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Managing adult-onset Still's disease in pregnancy:A case report
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作者 Ji-Hyoun Kang 《World Journal of Clinical Cases》 SCIE 2024年第16期2837-2841,共5页
BACKGROUND Adult-onset Still’s disease(AOSD)is a rare systemic inflammatory disorder characterized by fever,arthritis,skin rash,and systemic symptoms.The etiology of AOSD is unknown;however,it is thought to be relate... BACKGROUND Adult-onset Still’s disease(AOSD)is a rare systemic inflammatory disorder characterized by fever,arthritis,skin rash,and systemic symptoms.The etiology of AOSD is unknown;however,it is thought to be related to immune dysregulation.Although a rare disease,AOSD can significantly impact reproductive health,particularly during pregnancy.This case study assesses the implications of pregnancy in a patient with AOSD,as well as the potential for heredity of the disease.Neonatal hemophagocytic lympho-histiocytosis(HLH)is a rare and lifethreatening disorder characterized by hyperinflammation and uncontrolled activation of immune cells,leading to multiple organ dysfunction.This case report aimed to introduce neonatal HLH from a mother with AOSD.CASE SUMMARY This case study presents a 29-year-old female with AOSD who became pregnant and gave birth to a premature infant who was diagnosed with neonatal HLH.AOSD can significantly impact pregnancy and childbirth,as it may become more severe during pregnancy,with an increased risk of fetal loss and preterm birth.The management of AOSD during pregnancy involves the use of nonsteroidal anti-inflammatory drugs and glucocorticoids,as well as immunosuppressive agents in severe cases.However,the use of immunosuppressive agents during pregnancy may be associated with potential risks to the fetus.The hereditary implications of AOSD are unclear;however,available evidence suggests that genetic factors may play a role in the disease development.CONCLUSION AOSD can have significant implications for pregnancy and childbirth,including an increased risk of fetal loss and preterm birth.Neonatal HLH,a complication of AOSD in pregnancy,requires prompt diagnosis and management.Women with AOSD who are considering pregnancy should discuss their options with their healthcare provider and develop a management plan that addresses the potential risks to both mother and fetus. 展开更多
关键词 NEONATE Hemophagocytic lympho-histiocytosis adult-onset still’s disease PREGNANCY Case report
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Multimodal imaging diagnosis and analysis of prognostic factors in patients with adult-onset Coats disease
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作者 Wei Zhou Hui Zhou +6 位作者 Yuan-Yuan Liu Meng-Xuan Li Xiao-Han Wu Jiao Liang Jing Hao Sheng-Nan Liu Chun-Jie Jin 《International Journal of Ophthalmology(English edition)》 SCIE CAS 2024年第8期1469-1476,共8页
AIM:To describe the multimodal imaging features,treatment,and outcomes of patients diagnosed with adultonset Coats disease.METHODS:This retrospective study included patients first diagnosed with Coats disease at≥18 y... AIM:To describe the multimodal imaging features,treatment,and outcomes of patients diagnosed with adultonset Coats disease.METHODS:This retrospective study included patients first diagnosed with Coats disease at≥18 years of age between September 2017 and September 2021.Some patients received anti-vascular endothelial growth factor(VEGF)therapy(conbercept,0.5 mg)as the initial treatment,which was combined with laser photocoagulation as needed.All the patients underwent best corrected visual acuity(BCVA)and intraocular pressure examinations,fundus color photography,spontaneous fluorescence tests,fundus fluorescein angiography,optical coherence tomography(OCT),OCT angiography,and other examinations.BCVA alterations and multimodal image findings in the affected eyes following treatment were compared and the prognostic factors were analyzed.RESULTS:The study included 15 patients who were aged 24-72(57.33±12.61)y at presentation.Systemic hypertension was the most common associated systemic condition,occurring in 13(86.7%)patients.Baseline BCVA ranged from 2.0 to 5.0(4.0±1.1),which showed improvement following treatment(4.2±1.0).Multimodal imaging revealed retinal telangiectasis in 13 patients(86.7%),patchy hemorrhage in 5 patients(33.3%),and stage 2B disease(Shield’s staging criteria)in 11 patients(73.3%).OCT revealed that the baseline central macular thickness(CMT)ranged from 129 to 964μm(473.0±230.1μm),with 13 patients(86.7%)exhibiting a baseline CMT exceeding 250μm.Furthermore,8 patients(53.3%)presented with an epiretinal membrane at baseline or during follow-up.Hyper-reflective scars were observed on OCT in five patients(33.3%)with poor visual prognosis.Vision deteriorated in one patient who did not receive treatment.Final vision was stable in three patients who received laser treatment,whereas improvement was observed in one of two patients who received anti-VEGF therapy alone.In addition,8 of 9 patients(88.9%)who received laser treatment and conbercept exhibited stable or improved BCVA.CONCLUSION:Multimodal imaging can help diagnose adult-onset Coats disease.Anti-VEGF treatment combined with laser therapy can be an option for improving or maintaining BCVA and resolving macular edema.The final visual outcome depends on macular involvement and the disease stage. 展开更多
关键词 adult-onset Coats disease multimodal imaging anti-vascular endothelial growth factor conbercept
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Adult-Onset Still's Disease Misdiagnosed as Acute Fibrinous and Organizing Pneumonia: A Case Report and Literature Review
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作者 Chunhua Huang Chunting Lai 《Journal of Clinical and Nursing Research》 2024年第6期90-93,共4页
Adult-onset Stil's disease(AOSD)is a rare condition that lies between autoinflammatory syndrome and autoimmune disease.The main clinical manifestations include fever,chills,rash,joint swelling and pain,peripheral ... Adult-onset Stil's disease(AOSD)is a rare condition that lies between autoinflammatory syndrome and autoimmune disease.The main clinical manifestations include fever,chills,rash,joint swelling and pain,peripheral blood leukocytosis,splenomegaly,etc.It is a systemic disease affecting between 1 and 34 people per million.The average age of onset is 35 years old,with a slightly higher prevalence rate in women.Since AOSD lacks early specific symptoms and signs,non-specialist doctors have limited understanding of the disease,and patients are prone to clinical misdiagnosis,mistreatment,and delayed disease progression.This paper reports a patient whose AOSD was misdiagnosed as acute fibrinous and organizing pneumonia. 展开更多
关键词 adult-onset still's disease Acute fibrinous and organizing pneumonia Lung disease
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Hepatitis A virus-associated acute acalculous cholecystitis in an adult-onset Still’s disease patient:A case report and review of the literature 被引量:2
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作者 Chu-Heng Chang You-Yang Wang Yang Jiao 《World Journal of Clinical Cases》 SCIE 2023年第6期1410-1418,共9页
BACKGROUND Acute acalculous cholecystitis(AAC)is inflammation of the gallbladder without evidence of calculi.Although rarely reported,its etiologies include hepatitis virus infection(e.g.,hepatitis A virus,HAV)and adu... BACKGROUND Acute acalculous cholecystitis(AAC)is inflammation of the gallbladder without evidence of calculi.Although rarely reported,its etiologies include hepatitis virus infection(e.g.,hepatitis A virus,HAV)and adult-onset Still’s disease(AOSD).There are no reports of HAV-associated AAC in an AOSD patient.CASE SUMMARY Here we report a rare case of HAV infection-associated AAC in a 39-year-old woman who had a history of AOSD.The patient presented with an acute abdomen and hypotension.Elevated hepatobiliary enzymes and a thickened and distended gallbladder without gallstones on ultrasonography suggested AAC,but there were no signs of anemia nor thrombocytopenia.Serological screening revealed anti-HAV IgM antibodies.Steroid treatment did not alleviate her symptoms,and she was referred for laparoscopic cholecystectomy.The resected gallbladder was hydropic without perforation,and her clinical signs gradually improved after surgery.CONCLUSION AAC can be caused by HAV in AOSD patients.It is crucial to search for the underlying etiology for AAC,especially uncommon viral causes. 展开更多
关键词 Acalculous cholecystitis Hepatitis A virus adult-onset still’s disease Acute abdomen CHOLECYsTECTOMY Case report
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Converging links between adult-onset neurodegenerative Alzheimer’s disease and early life neurodegenerative neuronal ceroid lipofuscinosis? 被引量:1
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作者 Marcel Klein Guido Hermey 《Neural Regeneration Research》 SCIE CAS CSCD 2023年第7期1463-1471,共9页
Evidence from genetics and from analyzing cellular and animal models have converged to suggest links between neurodegenerative disorders of early and late life.Here,we summarize emerging links between the most common ... Evidence from genetics and from analyzing cellular and animal models have converged to suggest links between neurodegenerative disorders of early and late life.Here,we summarize emerging links between the most common late life neurodegenerative disease,Alzheimer’s disease,and the most common early life neurodegenerative diseases,neuronal ceroid lipofuscinoses.Genetic studies reported an overlap of clinically diagnosed Alzheimer’s disease and mutations in genes known to cause neuronal ceroid lipofuscinoses.Accumulating data strongly suggest dysfunction of intracellular trafficking mechanisms and the autophagy-endolysosome system in both types of neurodegenerative disorders.This suggests shared cytopathological processes underlying these different types of neurodegenerative diseases.A better understanding of the common mechanisms underlying the different diseases is important as this might lead to the identification of novel targets for therapeutic concepts,the transfer of therapeutic strategies from one disease to the other and therapeutic approaches tailored to patients with specific mutations.Here,we review dysfunctions of the endolysosomal autophagy pathway in Alzheimer’s disease and neuronal ceroid lipofuscinoses and summarize emerging etiologic and genetic overlaps. 展开更多
关键词 Alzheimer’s disease autophagy Batten disease CLN3 disease dementia ENDOsOME LYsOsOME neurodegeneration neuronal ceroid lipofuscinosis PREsENILIN
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Adult-onset Still's disease evolving with multiple organ failure and death:A case report and review of the literature 被引量:2
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作者 Zhong-Bin Han Ju Wu +3 位作者 Jing Liu He-Ming Li Kai Guo Tong Sun 《World Journal of Clinical Cases》 SCIE 2021年第4期886-897,共12页
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
关键词 adult-onset still's disease Macrophage activation syndrome Disseminated intravascular coagulopathy Multiple organ failure DEATH Case report
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Adult-onset Still's disease: A case report 被引量:1
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作者 Arun Agarwal Darshan N Gondaliya 《Journal of Acute Disease》 2020年第4期179-182,共4页
Rationale: Fever of unknown origin (FUO) is a frequently observed phenomenon in clinical practice. Definite diagnosis of FUO is a great challenge in clinical practice since potential causes for FUO involve more than 2... Rationale: Fever of unknown origin (FUO) is a frequently observed phenomenon in clinical practice. Definite diagnosis of FUO is a great challenge in clinical practice since potential causes for FUO involve more than 200 diseases. Adult-onset Still's disease is a defined clinical entity and a known rare cause of FUO. Patient's concern: A 19-year girl was referred to the clinic with the concern of intermittent fevers and shivering for almost a year despite multiple investigations and consultations. She had undergone intensive serologic, radiologic, laboratory investigations to exclude infectious diseases, connective tissue diseases, and malignancy, and all the investigation showed no conclusive diagnosis. Diagnosis: Adult-onset Stills disease. Intervention: Steroids and supportive treatment. Outcomes:The symptoms were relieved within three days, and the patient became asymptomatic. Lessons: Physicians need to be familiar with the diagnostic criteria of adult-onset Still's disease, or it shall remain a diagnostic dilemma. Besides, all shivers are not infections. 展开更多
关键词 Fever of unknown origin adult-onset still's disease Autoinflammatory disease Yamaguchi criteria Fautrel criteria
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Successful treatment of adult-onset still disease caused by pulmonary infection-associated hemophagocytic lymphohistiocytosis: A case report 被引量:1
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作者 Gui Wang Xiao-Rong Jin De-Xun Jiang 《World Journal of Clinical Cases》 SCIE 2020年第3期560-567,共8页
BACKGROUND Adult-onset still disease(AOSD) and hemophagocytic syndrome(HPS) are two inflammatory diseases with very similar clinical manifestations. HPS is one of the most serious complications of AOSD and its risk of... BACKGROUND Adult-onset still disease(AOSD) and hemophagocytic syndrome(HPS) are two inflammatory diseases with very similar clinical manifestations. HPS is one of the most serious complications of AOSD and its risk of death is very high. It is difficult to identify HPS early in patients with AOSD, but early identification and proper treatment directly affects the prognosis.CASE SUMMARY A 39-year-old male showed a high spiking fever and myalgia. Laboratory data revealed elevated white blood cell, serum ferritin, and neutrophil percentage.However, his fever failed to relieve after a clear diagnosis of AOSD caused by pulmonary infection and treatment by antibiotics and corticosteroids;further laboratory data showed elevated serum ferritin, C-reactive protein, erythrocyte sedimentation rate and triglyceride, as well as liver abnormalities. Bone marrow smear showed hemophagocytosis. Secondary HPS was definitely diagnosed. The high fever disappeared and the laboratory findings returned to normal values after treatment by high-dose intravenous methylprednisolone and methotrexate.CONCLUSION For AOSD patients with high suspicion of HPS, active examination needs to be considered for early diagnosis, and timely using of adequate amount of corticosteroids is the key to reducing risk of HPS death. 展开更多
关键词 Hemophagocytic syndrome adult-onset still disease HEMOPHAGOCYTOsIs Case report
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Atypical adult-onset Still’s disease with an initial and sole manifestation of liver injury: A case report and review of literature
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作者 Fan Yu Shao-You Qin +4 位作者 Chang-Yu Zhou Lu Zhao Yan Xu Er-Na Jia Jiang-Bin Wang 《World Journal of Clinical Cases》 SCIE 2021年第1期224-231,共8页
BACKGROUND Adult-onset Still's disease(AOSD)typically presents with a high spiking fever,polyarthritis,transient maculopapular rash,neutrophilic leukocytosis,and hepatosplenomegaly.It has a wide spectrum of clinic... BACKGROUND Adult-onset Still's disease(AOSD)typically presents with a high spiking fever,polyarthritis,transient maculopapular rash,neutrophilic leukocytosis,and hepatosplenomegaly.It has a wide spectrum of clinical symptoms ranging from mild to severe,with extensive involvement of almost every organ.Although liver involvement in the form of increased hepatic enzymes and bilirubin is common,no AOSD case with liver involvement as the initial manifestation of AOSD has been reported.CASE SUMMARY A 35-year-old woman presented to the hepatology department with progressively worsening jaundice for one week.Liver chemistry tests revealed a significantly increased liver enzymes and bilirubin level.Given that the clinical examination was unremarkable,liver biopsy was considered because the patient had a history of AOSD 6 years ago.Liver histopathology revealed that most hepatic lobules were still recognizable.Fusional necrosis was observed around most central veins.A few bridging necrotic zones were also found.Infiltration of multiple plasma cells were observed in the necrotic zone,and the reticular scaffold was still expanded.Additionally,no obvious fibrosis was observed in the portal area.Mild mixed inflammatory cell infiltration was noted in the interstitium of the portal area.Further examination was unremarkable except for a remarkably high level of ferritin.Collectively,a presumptive diagnosis of liver injury secondary to AOSD was made.The hepatic involvement responded well to glucocorticoid treatment.CONCLUSION This case highlights that hepatic involvement as an initial and sole manifestation could be a pattern of relapsed AOSD.The diagnosis of AOSD should be considered in the case of nonresolving liver injury after the exclusion of common etiologies for liver diseases.A liver biopsy can be useful for the differential diagnosis of liver injury associated with AOSD. 展开更多
关键词 adult-onset still's disease Liver injury Liver biopsy Histopathology Glucocorticoid treatment Case report
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The role of exosomes in adult neurogenesis:implications for neurodegenerative diseases 被引量:2
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作者 Zhuoyang Yu Yan Teng +1 位作者 Jing Yang Lu Yang 《Neural Regeneration Research》 SCIE CAS CSCD 2024年第2期282-288,共7页
Exosomes are cup-shaped extracellular vesicles with a lipid bilayer that is approximately 30 to 200 nm in thickness.Exosomes are widely distributed in a range of body fluids,including urine,blood,milk,and saliva.Exoso... Exosomes are cup-shaped extracellular vesicles with a lipid bilayer that is approximately 30 to 200 nm in thickness.Exosomes are widely distributed in a range of body fluids,including urine,blood,milk,and saliva.Exosomes exert biological function by transporting factors between different cells and by regulating biological pathways in recipient cells.As an important form of intercellular communication,exosomes are increasingly being investigated due to their ability to transfer bioactive molecules such as lipids,proteins,mRNAs,and microRNAs between cells,and because they can regulate physiological and pathological processes in the central nervous system.Adult neurogenesis is a multistage process by which new neurons are generated and migrate to be integrated into existing neuronal circuits.In the adult brain,neurogenesis is mainly localized in two specialized niches:the subventricular zone adjacent to the lateral ventricles and the subgranular zone of the dentate gyrus.An increasing body of evidence indicates that adult neurogenesis is tightly controlled by environmental conditions with the niches.In recent studies,exosomes released from different sources of cells were shown to play an active role in regulating neurogenesis both in vitro and in vivo,thereby participating in the progression of neurodegenerative disorders in patients and in various disease models.Here,we provide a state-of-the-art synopsis of existing research that aimed to identify the diverse components of exosome cargoes and elucidate the therapeutic potential of exosomal contents in the regulation of neurogenesis in several neurodegenerative diseases.We emphasize that exosomal cargoes could serve as a potential biomarker to monitor functional neurogenesis in adults.In addition,exosomes can also be considered as a novel therapeutic approach to treat various neurodegenerative disorders by improving endogenous neurogenesis to mitigate neuronal loss in the central nervous system. 展开更多
关键词 adult neurogenesis Alzheimer’s disease amyotrophic lateral sclerosis EXOsOME Huntington’s disease neurodegenerative disease neurogenic niches Parkinson’s disease
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Mitophagy in neurodegenerative disease pathogenesis 被引量:2
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作者 Kan Yang Yuqing Yan +7 位作者 Anni Yu Ru Zhang Yuefang Zhang Zilong Qiu Zhengyi Li Qianlong Zhang Shihao Wu Fei Li 《Neural Regeneration Research》 SCIE CAS CSCD 2024年第5期998-1005,共8页
Mitochondria are critical cellular energy resources and are central to the life of the neuron.Mitophagy selectively clears damaged or dysfunctional mitochondria through autophagic machinery to maintain mitochondrial q... Mitochondria are critical cellular energy resources and are central to the life of the neuron.Mitophagy selectively clears damaged or dysfunctional mitochondria through autophagic machinery to maintain mitochondrial quality control and homeostasis.Mature neurons are postmitotic and consume substantial energy,thus require highly efficient mitophagy pathways to turn over damaged or dysfunctional mitochondria.Recent evidence indicates that mitophagy is pivotal to the pathogenesis of neurological diseases.However,more work is needed to study mitophagy pathway components as potential therapeutic targets.In this review,we briefly discuss the characteristics of nonselective autophagy and selective autophagy,including ERphagy,aggrephagy,and mitophagy.We then introduce the mechanisms of Parkin-dependent and Parkin-independent mitophagy pathways under physiological conditions.Next,we summarize the diverse repertoire of mitochondrial membrane receptors and phospholipids that mediate mitophagy.Importantly,we review the critical role of mitophagy in the pathogenesis of neurodegenerative diseases including Alzheimer’s disease,Parkinson’s disease,and amyotrophic lateral sclerosis.Last,we discuss recent studies considering mitophagy as a potential therapeutic target for treating neurodegenerative diseases.Together,our review may provide novel views to better understand the roles of mitophagy in neurodegenerative disease pathogenesis. 展开更多
关键词 Alzheimer’s disease amyotrophic lateral sclerosis autophagy mitochondria MITOPHAGY mitophagy receptor PARKIN Parkinson’s disease PINK1
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Targeting tau in Alzheimer's disease:from mechanisms to clinical therapy 被引量:6
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作者 Jinwang Ye Huali Wan +1 位作者 Sihua Chen Gong-Ping Liu 《Neural Regeneration Research》 SCIE CAS CSCD 2024年第7期1489-1498,共10页
Alzheimer’s disease is the most prevalent neurodegenerative disease affecting older adults.Primary features of Alzheimer’s disease include extra cellular aggregation of amyloid-βplaques and the accumulation of neur... Alzheimer’s disease is the most prevalent neurodegenerative disease affecting older adults.Primary features of Alzheimer’s disease include extra cellular aggregation of amyloid-βplaques and the accumulation of neurofibrillary tangles,fo rmed by tau protein,in the cells.While there are amyloid-β-ta rgeting therapies for the treatment of Alzheimer’s disease,these therapies are costly and exhibit potential negative side effects.Mounting evidence suggests significant involvement of tau protein in Alzheimer’s disease-related neurodegeneration.As an important microtubule-associated protein,tau plays an important role in maintaining the stability of neuronal microtubules and promoting axonal growth.In fact,clinical studies have shown that abnormal phosphorylation of tau protein occurs before accumulation of amyloid-βin the brain.Various therapeutic strategies targeting tau protein have begun to emerge,and are considered possible methods to prevent and treat Alzheimer’s disease.Specifically,abnormalities in post-translational modifications of the tau protein,including aberrant phosphorylation,ubiquitination,small ubiquitin-like modifier(SUMO)ylation,acetylation,and truncation,contribute to its microtubule dissociation,misfolding,and subcellular missorting.This causes mitochondrial damage,synaptic impairments,gliosis,and neuroinflammation,eventually leading to neurodegeneration and cognitive deficits.This review summarizes the recent findings on the underlying mechanisms of tau protein in the onset and progression of Alzheimer’s disease and discusses tau-targeted treatment of Alzheimer’s disease. 展开更多
关键词 ACETYLATION Alzheimer’s disease cognitive deficits GLIOsIs mitochondria damage NEUROINFLAMMATION phosphorylation synaptic impairments TAU tau immunotherapy
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Olfactory dysfunction and its related molecular mechanisms in Parkinson’s disease 被引量:3
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作者 Yingying Gu Jiaying Zhang +4 位作者 Xinru Zhao Wenyuan Nie Xiaole Xu Mingxuan Liu Xiaoling Zhang 《Neural Regeneration Research》 SCIE CAS CSCD 2024年第3期583-590,共8页
Changes in olfactory function are considered to be early biomarkers of Parkinson’s disease.Olfactory dysfunction is one of the earliest non-motor features of Parkinson’s disease,appearing in about 90%of patients wit... Changes in olfactory function are considered to be early biomarkers of Parkinson’s disease.Olfactory dysfunction is one of the earliest non-motor features of Parkinson’s disease,appearing in about 90%of patients with early-stage Parkinson’s disease,and can often predate the diagnosis by years.Therefore,olfactory dysfunction should be considered a reliable marker of the disease.However,the mechanisms responsible for olfactory dysfunction are currently unknown.In this article,we clearly explain the pathology and medical definition of olfactory function as a biomarker for early-stage Parkinson’s disease.On the basis of the findings of clinical olfactory function tests and animal model experiments as well as neurotransmitter expression levels,we further characterize the relationship between olfactory dysfunction and neurodegenerative diseases as well as the molecular mechanisms underlying olfactory dysfunction in the pathology of early-stage Parkinson’s disease.The findings highlighted in this review suggest that olfactory dysfunction is an important biomarker for preclinical-stage Parkinson’s disease.Therefore,therapeutic drugs targeting non-motor symptoms such as olfactory dysfunction in the early stage of Parkinson’s disease may prevent or delay dopaminergic neurodegeneration and reduce motor symptoms,highlighting the potential of identifying effective targets for treating Parkinson’s disease by inhibiting the deterioration of olfactory dysfunction. 展开更多
关键词 BIOMARKER EARLY-sTAGE olfactory disorders olfactory dysfunction Parkinson’s disease
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Diagnostic delay in inflammatory bowel diseases in a German population 被引量:4
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作者 Elisabeth Blüthner Annalena Dehe +11 位作者 Carsten Büning Britta Siegmund Matthias Prager Jochen Maul Alexander Krannich Jan Preiß Bertram Wiedenmann Florian Rieder Raneem Khedraki Frank Tacke Andreas Sturm Anja Schirbel 《World Journal of Gastroenterology》 SCIE CAS 2024年第29期3465-3478,共14页
BACKGROUND Early diagnosis is key to prevent bowel damage in inflammatory bowel disease(IBD).Risk factor analyses linked with delayed diagnosis in European IBD patients are scarce and no data in German IBD patients ex... BACKGROUND Early diagnosis is key to prevent bowel damage in inflammatory bowel disease(IBD).Risk factor analyses linked with delayed diagnosis in European IBD patients are scarce and no data in German IBD patients exists.AIM To identify risk factors leading to prolonged diagnostic time in a German IBD cohort.METHODS Between 2012 and 2022,430 IBD patients from four Berlin hospitals were enrolled in a prospective study and asked to complete a 16-item questionnaire to determine features of the path leading to IBD diagnosis.Total diagnostic time was defined as the time from symptom onset to consulting a physician(patient waiting time)and from first consultation to IBD diagnosis(physician diagnostic time).Univariate and multivariate analyses were performed to identify risk factors for each time period.RESULTS The total diagnostic time was significantly longer in Crohn’s disease(CD)compared to ulcerative colitis(UC)patients(12.0 vs 4.0 mo;P<0.001),mainly due to increased physician diagnostic time(5.5 vs 1.0 mo;P<0.001).In a multivariate analysis,the predominant symptoms diarrhea(P=0.012)and skin lesions(P=0.028)as well as performed gastroscopy(P=0.042)were associated with longer physician diagnostic time in CD patients.In UC,fever was correlated(P=0.020)with shorter physician diagnostic time,while fatigue(P=0.011)and positive family history(P=0.046)were correlated with longer physician diagnostic time.CONCLUSION We demonstrated that CD patients compared to UC are at risk of long diagnostic delay.Future efforts should focus on shortening the diagnostic delay for a better outcome in these patients. 展开更多
关键词 Diagnostic time Diagnostic delay Crohn’s disease Ulcerative colitis GERMANY
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Effects of mesenchymal stem cell on dopaminergic neurons,motor and memory functions in animal models of Parkinson's disease:a systematic review and meta-analysis 被引量:4
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作者 Jong Mi Park Masoud Rahmati +2 位作者 Sang Chul Lee Jae Il Shin Yong Wook Kim 《Neural Regeneration Research》 SCIE CAS CSCD 2024年第7期1584-1592,共9页
Parkinson’s disease is chara cterized by the loss of dopaminergic neurons in the substantia nigra pars com pacta,and although restoring striatal dopamine levels may improve symptoms,no treatment can cure or reve rse ... Parkinson’s disease is chara cterized by the loss of dopaminergic neurons in the substantia nigra pars com pacta,and although restoring striatal dopamine levels may improve symptoms,no treatment can cure or reve rse the disease itself.Stem cell therapy has a regenerative effect and is being actively studied as a candidate for the treatment of Parkinson’s disease.Mesenchymal stem cells are considered a promising option due to fewer ethical concerns,a lower risk of immune rejection,and a lower risk of teratogenicity.We performed a meta-analysis to evaluate the therapeutic effects of mesenchymal stem cells and their derivatives on motor function,memory,and preservation of dopamine rgic neurons in a Parkinson’s disease animal model.We searched bibliographic databases(PubMed/MEDLINE,Embase,CENTRAL,Scopus,and Web of Science)to identify articles and included only pee r-reviewed in vivo interve ntional animal studies published in any language through J une 28,2023.The study utilized the random-effect model to estimate the 95%confidence intervals(CI)of the standard mean differences(SMD)between the treatment and control groups.We use the systematic review center for laboratory animal expe rimentation’s risk of bias tool and the collaborative approach to meta-analysis and review of animal studies checklist for study quality assessment.A total of 33studies with data from 840 Parkinson’s disease model animals were included in the meta-analysis.Treatment with mesenchymal stem cells significantly improved motor function as assessed by the amphetamine-induced rotational test.Among the stem cell types,the bone marrow MSCs with neurotrophic factor group showed la rgest effect size(SMD[95%CI]=-6.21[-9.50 to-2.93],P=0.0001,I^(2)=0.0%).The stem cell treatment group had significantly more tyrosine hydroxylase positive dopamine rgic neurons in the striatum([95%CI]=1.04[0.59 to 1.49],P=0.0001,I^(2)=65.1%)and substantia nigra(SMD[95%CI]=1.38[0.89 to 1.87],P=0.0001,I^(2)=75.3%),indicating a protective effect on dopaminergic neurons.Subgroup analysis of the amphetamine-induced rotation test showed a significant reduction only in the intracranial-striatum route(SMD[95%CI]=-2.59[-3.25 to-1.94],P=0.0001,I^(2)=74.4%).The memory test showed significant improvement only in the intravenous route(SMD[95%CI]=4.80[1.84 to 7.76],P=0.027,I^(2)=79.6%).Mesenchymal stem cells have been shown to positively impact motor function and memory function and protect dopaminergic neurons in preclinical models of Parkinson’s disease.Further research is required to determine the optimal stem cell types,modifications,transplanted cell numbe rs,and delivery methods for these protocols. 展开更多
关键词 ANIMAL animal experimentation mesenchymal stem cells models Parkinson’s disease stem cell transplantation
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Activation of autophagy by Citri Reticulatae Semen extract ameliorates amyloid-beta-induced cell death and cognition deficits in Alzheimer’s disease 被引量:3
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作者 Yong Tang Jing Wei +14 位作者 Xiao-Fang Wang Tao Long Xiaohong Xiang Liqun Qu Xingxia Wang Chonglin Yu Xingli Xiao Xueyuan Hu Jing Zeng Qin Xu Anguo Wu Jianming Wu Dalian Qin Xiaogang Zhou Betty Yuen-Kwan Law 《Neural Regeneration Research》 SCIE CAS CSCD 2024年第11期2467-2479,共13页
Amyloid-beta-induced neuronal cell death contributes to cognitive decline in Alzheimer’s disease.Citri Reticulatae Semen has diverse beneficial effects on neurodegenerative diseases,including Parkinson’s and Hunting... Amyloid-beta-induced neuronal cell death contributes to cognitive decline in Alzheimer’s disease.Citri Reticulatae Semen has diverse beneficial effects on neurodegenerative diseases,including Parkinson’s and Huntington’s diseases,however,the effect of Citri Reticulatae Semen on Alzheimer’s disease remains unelucidated.In the current study,the anti-apoptotic and autophagic roles of Citri Reticulatae Semen extract on amyloid-beta-induced apoptosis in PC12 cells were first investigated.Citri Reticulatae Semen extract protected PC12 cells from amyloid-beta-induced apoptosis by attenuating the Bax/Bcl-2 ratio via activation of autophagy.In addition,Citri Reticulatae Semen extract was confirmed to bind amyloid-beta as revealed by biolayer interferometry in vitro,and suppress amyloid-beta-induced pathology such as paralysis,in a transgenic Caenorhabditis elegans in vivo model.Moreover,genetically defective Caenorhabditis elegans further confirmed that the neuroprotective effect of Citri Reticulatae Semen extract was autophagy-dependent.Most importantly,Citri Reticulatae Semen extract was confirmed to improve cognitive impairment,neuronal injury and amyloid-beta burden in 3×Tg Alzheimer’s disease mice.As revealed by both in vitro and in vivo models,these results suggest that Citri Reticulatae Semen extract is a potential natural therapeutic agent for Alzheimer’s disease via its neuroprotective autophagic effects. 展开更多
关键词 Alzheimer’s disease AMYLOID-BETA apoptosis AUTOPHAGY Caenorhabditis elegans Citri Reticulatae semen
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May ChatGPT be a tool producing medical information for common inflammatory bowel disease patients’questions?An evidencecontrolled analysis 被引量:3
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作者 Antonietta Gerarda Gravina Raffaele Pellegrino +6 位作者 Marina Cipullo Giovanna Palladino Giuseppe Imperio Andrea Ventura Salvatore Auletta Paola Ciamarra Alessandro Federico 《World Journal of Gastroenterology》 SCIE CAS 2024年第1期17-33,共17页
Artificial intelligence is increasingly entering everyday healthcare.Large language model(LLM)systems such as Chat Generative Pre-trained Transformer(ChatGPT)have become potentially accessible to everyone,including pa... Artificial intelligence is increasingly entering everyday healthcare.Large language model(LLM)systems such as Chat Generative Pre-trained Transformer(ChatGPT)have become potentially accessible to everyone,including patients with inflammatory bowel diseases(IBD).However,significant ethical issues and pitfalls exist in innovative LLM tools.The hype generated by such systems may lead to unweighted patient trust in these systems.Therefore,it is necessary to understand whether LLMs(trendy ones,such as ChatGPT)can produce plausible medical information(MI)for patients.This review examined ChatGPT’s potential to provide MI regarding questions commonly addressed by patients with IBD to their gastroenterologists.From the review of the outputs provided by ChatGPT,this tool showed some attractive potential while having significant limitations in updating and detailing information and providing inaccurate information in some cases.Further studies and refinement of the ChatGPT,possibly aligning the outputs with the leading medical evidence provided by reliable databases,are needed. 展开更多
关键词 Crohn’s disease Ulcerative colitis Inflammatory bowel disease Chat Generative Pre-trained Transformer Large language model Artificial intelligence
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Crohn’s disease as the intestinal manifestation of pan-lymphatic dysfunction:An exploratory proposal based on basic and clinical data 被引量:2
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作者 Yu-Wei Zhou Yue Ren +16 位作者 Miao-Miao Lu Ling-Ling Xu Wei-Xin Cheng Meng-Meng Zhang Lin-Ping Ding Dong Chen Jian-Guo Gao Juan Du Ci-Liang Jin Chun-Xiao Chen Yun-Fei Li Tao Cheng Peng-Lei Jiang Yi-Da Yang Peng-Xu Qian Peng-Fei Xu Xi Jin 《World Journal of Gastroenterology》 SCIE CAS 2024年第1期34-49,共16页
Crohn’s disease(CD)is caused by immune,environmental,and genetic factors.It can involve the entire gastrointestinal tract,and although its prevalence is rapidly increasing its etiology remains unclear.Emerging biolog... Crohn’s disease(CD)is caused by immune,environmental,and genetic factors.It can involve the entire gastrointestinal tract,and although its prevalence is rapidly increasing its etiology remains unclear.Emerging biological and small-molecule drugs have advanced the treatment of CD;however,a considerable proportion of patients are non-responsive to all known drugs.To achieve a breakthrough in this field,innovations that could guide the further development of effective therapies are of utmost urgency.In this review,we first propose the innovative concept of pan-lymphatic dysfunction for the general distribution of lymphatic dysfunction in various diseases,and suggest that CD is the intestinal manifestation of pan-lymphatic dysfunction based on basic and clinical preliminary data.The supporting evidence is fully summarized,including the existence of lymphatic system dysfunction,recognition of the inside-out model,disorders of immune cells,changes in cell plasticity,partial overlap of the underlying mechanisms,and common gut-derived fatty and bile acid metabolism.Another benefit of this novel concept is that it proposes adopting the zebrafish model for studying intestinal diseases,especially CD,as this model is good at presenting and mimicking lymphatic dysfunction.More importantly,the ensuing focus on improving lymphatic function may lead to novel and promising therapeutic strategies for CD. 展开更多
关键词 Inflammatory bowel disease Crohn’s disease Lymphatic system Inside-out model Immune cells ZEBRAFIsH
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Antisense therapy:a potential breakthrough in the treatment of neurodegenerative diseases 被引量:1
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作者 Roberta Romano Cecilia Bucci 《Neural Regeneration Research》 SCIE CAS CSCD 2024年第5期1027-1035,共9页
Neurodegenerative diseases are a group of disorders characterized by the progressive degeneration of neurons in the central or peripheral nervous system.Currently,there is no cure for neurodegenerative diseases and th... Neurodegenerative diseases are a group of disorders characterized by the progressive degeneration of neurons in the central or peripheral nervous system.Currently,there is no cure for neurodegenerative diseases and this means a heavy burden for patients and the health system worldwide.Therefore,it is necessary to find new therapeutic approaches,and antisense therapies offer this possibility,having the great advantage of not modifying cellular genome and potentially being safer.Many preclinical and clinical studies aim to test the safety and effectiveness of antisense therapies in the treatment of neurodegenerative diseases.The objective of this review is to summarize the recent advances in the development of these new technologies to treat the most common neurodegenerative diseases,with a focus on those antisense therapies that have already received the approval of the U.S.Food and Drug Administration. 展开更多
关键词 Alzheimer’s disease amyotrophic lateral sclerosis antisense oligonucleotide Huntington’s disease neurodegenerative disorders Parkinson’s disease sIRNA
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Predicting short-term major postoperative complications in intestinal resection for Crohn’s disease:A machine learning-based study 被引量:6
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作者 Fang-Tao Wang Yin Lin +8 位作者 Xiao-Qi Yuan Ren-Yuan Gao Xiao-Cai Wu Wei-Wei Xu Tian-Qi Wu Kai Xia Yi-Ran Jiao Lu Yin Chun-Qiu Chen 《World Journal of Gastrointestinal Surgery》 SCIE 2024年第3期717-730,共14页
BACKGROUND Due to the complexity and numerous comorbidities associated with Crohn’s disease(CD),the incidence of postoperative complications is high,significantly impacting the recovery and prognosis of patients.Cons... BACKGROUND Due to the complexity and numerous comorbidities associated with Crohn’s disease(CD),the incidence of postoperative complications is high,significantly impacting the recovery and prognosis of patients.Consequently,additional stu-dies are required to precisely predict short-term major complications following intestinal resection(IR),aiding surgical decision-making and optimizing patient care.AIM To construct novel models based on machine learning(ML)to predict short-term major postoperative complications in patients with CD following IR.METHODS A retrospective analysis was performed on clinical data derived from a patient cohort that underwent IR for CD from January 2017 to December 2022.The study participants were randomly allocated to either a training cohort or a validation cohort.The logistic regression and random forest(RF)were applied to construct models in the training cohort,with model discrimination evaluated using the area under the curves(AUC).The validation cohort assessed the performance of the constructed models.RESULTS Out of the 259 patients encompassed in the study,5.0%encountered major postoperative complications(Clavien-Dindo≥III)within 30 d following IR for CD.The AUC for the logistic model was 0.916,significantly lower than the AUC of 0.965 for the RF model.The logistic model incorporated a preoperative CD activity index(CDAI)of≥220,a diminished preoperative serum albumin level,conversion to laparotomy surgery,and an extended operation time.A nomogram for the logistic model was plotted.Except for the surgical approach,the other three variables ranked among the top four important variables in the novel ML model.CONCLUSION Both the nomogram and RF exhibited good performance in predicting short-term major postoperative complic-ations in patients with CD,with the RF model showing more superiority.A preoperative CDAI of≥220,a di-minished preoperative serum albumin level,and an extended operation time might be the most crucial variables.The findings of this study can assist clinicians in identifying patients at a higher risk for complications and offering personalized perioperative management to enhance patient outcomes. 展开更多
关键词 Crohn’s disease Postoperative complications NOMOGRAM Random forest Intestinal resection
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