Moyamoya disease(MMD),characterized by progressive internal carotid artery stenosis and collateral vessel formation,prompts cerebral perfusion complications and is stratified into idiopathic and Moyamoya syndrome subt...Moyamoya disease(MMD),characterized by progressive internal carotid artery stenosis and collateral vessel formation,prompts cerebral perfusion complications and is stratified into idiopathic and Moyamoya syndrome subtypes.A multifa-ceted approach toward MMD management addresses cerebral infarctions through revascularization surgery and adjunctive medical therapy,while also navigating risks such as intracranial hemorrhage and cerebral infarction resulting from arte-rial stenosis and fragile collateral vessels.Addressing antithrombotic management reveals a potential role for treatments like antiplatelet agents and anticoagulants,despite the ambiguous contribution of thrombosis to MMD-related infarctions and the critical balance between preventing ischemic events and averting hemo-rrhagic complications.Transcranial doppler has proven useful in thromboembolic detection,despite persisting challenges concerning the efficacy and safety of an-tithrombotic treatments.Furthermore,antihypertensive interventions aim to ma-nage blood pressure meticulously,especially during intracerebral hemorrhage,with recommendations and protocols varying based on the patient’s hypertension status.Additionally,lipid-lowering therapeutic strategies,particularly employing statins,are appraised for their possible beneficial role in MMD management,even as comprehensive data from disease-specific clinical trials remains elusive.Com-prehensive guidelines and protocols to navigate the multifaceted therapeutic ave-nues for MMD,while maintaining a delicate balance between efficacy and safety,warrant further meticulous research and development.This protocol manuscript seeks to elucidate the various aspects and challenges imbued in managing and navigating through the complex landscape of MMD treatment.展开更多
Although intracranial hemorrhage in moyamoya disease can occur repeatedly,predicting the disease is difficult.Deep learning algorithms developed in recent years provide a new angle for identifying hidden risk factors,...Although intracranial hemorrhage in moyamoya disease can occur repeatedly,predicting the disease is difficult.Deep learning algorithms developed in recent years provide a new angle for identifying hidden risk factors,evaluating the weight of different factors,and quantitatively evaluating the risk of intracranial hemorrhage in moyamoya disease.To investigate whether convolutional neural network algorithms can be used to recognize moyamoya disease and predict hemorrhagic episodes,we retrospectively selected 460 adult unilateral hemispheres with moyamoya vasculopathy as positive samples for diagnosis modeling,including 418 hemispheres with moyamoya disease and 42 hemispheres with moyamoya syndromes.Another 500 hemispheres with normal vessel appearance were selected as negative samples.We used deep residual neural network(ResNet-152)algorithms to extract features from raw data obtained from digital subtraction angiography of the internal carotid artery,then trained and validated the model.The accuracy,sensitivity,and specificity of the model in identifying unilateral moyamoya vasculopathy were 97.64±0.87%,96.55±3.44%,and 98.29±0.98%,respectively.The area under the receiver operating characteristic curve was 0.990.We used a combined multi-view conventional neural network algorithm to integrate age,sex,and hemorrhagic factors with features of the digital subtraction angiography.The accuracy of the model in predicting unilateral hemorrhagic risk was 90.69±1.58%and the sensitivity and specificity were 94.12±2.75%and 89.86±3.64%,respectively.The deep learning algorithms we proposed were valuable and might assist in the automatic diagnosis of moyamoya disease and timely recognition of the risk for re-hemorrhage.This study was approved by the Institutional Review Board of Huashan Hospital,Fudan University,China(approved No.2014-278)on January 12,2015.展开更多
The etiology and pathogenesis of moyamoya disease（MMD） remain elusive. Some inflammatory proteins, such as cyclooxygenase（COX）-2, are believed to be implicated in the development of MMD. So far, the relationship b...The etiology and pathogenesis of moyamoya disease（MMD） remain elusive. Some inflammatory proteins, such as cyclooxygenase（COX）-2, are believed to be implicated in the development of MMD. So far, the relationship between COX-2 and MMD is poorly understood and reports on the intracranial vessels of MMD patients are scanty. In this study, tiny pieces of middle cerebral artery（MCA） and superficial temporal artery（STA） from 13 MMD patients were surgically harvested. The MCA and STA samples from 5 control patients were also collected by using the same technique. The expression of COX-2 was immunohistochemically detected and the average absorbance（A） of positively-stained areas was measured. High-level COX-2 expression was found in all layers of the MCA samples from all 5 hemorrhagic MMD patients, while positive but weak expression of COX-2 was observed only in the endothelial layer of the MCA samples from most ischemic MMD patients（6/8, 75%）. The average A values of COX-2 in the hemorrhagic MMD patients were substantially higher than those in their ischemic counterparts（t=4.632, P=0.001）. There was no significant difference in the COX-2 expression among the ＂gender＂ groups, or ＂radiographic grade＂ groups, or ＂lesion location＂ groups（P0.05 for all）. The COX-2 expression was detected neither in the MCA samples from the controls nor in all STA specimens. Our results suggested that COX-2 was up-regulated in the MCA of MMD patients, especially in hemorrhagic MMD patients. We are led to speculate that COX-2 may be involved in the pathogenesis of MMD and even contribute to the hemorrhagic stroke of MMD patients.展开更多
BACKGROUND Ménétrier’s disease is a rare condition characterized by enlarged gastric folds,usually located in the whole body and fundus of the stomach.This report presents an unusual case of localized M...BACKGROUND Ménétrier’s disease is a rare condition characterized by enlarged gastric folds,usually located in the whole body and fundus of the stomach.This report presents an unusual case of localized Ménétrier’s disease elevated by a submucosal lipoma and thus looking like a polypoid mass and causing an episode of upper gastrointestinal bleeding.The mass was successfully removed with endoscopic submucosal dissection.CASE SUMMARY Esophagogastroduodenoscopy was performed on a 76-year-old male patient after an episode of upper gastrointestinal bleeding,manifesting as fatigue and melena.A large polypoid mass(4 cm×1 cm)with enlarged mucosal folds was found in the body of the stomach,between the lesser curvature and posterior wall.A small ulcer at the distal end of the mass was identified as the source of the bleeding.Biopsy was negative for neoplasia.Computed tomography showed a submucosal lesion beneath the affected mucosa,most likely a lipoma.The mass was removed en bloc with tunneling endoscopic submucosal dissection.Final pathology determined that the mass included Ménétrier’s disease and a submucosal lipoma.The patient was scheduled for follow-up esophagogastroduodenoscopy.CONCLUSION Localized Ménétrier’s disease can coexist with a submucosal lipoma creating a polypoid mass with risk of bleeding.展开更多
Severe gastrointestinal (GI) hemorrhage is a rare complication of Crohn’s disease (CD). Although several surgical and non-surgical approaches have been described over the last 2 decades this complication s...Severe gastrointestinal (GI) hemorrhage is a rare complication of Crohn’s disease (CD). Although several surgical and non-surgical approaches have been described over the last 2 decades this complication still poses significant diagnostic and therapeutic challenges. Given the relative infrequency of severe bleeding in CD, available medical literature on this topic is mostly in the form of retrospective case series and reports. In this article we review the risk factors, diagnostic modalities and treatment options for the management of CD presenting as GI hemorrhage.展开更多
Behcet's disease(BD) is a rare and life-long disorder characterized by inflammation of blood vessels throughout the body. BD was originally described in 1937 as a syndrome involving oral and genital ulceration in ...Behcet's disease(BD) is a rare and life-long disorder characterized by inflammation of blood vessels throughout the body. BD was originally described in 1937 as a syndrome involving oral and genital ulceration in addition to ocular inflammation. Intestinal BD refers to colonic ulcerative lesions documented by objective measures in patients with BD. Many studies have shown that over 40% of BD patients have gastrointestinal complaints. Symptoms include abdominal pain, diarrhea, nausea, anorexia and abdominal distension. Although gastrointestinal symptoms are common, the demonstration of gastrointestinal ulcers is rare. This so-called intestinal BD accounts for approximately 1% of cases. There is no specific test for BD, and the diagnosis is based on clinical criteria. The manifestations of intestinal BD are similar to those of other colitis conditions such as Crohn's disease or intestinal tuberculosis, thus, it is challenging for gastroenterologists to accurately diagnose intestinal BD in patients with ileocolonic ulcers. However, giant ulcers distributed in the esophagus and ileocecal junction with gastrointestinal hemorrhage are rare in intestinal BD. Here, we present a case of untypical intestinal BD. The patient had recurrent aphthous ulceration of the oral mucosa, and esophageal and ileo-colonic ulceration, but no typical extra-intestinal symptoms. During examination, the patient had massive acute lower gastrointestinal bleeding. The patient underwent ileostomy after an emergency right hemicolectomy and partial ileectomy, and was subsequently diagnosed with incomplete-type intestinal BD by pathology. The literature on the evaluation and management of this condition is reviewed.展开更多
BACKGROUND Acute severe lower gastrointestinal bleeding(LGIB)is an uncommon but challenging complication of Crohn’s disease(CD).AIM To identify the predictors of acute severe LGIB and to evaluate the impact of acute ...BACKGROUND Acute severe lower gastrointestinal bleeding(LGIB)is an uncommon but challenging complication of Crohn’s disease(CD).AIM To identify the predictors of acute severe LGIB and to evaluate the impact of acute severe LGIB on the subsequent clinical course in CD patients.METHODS A retrospective inception cohort study was conducted in 75 CD patients with acute severe LGIB and 1359 CD patients without acute severe LGIB who were diagnosed between February 1991 and November 2019 at Asan Medical Center,a tertiary university hospital in Korea.Multivariable analysis with Cox proportional hazard regression was performed to identify the risk factors for acute severe LGIB.A matched analysis using 72 patients with bleeding and 267 matched patients without within the cohort was also conducted to investigate whether acute severe LGIB is a predictor of clinical outcomes of CD.RESULTS Multivariable Cox regression analysis revealed that early use of thiopurines[hazard ratio(HR):0.23,95%confidence interval(CI):0.12-0.48;P<0.001]and female sex(HR:0.51,95%CI:0.27-0.94;P=0.031)were significantly associated with a lower risk of acute severe LGIB.The cumulative risks of behavioral progression and intestinal resection were not significantly different between the two matched groups(P=0.139 and P=0.769,respectively).The hospitalization rate was higher in the bleeding group than in the matched non-bleeding group(22.1/100 vs 13.2/100 patient-years;P=0.012).However,if hospitalizations due to bleeding episodes were excluded from the analysis,the hospitalization rate was not significantly different between the bleeding group and the matched nonbleeding group(14.5/100 vs 13.2/100 patient-years;P=0.631).CONCLUSION Early use of thiopurines may reduce the risk of acute severe LGIB.History of acute severe LGIB may not have a significant prognostic value in patients with CD.展开更多
Capsule endoscopy(CE) is a simple,safe,non-invasive,reliable technique,well accepted and tolerated by the patients,which allows complete exploration of the small intestine.The advent of CE in 2000 has dramatically cha...Capsule endoscopy(CE) is a simple,safe,non-invasive,reliable technique,well accepted and tolerated by the patients,which allows complete exploration of the small intestine.The advent of CE in 2000 has dramatically changed the diagnosis and management of many diseases of the small intestine,such as obscure gastrointestinal bleeding,Crohn's disease,small bowel tumors,polyposis syndromes,etc.CE has become the gold standard for the diagnosis of most diseases of the small bowel.Lately this technique has also been used for esophageal and colonic diseases.展开更多
文摘Moyamoya disease(MMD),characterized by progressive internal carotid artery stenosis and collateral vessel formation,prompts cerebral perfusion complications and is stratified into idiopathic and Moyamoya syndrome subtypes.A multifa-ceted approach toward MMD management addresses cerebral infarctions through revascularization surgery and adjunctive medical therapy,while also navigating risks such as intracranial hemorrhage and cerebral infarction resulting from arte-rial stenosis and fragile collateral vessels.Addressing antithrombotic management reveals a potential role for treatments like antiplatelet agents and anticoagulants,despite the ambiguous contribution of thrombosis to MMD-related infarctions and the critical balance between preventing ischemic events and averting hemo-rrhagic complications.Transcranial doppler has proven useful in thromboembolic detection,despite persisting challenges concerning the efficacy and safety of an-tithrombotic treatments.Furthermore,antihypertensive interventions aim to ma-nage blood pressure meticulously,especially during intracerebral hemorrhage,with recommendations and protocols varying based on the patient’s hypertension status.Additionally,lipid-lowering therapeutic strategies,particularly employing statins,are appraised for their possible beneficial role in MMD management,even as comprehensive data from disease-specific clinical trials remains elusive.Com-prehensive guidelines and protocols to navigate the multifaceted therapeutic ave-nues for MMD,while maintaining a delicate balance between efficacy and safety,warrant further meticulous research and development.This protocol manuscript seeks to elucidate the various aspects and challenges imbued in managing and navigating through the complex landscape of MMD treatment.
基金This study was supported by the National Natural Science Foundation of China,Nos.81801155(to YL),81771237(to YXG)the New Technology Projects of Shanghai Science and Technology Innovation Action Plan,China,No.18511102800(to YXG)+1 种基金the Shanghai Municipal Science and Technology Major Project and ZJLab,China,No.2018SHZDZX01(to YM)the Shanghai Health and Family Planning Commission,China,No.2017BR022(to YXG).
文摘Although intracranial hemorrhage in moyamoya disease can occur repeatedly,predicting the disease is difficult.Deep learning algorithms developed in recent years provide a new angle for identifying hidden risk factors,evaluating the weight of different factors,and quantitatively evaluating the risk of intracranial hemorrhage in moyamoya disease.To investigate whether convolutional neural network algorithms can be used to recognize moyamoya disease and predict hemorrhagic episodes,we retrospectively selected 460 adult unilateral hemispheres with moyamoya vasculopathy as positive samples for diagnosis modeling,including 418 hemispheres with moyamoya disease and 42 hemispheres with moyamoya syndromes.Another 500 hemispheres with normal vessel appearance were selected as negative samples.We used deep residual neural network(ResNet-152)algorithms to extract features from raw data obtained from digital subtraction angiography of the internal carotid artery,then trained and validated the model.The accuracy,sensitivity,and specificity of the model in identifying unilateral moyamoya vasculopathy were 97.64±0.87%,96.55±3.44%,and 98.29±0.98%,respectively.The area under the receiver operating characteristic curve was 0.990.We used a combined multi-view conventional neural network algorithm to integrate age,sex,and hemorrhagic factors with features of the digital subtraction angiography.The accuracy of the model in predicting unilateral hemorrhagic risk was 90.69±1.58%and the sensitivity and specificity were 94.12±2.75%and 89.86±3.64%,respectively.The deep learning algorithms we proposed were valuable and might assist in the automatic diagnosis of moyamoya disease and timely recognition of the risk for re-hemorrhage.This study was approved by the Institutional Review Board of Huashan Hospital,Fudan University,China(approved No.2014-278)on January 12,2015.
基金supported by grants from the National Natural Science Foundation of China(No.81571146)the Key Project of the Natural Science Foundation of Hubei Province of China(No.ZRZ2014000254)
文摘The etiology and pathogenesis of moyamoya disease（MMD） remain elusive. Some inflammatory proteins, such as cyclooxygenase（COX）-2, are believed to be implicated in the development of MMD. So far, the relationship between COX-2 and MMD is poorly understood and reports on the intracranial vessels of MMD patients are scanty. In this study, tiny pieces of middle cerebral artery（MCA） and superficial temporal artery（STA） from 13 MMD patients were surgically harvested. The MCA and STA samples from 5 control patients were also collected by using the same technique. The expression of COX-2 was immunohistochemically detected and the average absorbance（A） of positively-stained areas was measured. High-level COX-2 expression was found in all layers of the MCA samples from all 5 hemorrhagic MMD patients, while positive but weak expression of COX-2 was observed only in the endothelial layer of the MCA samples from most ischemic MMD patients（6/8, 75%）. The average A values of COX-2 in the hemorrhagic MMD patients were substantially higher than those in their ischemic counterparts（t=4.632, P=0.001）. There was no significant difference in the COX-2 expression among the ＂gender＂ groups, or ＂radiographic grade＂ groups, or ＂lesion location＂ groups（P0.05 for all）. The COX-2 expression was detected neither in the MCA samples from the controls nor in all STA specimens. Our results suggested that COX-2 was up-regulated in the MCA of MMD patients, especially in hemorrhagic MMD patients. We are led to speculate that COX-2 may be involved in the pathogenesis of MMD and even contribute to the hemorrhagic stroke of MMD patients.
文摘BACKGROUND Ménétrier’s disease is a rare condition characterized by enlarged gastric folds,usually located in the whole body and fundus of the stomach.This report presents an unusual case of localized Ménétrier’s disease elevated by a submucosal lipoma and thus looking like a polypoid mass and causing an episode of upper gastrointestinal bleeding.The mass was successfully removed with endoscopic submucosal dissection.CASE SUMMARY Esophagogastroduodenoscopy was performed on a 76-year-old male patient after an episode of upper gastrointestinal bleeding,manifesting as fatigue and melena.A large polypoid mass(4 cm×1 cm)with enlarged mucosal folds was found in the body of the stomach,between the lesser curvature and posterior wall.A small ulcer at the distal end of the mass was identified as the source of the bleeding.Biopsy was negative for neoplasia.Computed tomography showed a submucosal lesion beneath the affected mucosa,most likely a lipoma.The mass was removed en bloc with tunneling endoscopic submucosal dissection.Final pathology determined that the mass included Ménétrier’s disease and a submucosal lipoma.The patient was scheduled for follow-up esophagogastroduodenoscopy.CONCLUSION Localized Ménétrier’s disease can coexist with a submucosal lipoma creating a polypoid mass with risk of bleeding.
文摘Severe gastrointestinal (GI) hemorrhage is a rare complication of Crohn’s disease (CD). Although several surgical and non-surgical approaches have been described over the last 2 decades this complication still poses significant diagnostic and therapeutic challenges. Given the relative infrequency of severe bleeding in CD, available medical literature on this topic is mostly in the form of retrospective case series and reports. In this article we review the risk factors, diagnostic modalities and treatment options for the management of CD presenting as GI hemorrhage.
文摘Behcet's disease(BD) is a rare and life-long disorder characterized by inflammation of blood vessels throughout the body. BD was originally described in 1937 as a syndrome involving oral and genital ulceration in addition to ocular inflammation. Intestinal BD refers to colonic ulcerative lesions documented by objective measures in patients with BD. Many studies have shown that over 40% of BD patients have gastrointestinal complaints. Symptoms include abdominal pain, diarrhea, nausea, anorexia and abdominal distension. Although gastrointestinal symptoms are common, the demonstration of gastrointestinal ulcers is rare. This so-called intestinal BD accounts for approximately 1% of cases. There is no specific test for BD, and the diagnosis is based on clinical criteria. The manifestations of intestinal BD are similar to those of other colitis conditions such as Crohn's disease or intestinal tuberculosis, thus, it is challenging for gastroenterologists to accurately diagnose intestinal BD in patients with ileocolonic ulcers. However, giant ulcers distributed in the esophagus and ileocecal junction with gastrointestinal hemorrhage are rare in intestinal BD. Here, we present a case of untypical intestinal BD. The patient had recurrent aphthous ulceration of the oral mucosa, and esophageal and ileo-colonic ulceration, but no typical extra-intestinal symptoms. During examination, the patient had massive acute lower gastrointestinal bleeding. The patient underwent ileostomy after an emergency right hemicolectomy and partial ileectomy, and was subsequently diagnosed with incomplete-type intestinal BD by pathology. The literature on the evaluation and management of this condition is reviewed.
文摘BACKGROUND Acute severe lower gastrointestinal bleeding(LGIB)is an uncommon but challenging complication of Crohn’s disease(CD).AIM To identify the predictors of acute severe LGIB and to evaluate the impact of acute severe LGIB on the subsequent clinical course in CD patients.METHODS A retrospective inception cohort study was conducted in 75 CD patients with acute severe LGIB and 1359 CD patients without acute severe LGIB who were diagnosed between February 1991 and November 2019 at Asan Medical Center,a tertiary university hospital in Korea.Multivariable analysis with Cox proportional hazard regression was performed to identify the risk factors for acute severe LGIB.A matched analysis using 72 patients with bleeding and 267 matched patients without within the cohort was also conducted to investigate whether acute severe LGIB is a predictor of clinical outcomes of CD.RESULTS Multivariable Cox regression analysis revealed that early use of thiopurines[hazard ratio(HR):0.23,95%confidence interval(CI):0.12-0.48;P<0.001]and female sex(HR:0.51,95%CI:0.27-0.94;P=0.031)were significantly associated with a lower risk of acute severe LGIB.The cumulative risks of behavioral progression and intestinal resection were not significantly different between the two matched groups(P=0.139 and P=0.769,respectively).The hospitalization rate was higher in the bleeding group than in the matched non-bleeding group(22.1/100 vs 13.2/100 patient-years;P=0.012).However,if hospitalizations due to bleeding episodes were excluded from the analysis,the hospitalization rate was not significantly different between the bleeding group and the matched nonbleeding group(14.5/100 vs 13.2/100 patient-years;P=0.631).CONCLUSION Early use of thiopurines may reduce the risk of acute severe LGIB.History of acute severe LGIB may not have a significant prognostic value in patients with CD.
文摘Capsule endoscopy(CE) is a simple,safe,non-invasive,reliable technique,well accepted and tolerated by the patients,which allows complete exploration of the small intestine.The advent of CE in 2000 has dramatically changed the diagnosis and management of many diseases of the small intestine,such as obscure gastrointestinal bleeding,Crohn's disease,small bowel tumors,polyposis syndromes,etc.CE has become the gold standard for the diagnosis of most diseases of the small bowel.Lately this technique has also been used for esophageal and colonic diseases.
