing major blood vessels remains a substantial clinical challenge.Traditionally,these cases often require open abdominal surgery or combined organ resections,and in some instances,the tumors are considered unresectable.Currently,no reports have described the resection of such tumors via laparoscopy.CASE SUMMARY A 35-year-old woman was admitted to our hospital after the incidental discovery of a retroperitoneal space-occupying lesion.Imaging revealed a mass with the celiac axis and superior mesenteric artery passing through it.A neurogenic tumor was suspected,with ganglioneuroma being the most likely diagnosis.Following comprehensive preoperative preparation,the retroperitoneal tumor was resected using a three-dimensional laparoscopy combined with an organ suspension technique.The surgical approach involved incising the tumor along the vascular axis and conducting meticulous,vascular-preserving tumor excision.The operation lasted approximately 458 minutes,with an estimated blood loss of 50 mL.The patient was discharged on the 8th postoperative day.A transient liver injury occurred after surgery but improved rapidly.After 11 months of postoperative follow-up,no complications or tumor recurrence were observed.CONCLUSION This case illustrates the feasibility of minimally invasive laparoscopic resection for retroperitoneal ganglioneuromas encasing major blood vessels.展开更多
BACKGROUND Congenital hypothyroidism(CH)is a common condition in both preterm and term infants characterized by either thyroid gland absence or hypofunctionality.The clinical association of refractory lactic acidosis ...BACKGROUND Congenital hypothyroidism(CH)is a common condition in both preterm and term infants characterized by either thyroid gland absence or hypofunctionality.The clinical association of refractory lactic acidosis and heart failure has rarely been observed in cases of pediatric patients with CH pathology.Here,we explored the etiological relationship between CH,heart failure,and refractory lactic acidosis to reflect the importance of thyroid function screening in neonates with heart disease.CASE SUMMARY A 33-day-old extremely premature female infant presented with tachypnea,respiratory distress,recurrent infections,and abdominal distension postnatal.On admission to our facility,she had cardiomegaly,hepatomegaly,and lactic acidosis(revealed on blood gas analysis),with lactate progressively rising to 25 mmol/L.Chest radiographs showed pulmonary congestion,while echocardiography revealed cardiac enlargement,left ventricular wall thickening,and pericardial effusion.Initial management aimed at correcting acidosis and treating heart failure proved ineffective.After reassessment,thyroid function tests showed significantly decreased triiodothyronine,free triiodothyronine,thyroxine,and free thyroxine levels,with a significantly increased thyroidstimulating hormone level,confirming a CH diagnosis.Levothyroxine was administered,resulting in rapid correction of lactic acidosis and gradual improvement of thyroid function and systemic symptoms,culminating in full recovery and discharge.We also reviewed the relevant literature on thyroid and cardiac dysfunctions in order to explore their deeper association.CONCLUSION This case links CH-induced heart failure with refractory lactic acidosis,urging prompt thyroid screening in affected neonates to reduce mortality.展开更多
BACKGROUND SMARCB1/INI1-deficient pancreatic undifferentiated rhabdoid carcinoma is a highly aggressive tumor,and spontaneous splenic rupture(SSR)as its presenting manifestation is rarely reported among pancreatic mal...BACKGROUND SMARCB1/INI1-deficient pancreatic undifferentiated rhabdoid carcinoma is a highly aggressive tumor,and spontaneous splenic rupture(SSR)as its presenting manifestation is rarely reported among pancreatic malignancies.CASE SUMMARY We herein report a rare case of a 59-year-old female who presented with acute left upper quadrant abdominal pain without any history of trauma.Abdominal imaging demonstrated a heterogeneous splenic lesion with hemoperitoneum,raising clinical suspicion of SSR.Emergency laparotomy revealed a pancreatic tumor invading the spleen and left kidney,with associated splenic rupture and dense adhesions,necessitating en bloc resection of the distal pancreas,spleen,and left kidney.Histopathology revealed a biphasic malignancy composed of moderately differentiated pancreatic ductal adenocarcinoma and an undifferentiated carcinoma with rhabdoid morphology and loss of SMARCB1 expression.Immunohistochemical analysis confirmed complete loss of SMARCB1/INI1 in the undifferentiated component,along with a high Ki-67 index(approximately 80%)and CD10 positivity.The ductal adenocarcinoma component retained SMARCB1/INI1 expression and was positive for CK7 and CK-pan.Transitional zones between the two tumor components suggested progressive dedifferentiation and underlying genomic instability.The patient received adjuvant chemotherapy with gemcitabine and nab-paclitaxel and maintained a satisfactory quality of life at the 6-month follow-up.CONCLUSION This study reports a rare case of SMARCB1/INI1-deficient undifferentiated rhabdoid carcinoma of the pancreas combined with ductal adenocarcinoma,presenting as SSR-an exceptionally uncommon initial manifestation of pancreatic malignancy.展开更多
BACKGROUND Sorafenib has been the conventional treatment for advanced hepatocellular carcinoma(HCC)since 2008.While radiological complete responses are extremely rare,improved supportive care and multidisciplinary app...BACKGROUND Sorafenib has been the conventional treatment for advanced hepatocellular carcinoma(HCC)since 2008.While radiological complete responses are extremely rare,improved supportive care and multidisciplinary approaches in clinical practice may explain the recent increase in case reports and retrospective series documenting such responses.CASE SUMMARY This case series describes 3 patients with advanced HCC who achieved durable complete responses using first-line sorafenib therapy,even in the presence of portal vein thrombosis or extrahepatic spread,and highlights the potential for sustained remission in selected patients.Dermatologic toxicity and non-viral etiology may correlate with favorable outcomes;however,reliable predictive biomarkers for sorafenib response are lacking.CONCLUSION Future research into the etiology and molecular differences in HCC is necessary to develop more personalized therapy options.展开更多
BACKGROUND Edwardsiella tarda(E.tarda)belongs to the family Enterobacteriaceae and is generally seen to cause infections mainly in fish,but is also capable of infecting humans.Extraintestinal infections occur in patie...BACKGROUND Edwardsiella tarda(E.tarda)belongs to the family Enterobacteriaceae and is generally seen to cause infections mainly in fish,but is also capable of infecting humans.Extraintestinal infections occur in patients with certain risk factors,including immunocompromised status.We recently diagnosed a case of spontaneous bacterial peritonitis(SBP)due to E.tarda in an immuno-compromised dialysis patient.CASE SUMMARY Patient was a 55-year-old male,with a history of diabetic nephropathy being treated with hemodialysis three times a week.He was referred to our hospital due to an increased volume of ascites,and blood examination revealed increased inflammatory reaction.At our emergency department,he developed fever,disturbance of consciousness,abdominal distension,and abdomen-wide pain.In addition,a dialysis shunt was confirmed in his right forearm,and the shunt site showed no signs of inflammation.No wounds were confirmed on or in his body.A blood examination revealed increased values of white blood cells,C-reactive protein,and creatinine.Plain chest and abdominal computed tomography scanning revealed increased ascites volume.Abdominal paracentesis was performed and a Gram stain revealed Gramnegative bacillus.These findings prompted diagnosis of SBP.The patient was admitted and treated with cefmetazole,causing fever resolution and symptom improvements.Later,E.tarda was identified in ascites culture.The patient improved with decreased inflammatory response and was discharged on the 12th day of hospitalization.The antibiotic was terminated after 14 days of treatment.SBP in this case may have developed from chronic renal failure and diabetes mellitus.CONCLUSION We report the first known case of SBP due to E.tarda in an immuno-compromised dialysis patient.展开更多
BACKGROUND Rhabdomyosarcoma(RMS)is a type of malignant tumor originating from rhabdomyocytes or mesenchymal cells differentiating into rhabdomyocytes.Hepatic pleomorphic RMS is a rare malignant liver tumor.Hepatic sar...BACKGROUND Rhabdomyosarcoma(RMS)is a type of malignant tumor originating from rhabdomyocytes or mesenchymal cells differentiating into rhabdomyocytes.Hepatic pleomorphic RMS is a rare malignant liver tumor.Hepatic sarcomatoid carcinoma is also a rare epithelial malignant tumor originating from the liver;it is characterized by the coexistence of both carcinomatous and sarcomatoid spindle cell components.CASE SUMMARY This paper reports a special case of an elderly woman whose initial liver puncture biopsy showed pleomorphic RMS.After chemotherapy with the vincristine+doxorubicin+cyclophosphamide regimen,the alpha-fetoprotein level increased significantly.Therefore,a second liver puncture was performed,the pathological result of which was hepatic sarcomatoid carcinoma.Next-generation sequencing revealed MET gene amplification with an average copy number of 9 in the tumor tissue;however,both fluorescence in situ hybridization and immunohistochemical tests were negative for MET amplification.The treatment regimen was adjusted to chemotherapy combined with immunotherapy;however,the disease progressed rapidly,and the overall survival was only 6 months.CONCLUSION By sharing the diagnosis and treatment process of this patient and reviewing the relevant literature,we aim to help clinicians enhance their understanding of two rare diseases,namely pleomorphic RMS and sarcomatoid carcinoma of the liver.展开更多
BACKGROUND Ankle fractures are well-documented in snow sports,but concomitant Achilles tendon and peroneal tendon ruptures are rare.This case report presents a previously unreported combination of Achilles tendon rupt...BACKGROUND Ankle fractures are well-documented in snow sports,but concomitant Achilles tendon and peroneal tendon ruptures are rare.This case report presents a previously unreported combination of Achilles tendon rupture,peroneal tendon rupture,and fibular fracture in a snowboarder,highlighting the complex nature of diagnosis,management,and rehabilitation.CASE SUMMARY A 50-year-old male snowboarder presented with severe right ankle pain following a high speed tumbling crash.Initial evaluation revealed an Achilles tendon rupture and a non-displaced distal lateral malleolus fracture.Subsequent magnetic resonance imaging confirmed complete tears of the Achilles tendon and both peroneus longus and brevis tendons,along with a Weber A lateral malleolus fracture.Surgical intervention included a 4-suture core Kraków repair of the Achilles tendon with calcaneal docking,open reduction and internal fixation of the distal fibula fracture,and primary repair of both peroneal tendons.Postoperatively,a modified Achilles repair protocol was implemented.At 16 weeks post-surgery,radiographs showed a well-healed fibular fracture,and physical examination confirmed intact Achilles and peroneal tendon repairs.By 6 months,the patient had regained full daily and work activities,including recreational pursuits.CONCLUSION This case underscores the importance of maintaining a high index of suspicion for concomitant injuries in high-energy ankle trauma during snow sports.Timely advanced imaging and a comprehensive surgical approach are crucial for optimal outcomes in such complex cases.展开更多
BACKGROUND Salvage of the infected long stem revision total knee arthroplasty is challenging due to the presence of well-fixed ingrown or cemented stems.Reconstructive options are limited.Above knee amputation(AKA)is ...BACKGROUND Salvage of the infected long stem revision total knee arthroplasty is challenging due to the presence of well-fixed ingrown or cemented stems.Reconstructive options are limited.Above knee amputation(AKA)is often recommended.We present a surgical technique that was successfully used on four such patients to convert them to a knee fusion(KF)using a cephalomedullary nail.CASE SUMMARY Four patients with infected long stem revision knee replacements that refused AKA had a single stage removal of their infected revision total knee followed by a KF.They were all treated with a statically locked antegrade cephalomedullary fusion nail,augmented with antibiotic impregnated bone cement.All patients had successful limb salvage and were ambulatory with assistive devices at the time of last follow-up.All were infection free at an average follow-up of 25.5 months(range 16-31).CONCLUSION Single stage cephalomedullary nailing can result in a successful KF in patients with infected long stem revision total knees.展开更多
BACKGROUND Well-differentiated small bowel mesenteric liposarcoma(LPS)is rare,with high malignancy,poor prognosis,and high preponderance to local recurrence.CASE SUMMARY Here we described a 71-year-old male,who compla...BACKGROUND Well-differentiated small bowel mesenteric liposarcoma(LPS)is rare,with high malignancy,poor prognosis,and high preponderance to local recurrence.CASE SUMMARY Here we described a 71-year-old male,who complains of persistent abdominal distension for a month.The clinical manifestation is a huge abdominal mass occupying almost the entire abdomen.Physical examination indicated palpable massive mass in the abdomen,hard texture,indefinable boundary,poor mobility.The abdominal enhanced computed tomography at another hospital scan showed multiple abdominal masses originating from the small bowel mesentery.Abdominal and pelvic magnetic resonance imaging at our hospital showed multiple masses in the abdominal and pelvic cavities,indicating that the tumor originated from the mesentery or peritoneum.Results of exploratory laparotomy indicated that the tremendous mass primarily results from the mesentery of the small intestine,occupying the entire abdominal cavity in a polymorphic and lobulated shape.The patient underwent complete surgical resection of the tumor,and the weight of the tumor was approximately 11 kg.The histopathological examination of the resected specimens confirmed the diagnosis of well-differentiated LPS of the small bowel mesentery.CONCLUSION Completed surgical resection was cornerstone,and histopathological and molecular confirmations were crucial.The necessity of adjuvant therapy should be phrased as a potential consideration to improve patient’s survival time.展开更多
BACKGROUND Hepatocellular carcinoma(HCC)is a major type of liver cancer worldwide.In advanced stages,portal vein tumor thrombosis(PVTT)and jaundice are common,whereas obstructive jaundice(OJ)is relatively rare.Both co...BACKGROUND Hepatocellular carcinoma(HCC)is a major type of liver cancer worldwide.In advanced stages,portal vein tumor thrombosis(PVTT)and jaundice are common,whereas obstructive jaundice(OJ)is relatively rare.Both conditions markedly reduce survival and increase therapeutic complexity.Recently,hepatic artery infusion chemotherapy(HAIC)in combination with targeted immunotherapy has shown promise for advanced HCC.CASE SUMMARY We report a 47-year-old male with advanced HCC complicated by PVTT and OJ,who was admitted with marked jaundice of the skin and sclera.Imaging revealed a large hepatic mass(14.5 cm×11.3 cm)in the right lobe with associated portal vein tumor thrombus.The tertiary bile duct was only mildly dilated,making percutaneous transhepatic cholangiography drainage infeasible.The patient underwent reduced-dose HAIC,which resulted in significant tumor shrinkage and marked reduction in serum bilirubin.This improvement enabled sequential treatment with lenvatinib and camrelizumab.After six cycles,both liver function and alphafetoprotein levels improved.The patient achieved a progression-free survival of 20 months and an overall survival of 29 months.CONCLUSION HAIC can treat high-bilirubin HCC with PVTT and OJ,allowing for subsequent targeted immunotherapy.展开更多
It is challenging to diagnose isolated hyperbilirubinemia with rare and complex etiologies under the constraints of traditional testing conditions.Herein,we present a rare case of coexisting Gilbert syndrome(GS)and er...It is challenging to diagnose isolated hyperbilirubinemia with rare and complex etiologies under the constraints of traditional testing conditions.Herein,we present a rare case of coexisting Gilbert syndrome(GS)and erythropoietic protoporphyria(EPP),which has not been previously documented.CASE SUMMARY We present a rare case of coexisting GS and EPP in a 23-year-old Chinese male with a long history of jaundice and recently found splenomegaly.Serial nonspecific hemolysis screening tests yielded inconsistent results,and investigations for common hemolytic etiologies were negative.However,Levitt’s carbon monoxide breath test,which measures erythrocyte lifespan(the gold-standard marker of hemolysis),demonstrated significant hemolysis,revealing a markedly shortened erythrocyte lifespan of 11 days(normal average 120 days).Genetic testing subsequently confirmed EPP with a homozygous ferrochelatase gene mutation and GS with a heterozygous uridine diphosphate glucuronosyl trans-ferase 1A1 gene mutation.CONCLUSION The rapid,non-invasive Levitt’s carbon monoxide breath test resolved the diagnostic challenge posed by a rare and complex cause of hyperbilirubinemia.展开更多
BACKGROUND While varices and variceal bleeds are well-known and feared complications of advanced cirrhosis and portal hypertension,omental variceal bleed are a rare sequala even in patients with known esophageal or ga...BACKGROUND While varices and variceal bleeds are well-known and feared complications of advanced cirrhosis and portal hypertension,omental variceal bleed are a rare sequala even in patients with known esophageal or gastric varices.While rare,omental varices pose a risk for hemoperitoneum if ruptured,which is a lifethreatening complication with high mortality rates despite surgical intervention.CASE SUMMARY This report reviews the case of a patient 36-year-old female with alcohol related cirrhosis decompensated by ascites,but no history of varices admitted for hemorrhagic shock from spontaneous rupture of omental varices requiring emergency surgery.She underwent the first documented successful orthotopic liver transplantation the same admission.CONCLUSION This case report and literature review stresses the importance of early consideration and identification of intraabdominal variceal sources in cirrhotic patients with refractory shock.展开更多
基金supported by the National Basic Research Program of China(No.2013CB035802)the 111 Project of China(No.B13044)
文摘An analytical model for chatter aero-engine casings is presented in this paper stability prediction in bull-nose end milling of And the mechanics and dynamics variations due to the complex cutter and workpiece geometry are considered by analyzing the effects of the lead angle on the milling process. Firstly, the tool-workpiece engagement region is obtained by using a previously developed method and divided into several disk elements along the tool-axis direction. Secondly, a 3D dynamic model for stability limit calculation is developed and simplified into a 1D model in normal direction considering only the dominant mode of the workpiece. Then the cutting force coefficients, the start and exit angles corresponding to each disk element are determined. And the total stability lobe diagram is calculated using an iterative algorithm. Finally, several experimental tests are carried out to validate the feasibility and effectiveness of the proposed ~rediction approach.
基金co-supported by the Natural Science Foundation of Beijing(No.3214043)the Project of State Key Lab of Tribology of Tsinghua University(No.SKLT2021D16)the National Natural Science Foundation of China(No.51975319)。
文摘In order to satisfy the machining requirements of aero-engine casing in modern aviation industry, this paper investigates three main issues during the design and development process of a five-axis machine tool with high accuracy, stiffness and efficiency, including whole structure design,key components design, and supporting stiffness design. First, an appropriate structure of five-axis machine tool is determined considering the processing characteristics of aero-engine casing. Then, a dual drive swing head and a compact motorized spindle are designed with enough drive capability and stiffness, and related structure, assembly method, cooling technology, and performance simulation are given in detail. Next, a design method of supporting stiffness of guide is proposed through the deformation prediction of the spindle end. Based on above work, a prototype of machine tool is developed, and some experiments are carried out, including performance tests of swing head and motorized spindle, and machining of a simulated workpiece of aero-engine casing. All experimental results show that the machine tool has satisfactory accuracy, stiffness and efficiency, which meets the machining requirements of aero-engine casing. The main work can be used as references for engineers and technicians, which are meaningful in practice.
基金co-supported by the National Science and Technology Major Project,China(No.J2019-I-0001-0001)the National Natural Science Foundation of China(No.52105545)。
文摘The original monitoring data from aero-engines possess characteristics such as high dimen-sionality,strong noise,and imbalance,which present substantial challenges to traditional anomalydetection methods.In response,this paper proposes a method based on Fuzzy Fusion of variablesand Discriminant mapping of features for Clustering(FFD-Clustering)to detect anomalies in originalmonitoring data from Aircraft Communication Addressing and Reporting System(ACARS).Firstly,associated variables are fuzzily grouped to extract the underlying distribution characteristics and trendsfrom the data.Secondly,a multi-layer contrastive denoising-based feature Fusion Encoding Network(FEN)is designed for each variable group,which can construct representative features for each variablegroup through eliminating strong noise and complex interrelations between variables.Thirdly,a featureDiscriminative Mapping Network(DMN)based on reconstruction difference re-clustering is designed,which can distinguish dissimilar feature vectors when mapping representative features to a unified fea-ture space.Finally,the K-means clustering is used to detect the abnormal feature vectors in the unifiedfeature space.Additionally,the algorithm is capable of reconstructing identified abnormal vectors,thereby locating the abnormal variable groups.The performance of this algorithm was tested ontwo public datasets and real original monitoring data from four aero-engines'ACARS,demonstratingits superiority and application potential in aero-engine anomaly detection.
基金National Natural Science Foundation of China (No. 51975200)Hunan Provincial Innovation Foundation for Postgraduate,China (No. QL20220201)。
文摘By using fatigue crack propagation testing and microstructural characterization,the crack fracture and propagation mechanisms of K4169 superalloy under various loads were investigated.The results demonstrate that the grain sizes of K4169 superalloy significantly increase,and the precipitation of the needle-likeδphase and the Laves phase is observed.Voids and microcracks form at location of Laves phase enrichment,creating conditions for crack propagation.By the a−N(a is the crack length,and N is the number of cycles)relationship curve,the change in the fatigue crack growth rate with the increasing number of cycles progresses through three separate stages.The fracture process of K4169 superalloy under low-stress cyclic loading(3 kN)exhibits the ductile fracture.Subsequently,the fracture process starts to change from the ductile fracture to the brittle fracture as the stress increases to 4.5 kN.In the microstructures of fractures in both stress states,intergranular propagation is the mechanism responsible for crack propagation.Moreover,the Laves phase exists near the fracture crack,which is in line with the post-service structural phenomenon.
基金supported by the Equipment Test and Evaluation Technology Research Project,China(No.2100070017)the Natural Science Foundation of Gansu Province,China(No.24JRRA415)。
文摘Precise and quantitative measurement of soot particle emission plays an essential role in accurately assessing the combustion performance of aero-engine combustors and infrared signature levels in aircraft exhausts.Among various intrusive or non-intrusive approaches for soot diagnostics,Laser-Induced Incandescence(LⅡ)technique has been increasingly applied for soot concentration measurement in various combustion environments such as laminar flames and internal combustion engines due to its high spatial resolution and sensitivity.As for LⅡmeasurement in aero-engine combustors,however,it normally suffers from very limited optical accesses and often faces mandatory requirements of oblique imaging from a small backward angle.In this work,we demonstrate a Two-Color(2C)LⅡsystem that simultaneously captures LⅡsignal images at two distinct wavelengths using a Scheimpflug imaging configuration.A projective transformation algorithm and image overlapping procedures were employed to spatially correct the raw Scheimpflug LⅡimages.Performance validation of the developed 2C-Scheimpflug LⅡsystem was first conducted under specified conditions in a laminar C_(2)H_(4)/air McKenna flame.The obtained Soot Volume Fraction(SVF)level and its spatial distribution are in consistent with previous studies under identical flame conditions reported by other research groups.Finally,as a demonstration of engineering benchmark application,we applied the developed 2C-Scheimpflug LⅡsystem to measure SVF distribution in the cross-section plane perpendicular to the direction of flame propagation at the exhaust of a single-sector dual-swirl aero-engine model combustor.Transient soot production events were observed and characteristics of the SVF distribution were investigated.These experimental results suggest the feasibility of the 2C-Scheimpflug LⅡtechnique developed in this work for precise and quantitative measurements of soot concentration in practical environments.
基金the financial support from the National Key R&D Program of China(No.2023YFE0125900)National Natural Science Foundation of China(Nos.12372008 and 12102234)+1 种基金Natural Science Foundation of Heilongjiang Province,China(No.YQ2022A008)Taif University,Saudi Arabia,for supporting this work through Project number(TU-DSPP-2024-73).
文摘This paper analyzes the nonlinear dynamic characteristics and stability of Aero-Engine Dual-Rotor(AEDR)systems under high-frequency excitation,based on the Adaptive Harmonic Balance with the Asymptotic Harmonic Selection(AHB-AHS)method.A finite element dynamic equation for the AEDR system is introduced,considering complex nonlinearities of the intershaft bearing,unbalanced excitations,and high-frequency excitation.A solving strategy combining the AHB-AHS method and improved arclength continuation method is proposed to solve highdimensional dynamic equations containing complex nonlinearities and to track periodic solutions with parameter variations.The Floquet theory is used to analyze the types of bifurcation points in the system and the stability of periodic motions.The results indicate that high-frequency excitation can couple high-order and low-order modes,especially when the system undergoes superharmonic resonance.High-frequency excitation leads to more combination frequency harmonics,among which N_(f)ω_(1)-2ω_(2)dominates.Furthermore,changing the parameters(amplitude and frequency)of high-frequency excitation widens or shifts the unstable regions of the system.These findings contribute to understanding the mechanism of high-frequency excitation on aero-engines and demonstrate that the proposed AHB-AHS method is a powerful tool for analyzing highdimensional complex nonlinear dynamic systems under multi-frequency excitation.
基金supported by the National Key Research and Development Program of China(No.2020YFA0405700).
文摘Quantitative measurement of Soot Volume Fraction (SVF) is an essential prerequisite for controlling soot particle emissions from aero-engine combustors. As an in-situ and non-intrusive optical diagnostic technique, Laser-Induced Incandescence (LII) has been increasingly applied for soot concentration quantification in various combustion environments such as laminar flame, vehicle exhaust, internal combustion chamber as well as aero-engine combustor. In this work, we experimentally measured the spatial and temporal distribution of SVF using two-color LII technique at the outlet of a single-sector dual-swirl aero-engine model combustor. The effect of inlet pressure and air preheat temperature on the SVF distribution was separately investigated within a pressure range of 241–425 kPa and a temperature range of 292–500 K. The results show that soot production increases with the inlet pressure but generally decreases with the air preheat temperature. Qualitative analysis was provided to explain the above results of parametric studies. The LII experiments were also conducted under 3 designed conditions to evaluate soot emission under practical operations. Particularly, weak soot emission was detected at the outlet under the idle condition. Our experimental results provide a valuable benchmark for evaluating soot emission in the exhaust plume of this aero-engine combustor during practical operations.
基金support of the National Science and Technology Major Project of China(No.J2019-V-0005-0096)the National Key Research and Development Program of China(No.2020YFA0405700).
文摘The spatiotemporal distribution of soot concentration in aero-engine combustor is important for assessing its combustion performance.Here,we report experimental measurements of soot concentration in terms of Soot Volume Fraction(SVF)and its spatiotemporal distribution in a single-sector dual-swirl aero-engine combustor using Two-Color Laser-Induced Incandescence(2C-LII).It is shown that soot predominantly forms in the symmetrical vortices of the primary combustion zone,exhibiting a V-type distribution with higher concentration in the lower half of the zone than the upper half,with a small amount distributed in the secondary recirculation zone.Soot emissions at the combustor outlet are relatively low under three typical operating conditions by LII experiments,which is aligned with Smoke Number(SN)from gas analysis.The effect of inlet air temperature on SVF distribution and dynamics in the primary combustion zone is studied,which suggests that the SVF level in the primary combustion zone monotonically increases with the temperature.Meanwhile,the SVF distribution becomes more symmetrical as the inlet temperature increases,although the overall SVF level in the lower half of the zone is still higher.We also investigate the influence of the inlet air pressure on the SVF distribution at the combustor outlet.The soot concentration at the combustor outlet increases with inlet pressure,mainly distributed irregularly across both sides and the center.On both sides,the distribution is continuous,while the center exhibits dot-like and linear patterns.Numerical simulations correlated SVF distribution with the flow field in the primary combustion zone,qualitatively explaining the observed SVF distribution behavior.These results under various conditions can provide valuable insights for improving the performance of this specific combustor and designing high-temperature-rise combustors in the future.
基金Supported by the Zhejiang Medical Science and Technology Project,No.2022KY1325 and No.2023KY381Public Welfare Project of Jinhua Science and Technology Plan,No.2023-4-084Major Project of Jinhua Science and Technology Plan,No.2023-3-066.
文摘BACKGROUND Ganglioneuroma is a rare,well-differentiated,slow-growing benign tumor of the peripheral nerves,with surgical resection being the only curative treatment.Surgical resection of ganglioneuromas encasing major blood vessels remains a substantial clinical challenge.Traditionally,these cases often require open abdominal surgery or combined organ resections,and in some instances,the tumors are considered unresectable.Currently,no reports have described the resection of such tumors via laparoscopy.CASE SUMMARY A 35-year-old woman was admitted to our hospital after the incidental discovery of a retroperitoneal space-occupying lesion.Imaging revealed a mass with the celiac axis and superior mesenteric artery passing through it.A neurogenic tumor was suspected,with ganglioneuroma being the most likely diagnosis.Following comprehensive preoperative preparation,the retroperitoneal tumor was resected using a three-dimensional laparoscopy combined with an organ suspension technique.The surgical approach involved incising the tumor along the vascular axis and conducting meticulous,vascular-preserving tumor excision.The operation lasted approximately 458 minutes,with an estimated blood loss of 50 mL.The patient was discharged on the 8th postoperative day.A transient liver injury occurred after surgery but improved rapidly.After 11 months of postoperative follow-up,no complications or tumor recurrence were observed.CONCLUSION This case illustrates the feasibility of minimally invasive laparoscopic resection for retroperitoneal ganglioneuromas encasing major blood vessels.
文摘BACKGROUND Congenital hypothyroidism(CH)is a common condition in both preterm and term infants characterized by either thyroid gland absence or hypofunctionality.The clinical association of refractory lactic acidosis and heart failure has rarely been observed in cases of pediatric patients with CH pathology.Here,we explored the etiological relationship between CH,heart failure,and refractory lactic acidosis to reflect the importance of thyroid function screening in neonates with heart disease.CASE SUMMARY A 33-day-old extremely premature female infant presented with tachypnea,respiratory distress,recurrent infections,and abdominal distension postnatal.On admission to our facility,she had cardiomegaly,hepatomegaly,and lactic acidosis(revealed on blood gas analysis),with lactate progressively rising to 25 mmol/L.Chest radiographs showed pulmonary congestion,while echocardiography revealed cardiac enlargement,left ventricular wall thickening,and pericardial effusion.Initial management aimed at correcting acidosis and treating heart failure proved ineffective.After reassessment,thyroid function tests showed significantly decreased triiodothyronine,free triiodothyronine,thyroxine,and free thyroxine levels,with a significantly increased thyroidstimulating hormone level,confirming a CH diagnosis.Levothyroxine was administered,resulting in rapid correction of lactic acidosis and gradual improvement of thyroid function and systemic symptoms,culminating in full recovery and discharge.We also reviewed the relevant literature on thyroid and cardiac dysfunctions in order to explore their deeper association.CONCLUSION This case links CH-induced heart failure with refractory lactic acidosis,urging prompt thyroid screening in affected neonates to reduce mortality.
文摘BACKGROUND SMARCB1/INI1-deficient pancreatic undifferentiated rhabdoid carcinoma is a highly aggressive tumor,and spontaneous splenic rupture(SSR)as its presenting manifestation is rarely reported among pancreatic malignancies.CASE SUMMARY We herein report a rare case of a 59-year-old female who presented with acute left upper quadrant abdominal pain without any history of trauma.Abdominal imaging demonstrated a heterogeneous splenic lesion with hemoperitoneum,raising clinical suspicion of SSR.Emergency laparotomy revealed a pancreatic tumor invading the spleen and left kidney,with associated splenic rupture and dense adhesions,necessitating en bloc resection of the distal pancreas,spleen,and left kidney.Histopathology revealed a biphasic malignancy composed of moderately differentiated pancreatic ductal adenocarcinoma and an undifferentiated carcinoma with rhabdoid morphology and loss of SMARCB1 expression.Immunohistochemical analysis confirmed complete loss of SMARCB1/INI1 in the undifferentiated component,along with a high Ki-67 index(approximately 80%)and CD10 positivity.The ductal adenocarcinoma component retained SMARCB1/INI1 expression and was positive for CK7 and CK-pan.Transitional zones between the two tumor components suggested progressive dedifferentiation and underlying genomic instability.The patient received adjuvant chemotherapy with gemcitabine and nab-paclitaxel and maintained a satisfactory quality of life at the 6-month follow-up.CONCLUSION This study reports a rare case of SMARCB1/INI1-deficient undifferentiated rhabdoid carcinoma of the pancreas combined with ductal adenocarcinoma,presenting as SSR-an exceptionally uncommon initial manifestation of pancreatic malignancy.
文摘BACKGROUND Sorafenib has been the conventional treatment for advanced hepatocellular carcinoma(HCC)since 2008.While radiological complete responses are extremely rare,improved supportive care and multidisciplinary approaches in clinical practice may explain the recent increase in case reports and retrospective series documenting such responses.CASE SUMMARY This case series describes 3 patients with advanced HCC who achieved durable complete responses using first-line sorafenib therapy,even in the presence of portal vein thrombosis or extrahepatic spread,and highlights the potential for sustained remission in selected patients.Dermatologic toxicity and non-viral etiology may correlate with favorable outcomes;however,reliable predictive biomarkers for sorafenib response are lacking.CONCLUSION Future research into the etiology and molecular differences in HCC is necessary to develop more personalized therapy options.
文摘BACKGROUND Edwardsiella tarda(E.tarda)belongs to the family Enterobacteriaceae and is generally seen to cause infections mainly in fish,but is also capable of infecting humans.Extraintestinal infections occur in patients with certain risk factors,including immunocompromised status.We recently diagnosed a case of spontaneous bacterial peritonitis(SBP)due to E.tarda in an immuno-compromised dialysis patient.CASE SUMMARY Patient was a 55-year-old male,with a history of diabetic nephropathy being treated with hemodialysis three times a week.He was referred to our hospital due to an increased volume of ascites,and blood examination revealed increased inflammatory reaction.At our emergency department,he developed fever,disturbance of consciousness,abdominal distension,and abdomen-wide pain.In addition,a dialysis shunt was confirmed in his right forearm,and the shunt site showed no signs of inflammation.No wounds were confirmed on or in his body.A blood examination revealed increased values of white blood cells,C-reactive protein,and creatinine.Plain chest and abdominal computed tomography scanning revealed increased ascites volume.Abdominal paracentesis was performed and a Gram stain revealed Gramnegative bacillus.These findings prompted diagnosis of SBP.The patient was admitted and treated with cefmetazole,causing fever resolution and symptom improvements.Later,E.tarda was identified in ascites culture.The patient improved with decreased inflammatory response and was discharged on the 12th day of hospitalization.The antibiotic was terminated after 14 days of treatment.SBP in this case may have developed from chronic renal failure and diabetes mellitus.CONCLUSION We report the first known case of SBP due to E.tarda in an immuno-compromised dialysis patient.
基金Supported by Shaanxi Provincial Natural Science Basic Research Program,No.2020JQ-951.
文摘BACKGROUND Rhabdomyosarcoma(RMS)is a type of malignant tumor originating from rhabdomyocytes or mesenchymal cells differentiating into rhabdomyocytes.Hepatic pleomorphic RMS is a rare malignant liver tumor.Hepatic sarcomatoid carcinoma is also a rare epithelial malignant tumor originating from the liver;it is characterized by the coexistence of both carcinomatous and sarcomatoid spindle cell components.CASE SUMMARY This paper reports a special case of an elderly woman whose initial liver puncture biopsy showed pleomorphic RMS.After chemotherapy with the vincristine+doxorubicin+cyclophosphamide regimen,the alpha-fetoprotein level increased significantly.Therefore,a second liver puncture was performed,the pathological result of which was hepatic sarcomatoid carcinoma.Next-generation sequencing revealed MET gene amplification with an average copy number of 9 in the tumor tissue;however,both fluorescence in situ hybridization and immunohistochemical tests were negative for MET amplification.The treatment regimen was adjusted to chemotherapy combined with immunotherapy;however,the disease progressed rapidly,and the overall survival was only 6 months.CONCLUSION By sharing the diagnosis and treatment process of this patient and reviewing the relevant literature,we aim to help clinicians enhance their understanding of two rare diseases,namely pleomorphic RMS and sarcomatoid carcinoma of the liver.
文摘BACKGROUND Ankle fractures are well-documented in snow sports,but concomitant Achilles tendon and peroneal tendon ruptures are rare.This case report presents a previously unreported combination of Achilles tendon rupture,peroneal tendon rupture,and fibular fracture in a snowboarder,highlighting the complex nature of diagnosis,management,and rehabilitation.CASE SUMMARY A 50-year-old male snowboarder presented with severe right ankle pain following a high speed tumbling crash.Initial evaluation revealed an Achilles tendon rupture and a non-displaced distal lateral malleolus fracture.Subsequent magnetic resonance imaging confirmed complete tears of the Achilles tendon and both peroneus longus and brevis tendons,along with a Weber A lateral malleolus fracture.Surgical intervention included a 4-suture core Kraków repair of the Achilles tendon with calcaneal docking,open reduction and internal fixation of the distal fibula fracture,and primary repair of both peroneal tendons.Postoperatively,a modified Achilles repair protocol was implemented.At 16 weeks post-surgery,radiographs showed a well-healed fibular fracture,and physical examination confirmed intact Achilles and peroneal tendon repairs.By 6 months,the patient had regained full daily and work activities,including recreational pursuits.CONCLUSION This case underscores the importance of maintaining a high index of suspicion for concomitant injuries in high-energy ankle trauma during snow sports.Timely advanced imaging and a comprehensive surgical approach are crucial for optimal outcomes in such complex cases.
文摘BACKGROUND Salvage of the infected long stem revision total knee arthroplasty is challenging due to the presence of well-fixed ingrown or cemented stems.Reconstructive options are limited.Above knee amputation(AKA)is often recommended.We present a surgical technique that was successfully used on four such patients to convert them to a knee fusion(KF)using a cephalomedullary nail.CASE SUMMARY Four patients with infected long stem revision knee replacements that refused AKA had a single stage removal of their infected revision total knee followed by a KF.They were all treated with a statically locked antegrade cephalomedullary fusion nail,augmented with antibiotic impregnated bone cement.All patients had successful limb salvage and were ambulatory with assistive devices at the time of last follow-up.All were infection free at an average follow-up of 25.5 months(range 16-31).CONCLUSION Single stage cephalomedullary nailing can result in a successful KF in patients with infected long stem revision total knees.
文摘BACKGROUND Well-differentiated small bowel mesenteric liposarcoma(LPS)is rare,with high malignancy,poor prognosis,and high preponderance to local recurrence.CASE SUMMARY Here we described a 71-year-old male,who complains of persistent abdominal distension for a month.The clinical manifestation is a huge abdominal mass occupying almost the entire abdomen.Physical examination indicated palpable massive mass in the abdomen,hard texture,indefinable boundary,poor mobility.The abdominal enhanced computed tomography at another hospital scan showed multiple abdominal masses originating from the small bowel mesentery.Abdominal and pelvic magnetic resonance imaging at our hospital showed multiple masses in the abdominal and pelvic cavities,indicating that the tumor originated from the mesentery or peritoneum.Results of exploratory laparotomy indicated that the tremendous mass primarily results from the mesentery of the small intestine,occupying the entire abdominal cavity in a polymorphic and lobulated shape.The patient underwent complete surgical resection of the tumor,and the weight of the tumor was approximately 11 kg.The histopathological examination of the resected specimens confirmed the diagnosis of well-differentiated LPS of the small bowel mesentery.CONCLUSION Completed surgical resection was cornerstone,and histopathological and molecular confirmations were crucial.The necessity of adjuvant therapy should be phrased as a potential consideration to improve patient’s survival time.
文摘BACKGROUND Hepatocellular carcinoma(HCC)is a major type of liver cancer worldwide.In advanced stages,portal vein tumor thrombosis(PVTT)and jaundice are common,whereas obstructive jaundice(OJ)is relatively rare.Both conditions markedly reduce survival and increase therapeutic complexity.Recently,hepatic artery infusion chemotherapy(HAIC)in combination with targeted immunotherapy has shown promise for advanced HCC.CASE SUMMARY We report a 47-year-old male with advanced HCC complicated by PVTT and OJ,who was admitted with marked jaundice of the skin and sclera.Imaging revealed a large hepatic mass(14.5 cm×11.3 cm)in the right lobe with associated portal vein tumor thrombus.The tertiary bile duct was only mildly dilated,making percutaneous transhepatic cholangiography drainage infeasible.The patient underwent reduced-dose HAIC,which resulted in significant tumor shrinkage and marked reduction in serum bilirubin.This improvement enabled sequential treatment with lenvatinib and camrelizumab.After six cycles,both liver function and alphafetoprotein levels improved.The patient achieved a progression-free survival of 20 months and an overall survival of 29 months.CONCLUSION HAIC can treat high-bilirubin HCC with PVTT and OJ,allowing for subsequent targeted immunotherapy.
文摘It is challenging to diagnose isolated hyperbilirubinemia with rare and complex etiologies under the constraints of traditional testing conditions.Herein,we present a rare case of coexisting Gilbert syndrome(GS)and erythropoietic protoporphyria(EPP),which has not been previously documented.CASE SUMMARY We present a rare case of coexisting GS and EPP in a 23-year-old Chinese male with a long history of jaundice and recently found splenomegaly.Serial nonspecific hemolysis screening tests yielded inconsistent results,and investigations for common hemolytic etiologies were negative.However,Levitt’s carbon monoxide breath test,which measures erythrocyte lifespan(the gold-standard marker of hemolysis),demonstrated significant hemolysis,revealing a markedly shortened erythrocyte lifespan of 11 days(normal average 120 days).Genetic testing subsequently confirmed EPP with a homozygous ferrochelatase gene mutation and GS with a heterozygous uridine diphosphate glucuronosyl trans-ferase 1A1 gene mutation.CONCLUSION The rapid,non-invasive Levitt’s carbon monoxide breath test resolved the diagnostic challenge posed by a rare and complex cause of hyperbilirubinemia.
文摘BACKGROUND While varices and variceal bleeds are well-known and feared complications of advanced cirrhosis and portal hypertension,omental variceal bleed are a rare sequala even in patients with known esophageal or gastric varices.While rare,omental varices pose a risk for hemoperitoneum if ruptured,which is a lifethreatening complication with high mortality rates despite surgical intervention.CASE SUMMARY This report reviews the case of a patient 36-year-old female with alcohol related cirrhosis decompensated by ascites,but no history of varices admitted for hemorrhagic shock from spontaneous rupture of omental varices requiring emergency surgery.She underwent the first documented successful orthotopic liver transplantation the same admission.CONCLUSION This case report and literature review stresses the importance of early consideration and identification of intraabdominal variceal sources in cirrhotic patients with refractory shock.
