小儿先天性动静脉瘘的诊治

目的 探讨对小儿先天性动静脉瘘(congenital arteriovenous fistula，CAVF)的诊断与治疗。方法 本组共收治15例CAVF患儿，男6例、女9例，年龄8～14岁。对全部患儿行多普勒超声，动脉造影或MRA检查。发现病变局限者5例，病变范围广泛性者10例，病变大多发生于四肢。动脉造影显示：动静脉之间出现瘘支；动脉分支多而紊乱，可呈碗蜒扭曲状，病变多为弥漫性；静脉象早期出现。对11例患儿行手术治疗，其中4例局限性病变者行病灶切除。对7例病变广泛者行1或多次减量手术，方法是沿动脉主干切除瘘支与病变组织，对分流量大又无法切除的病变组织予以缝扎，以减少分流量。对5例患儿行栓塞治疗(其中2例为术前栓塞治疗以减少手术出血)。1例未治疗。结果 随访1～6年。5例局限性患儿中3例治愈，2例病情控制。10例广泛性病变中，5例病情好转或控制，5例病情仍继续发展。结论 影像学检查是CAVF诊断与治疗的依据。CAVF治疗困难。尤其对广泛性病变者，无论手术或栓塞治疗疗效均欠理想。但对小儿CAVF应尽早治疗，这样可控制病情发展．防止并发症发生。     

Gastric angiodysplasia in a hereditary hemorrhagic telangiectasia type 2 patient

Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal-dominantly inherited disease that occurs in approximately one in 5000 to 8000 people. Clinical diagnosis of HHT is made when a person presents three of the following four criteria: family history, recurrent nosebleeds, mucocutaneous telangiectasis, and arteriovenous malformations (AVM) in the brain, lung, liver and gastrointestinal (GI) tract. Although epistaxis is the most common presenting symptom, AVMs affecting the lungs, brain and GI tract provoke a more serious outcome. Heterozygous mutations in endoglin, activin receptor-like kinase 1 (ACVRL1; ALK1), and SMAD4, the genes involved in the transforming growth factor-β family signaling cascade, cause HHT. We report here the case of a 63 year-old male patient who presented melena and GI bleeding episodes, proven to be caused by bleeding from multiple gastric angiodysplasia. Esophagogastroduodenoscopy revealed multiple angiodysplasia throughout the stomach. Endoscopic argon plasma coagulation was performed to control bleeding from a gastric angiodysplasia. The patient has been admitted several times with episodes of hemoptysis and hematochezia. One year ago, the patient was hospitalized due to right-sided weakness, which was caused by left basal ganglia hemorrhage as the part of HHT presentation. In family history, the patient's mother and elder sister had died, due to intracranial hemorrhage, and his eldest son has been suffered from recurrent epistaxis for 20 years. A genetic study revealed a mutation in exon 3 of ALK1 (c.199C > T; p.Arg67Trp) in the proband and his eldest son presenting epistaxis.     

Estenose aórtica grave: associações esquecidas

The authors present the case of a 68‐year‐old man with predominantly right heart failure in the context of severe aortic stenosis associated with pulmonary hypertension. Anemia was diagnosed which, after endoscopic study, was considered to be secondary to angiodysplasia and a diagnosis of Heyde syndrome was made.After valve replacement surgery the patient's heart failure improved and hemoglobin levels stabilized.We present this case to show the need to recognize less common associations of severe aortic stenosis, in order to provide immediate and appropriate treatment.     

彩色多普勒血流显像对椎动脉发育不良的检测

目的探讨椎动脉先天性发育不良对椎动脉颅外段血流动力学的影响。方法选择2008年4月1日-6月30日,首都医科大学宣武医院血管超声诊断科应用多普勒血流显像（CDFI）,连续检测可疑后循环缺血的脑血管病患者5500例,从中筛查出一侧椎动脉发育不良者288例。比较双侧椎动脉椎间隙段的管径、收缩期峰值流速（PSV）、舒张期流速（EDV）、阻力指数（RI）及血流频谱的差异。结果①椎动脉发育不良侧平均管径为（2．21±0．20）mm,与健侧管径（3．39±0．47）mm比较,差异有统计学意义（P〈0．01）。②288例患者中,发育不良侧管径≤2．0mm者61例（21．2％）,2．0mm〈管径≤2．5mm者227例（78．8％）,P〈0．01;左侧发育不良者81例（28．1％）,右侧207例（71．9％）,P〈0．01;女性162例（56．3％）,男性126例（43．7％）,P〈0．05。③发育不良侧椎动脉椎间隙段PSV、EDV及RI为（52±11）、（17±6）cm／s及0．66±0．07,健侧分别为（59±10）、（23±5）cm／s及0．61±0．06,均P〈0．01。发育不良侧椎动脉椎间隙段为高阻力型血流频谱改变。结论椎动脉发育不良可引起椎动脉血流动力学变化。     

左旋精氨酸甲酯对大鼠膈疝模型肺血管发育的影响

目的 通过对大鼠孕期一氧化氮(NO)合成的抑制,探讨一氧化氮/环磷酸鸟苷(NO/cGMP)通路在大鼠膈疝肺动脉高压发病中的意义及部分膈疝患者NO吸入效果不佳的原因.方法 随机取孕9.5 d SD大鼠13只,3只作为正常对照组(C组),给予橄榄油2 ml灌入胃内,10只予异草醚(nitrofen)橄榄油溶液(200 mg/只)灌入胃内.左旋精氨酸甲酯干预分小剂量组、大剂量组(分别为L-NAME 1组、L-NAME 2组)进行,每组各4只,自孕14 d开始分别皮下注射左旋精氨酸甲酯(L-NAME)溶液250 mg/kg/d、500 mg/kg/d至孕20 d,对照组(CL组)3只于同样时间段给予生理盐水注射.分析各组血管计数、中膜厚度百分比(MT%),同时对内皮细胞源性一氧化氮合成酶(eNOS)表达进行免疫组化检测.结果 L-NAME 1组、L-NAME 2组与CL组胎鼠成模率、生存率比较,差异无统计学意义;L-NAME 1组、L-NAME2组与CL组血管计数、MT%均较C组明显增高,但L-NAME 1组、L-NAME 2组与CL组之间相比,无显著差异;膈疝发生后,eNOS阳性表达率降低;使用L-NAME后eNOS阳性表达率进一步减低.结论 阻断NO/cGMP通路,膈疝胎鼠肺小动脉的结构未出现相应变化.NO/cGMP通路的变化仅为肺动脉压力改变的功能性因素之一,调节NO/cGMP通路无法独立影响肺动脉的压力.     

Outcome in obscure gastrointestinal bleeding after capsule endoscopy

AIM: To investigate the clinical impact of capsule endoscopy (CE) after an obscure gastrointestinal bleeding (OGIB) episode, focusing on diagnostic work-up, follow-up and predictive factors of rebleeding.METHODS: Patients who were referred to Hospital del Mar (Barcelona, Spain) between 2007 and 2009 for OGIB who underwent a CE were retrospectively analyzed. Demographic data, current treatment with non-steroid anti-inflammtory drugs or anticoagulant drugs, hemoglobin levels, transfusion requirements, previous diagnostic tests for the bleeding episode, as well as CE findings (significant or non-significant), work-up and patient outcomes were analyzed from electronic charts. Variables were compared by χ² analysis and Student t test. Risk factors of rebleeding were assessed by Log-rank test, Kaplan-Meier curves and Cox regression model.RESULTS: There were 105 patients [45.7% women, median age of 72 years old (interquartile range 56-79)] and a median follow-up of 326 d (interquartile range 123-641) included in this study. The overall diagnostic yield of CE was 58.1% (55.2% and 63.2%, for patients with occult OGIB and overt OGIB, respectively). In 73 patients (69.5%), OGIB was resolved. Multivariate analysis showed that hemoglobin levels lower than 8 g/dL at diagnosis [hazard ratios (HR) = 2.7, 95%CI: 1.9-6.3], patients aged 70 years and above (HR = 2.1, 95%CI: 1.2-6.1) and significant findings in CE (HR = 2.4, 95%CI: 1.1-5.8) were independent predictors of rebleeding.CONCLUSION: One third of the patients presented with rebleeding after CE; risk factors were hemoglobin levels < 8 g/dL, age ≥ 70 years or the presence of significant lesions.     

Angiodysplasia of the right colon treated by embolization with ivalon (polyvinyl alcohol)

Angiodysplasia of the colon is being recognized with increasing frequency as a source of lower gastrointestinal bleeding in the elderly. In one patient with colonic angiodysplasia, superselective segmental embolization of the ileo-colic artery with Ivalon resulted in the cessation of bleeding without causing bowel infarction. However, because of the development of collateral branches to the angiodysplastic malformation, bleeding recurred. While colectomy remains the treatment of choice in younger patients, in high risk patients embolization with a permanent embolic material, such as Ivalon, is an attractive alternative.     

Differential diagnosis of early opacification of the portal vein and its tributaries during arteriography

Twenty-two cases with communication of an artery and the portal vein or one of its tributaries are discussed. Four conditions in which relatively significant arterio-portal shunts may exist can be differentiated: (1) angiodysplasias or arteriovenous malformations, (3) traumatic and postoperative lesions, and (4) benign and malignant tumors. The significance of the portal vein's early opacification during arteriographic examinations of the abdominal organs is discussed, and the findings are compared to those reported in the literature.     

Intestinale Angiodysplasie als Ursache schwerer Dünndarmblutungen — Bericht über vier Fälle

Summary Angiodysplasia of the small intestine alone cannot be differentiated histologically from hereditary hemorrhagic telangiectasia (Morbus Rendu-Osler-Weber). Isolated localisation in the small intestine is rare and requires surgery in the event of massive bleeding. Four cases of bleeding from angiodysplasias of the small intestine in patients having undergone surgery at our institution over a ten-year period are presented. In three patients the particular intestine was resected. Two of these patients had an uneventful follow-up of even and ten years, one patient relapsed and was rehospitalized for surgical treatment. The fourth patient, who showed angiodysplasias scattered over the entire small intestine, had no further bleeding over a three-year period after transmural ligations of the lesions found by intraoperative endoscopical diaphany.

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