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Diffuse intestinal ganglioneuromatosis an uncommon manifestation of Cowden syndrome 被引量:5
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作者 Maria Teresa Herranz Bachiller Jesus Barrio Andrés +7 位作者 Fernando Pons Noelia Alcaide Suárez Rafael Ruiz-Zorrilla Lorena Sancho del Val Sara Lorenzo Pelayo Carlos De La Serna Higuera Ramon Atienza Sánchez Manuel Perez Miranda 《World Journal of Gastrointestinal Oncology》 SCIE CAS 2013年第2期34-37,共4页
Diffuse intestinal ganglioneuromatosis is a hamartomatous polyposis characterized by a disseminated, intramural or transmural proliferation of neural elements involving the enteric plexuses. It has been associated wit... Diffuse intestinal ganglioneuromatosis is a hamartomatous polyposis characterized by a disseminated, intramural or transmural proliferation of neural elements involving the enteric plexuses. It has been associated with MEN Ⅱ, neurofibromatosis type 1 and hamartomatous polyposis associated with phosphatase and tensin homolog mutation. We report the case of a female patient with a history of a breast and endometrial tumor who presented in a colonoscopy performed for rectal bleeding diffuse ganglioneuromatosis, which oriented the search for other characteristic findings of Cowden syndrome given the personal history of the patient. The presence of an esophagogastric polyposis was also noted. Cowden syndrome is characterized by skin lesions, but it is rarely diagnosed by these lesions, because they are usually overlooked. Intestinal polyposis is not a major diagnostic criterion but it is very useful for early diagnosis. The combination of colonic polyposis and glucogenic acanthosis should orient the diagnosis to Cowden syndrome. 展开更多
关键词 Ganglioneuromatosis Gastrointestinal poliposis PHOsPHATAsE and TENsIN HOMOLOG cowden síndrome HAMARTOMA
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Testicular Lipomatosis without Evidence of Cowden’s Disease
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作者 Ahmad Rezayi Azandariani Leili Ebrahimi Sedigheh Saedi 《Case Reports in Clinical Medicine》 2015年第12期364-368,共5页
Background: Testicular lipomatosis is a very rare and benign disorder of the testicles. It usually presents as multiple bilateral ill-defined hyper-echoic intra-testicular lesions of different sizes but generally with... Background: Testicular lipomatosis is a very rare and benign disorder of the testicles. It usually presents as multiple bilateral ill-defined hyper-echoic intra-testicular lesions of different sizes but generally with maximum 4 mm. Testicular lipomatosis is usually reported in association with Cowden’s syndrome. Aim: We aimed to show that there are cases of testicular lipomatosis occurring in the absence of Cowden’s syndrome. Case Presentation: We present a 28-year-old man with testicular pain, who was finally diagnosed as having isolated testicular lipomatosis without other clinical and biochemical abnormalities using magnetic resonance imaging (MRI) technique. Conclusion: We showed that the testicular lipomatosis may occur and be detected without any evidence of Cowden’s disease. 展开更多
关键词 TEsTICULAR LIPOMATOsIs cowdens DIsEAsE MAGNETIC REsONANCE Imaging
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Electrocardiographic changes during induced therapeutic hypothermia in comatose survivors after cardiac arrest 被引量:4
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作者 Pablo Salinas Esteban Lopez-de-Sa +4 位作者 Laura Pena-Conde Ana Viana-Tejedor Juan Ramon Rey-Blas Eduardo Armada Jose Luis Lopez-Sendon 《World Journal of Cardiology》 CAS 2015年第7期423-430,共8页
AIM: To assess the safety of therapeutic hypothermia(TH) concerning arrhythmias we analyzed serial electrocardiograms(ECG) during TH.METHODS: All patients recovered from a cardiac arrest with Glasgow < 9 at admissi... AIM: To assess the safety of therapeutic hypothermia(TH) concerning arrhythmias we analyzed serial electrocardiograms(ECG) during TH.METHODS: All patients recovered from a cardiac arrest with Glasgow < 9 at admission were treated with induced mild TH to 32-34℃. TH was obtained with cool fluid infusion or a specific intravascular device. Twelvelead ECG before,during,and after TH,as well as ECG telemetry data was recorded in all patients. From a total of 54 patients admitted with cardiac arrest during the study period,47 patients had the 3 ECG and telemetry data available. ECG analysis was blinded and performed with manual caliper by two independent cardiologists from blinded copies of original ECG,recorded at 25 mm/s and 10 mm/m V. Coronary care unit staff analyzed ECG telemetry for rhythm disturbances. Variables measured in ECG were rhythm,RR,PR,QT and corrected QT(QTc by Bazett formula,measured in lead v2) intervals,QRS duration,presence of Osborn's J wave and U wave,as well as ST segment displacement and T wave amplitude in leads Ⅱ,v2 and v5.RESULTS: Heart rate went down an average of 19 bpm during hypothermia and increased again 16 bpm with rewarming(P < 0.0005,both). There was a nonsignificant prolongation of the PR interval during TH and a significant decrease with rewarming(P = 0.041). QRS duration significantly prolonged(P = 0.041) with TH and shortened back(P < 0.005) with rewarming. QTc interval presented a mean prolongation of 58 ms(P < 0.005) during TH and a significant shortening with rewarming of 22.2 ms(P = 0.017). Osborn or J wave was found in 21.3% of the patients. New arrhythmias occurred in 38.3% of the patients. Most frequent arrhythmia was non-sustained ventricular tachycardia(19.1%),followed by severe bradycardia or paced rhythm(10.6%),accelerated nodal rhythm(8.5%) and atrial fibrillation(6.4%). No life threatening arrhythmias(sustained ventricular tachycardia,polymorphic ventricular tachycardia or ventricular fibrillation) occurred during TH. CONCLUSION: A 38.3% of patients had cardiac arrhythmias during TH but without life-threatening arrhythmias. A concern may rise when inducing TH to patients with long QT syndrome. 展开更多
关键词 CARDIAC ARREsT Therapeutic HYPOTHERMIA Post-cardiac ARREsT síndrome CARDIAC arrythmias QT INTERVAL
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胎儿左心室发育不良综合征并肺静脉共同腔闭锁尸检1例 被引量:5
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作者 闫风彩 商建峰 +2 位作者 刘冬梅 陈东 昌红 《诊断病理学杂志》 CSCD 2016年第11期833-835,共3页
目的探讨1例左心发育不良综合征并肺静脉共同腔闭锁胎儿的临床资料、超声分析及尸检解剖学特点。方法分析超声心动检查发现的复杂胎儿先天性心脏畸形病例,引产后进行尸体病理解剖,心、血管畸形按心脏节段分析方法进行分析和总结。结果... 目的探讨1例左心发育不良综合征并肺静脉共同腔闭锁胎儿的临床资料、超声分析及尸检解剖学特点。方法分析超声心动检查发现的复杂胎儿先天性心脏畸形病例,引产后进行尸体病理解剖,心、血管畸形按心脏节段分析方法进行分析和总结。结果胎儿左心发育不良综合征为复杂的先天性心脏畸形,同时合并肺静脉共同腔闭锁的病例更罕见,母体超声心动图可描述比较显著的解剖及生理特征,引产后详细的尸体解剖可进一步明确诊断。本例可见房间隔缺如、单心房、左心室腔小壁厚,二尖瓣闭锁及肺静脉共同腔闭锁,而且胎儿与婴幼儿的肺静脉共同腔闭锁不同,组织学不伴有肺淋巴管扩张。结论胎儿左心发育不良综合征合并肺静脉共同腔闭锁为少见的先天性心血管畸形,诊断更加困难。 展开更多
关键词 先天性心脏病 左心发育不良综合征 肺静脉共同腔闭锁 淋巴管扩张
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