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Contrast echocardiography for detection of pulmonary arteriovenous malformations

AMERICAN HEART JOURNAL 2001年第2期141卷 243-246页

作者： Nanthakumar, K Graham, AT Robinson, TI Grande, P Pugash, RA Clarke, JA Hutchison, SJ Mandzia, JL Hyland, RH Faughnan, ME Univ Toronto St Michaels Hosp Div Cardiol Toronto ON M5B 1W8 Canada Univ Toronto St Michaels Hosp Div Radiol Toronto ON M5B 1W8 Canada Univ Toronto St Michaels Hosp Div Respirol Toronto ON M5B 1W8 Canada

Background Pulmonary arteriovenous malformations (PAVMs) leed to stroke, brain abscess, and hemorrhage in hereditary hemorrhagic telangiectasia (HHT). The current screening approach for PAVMs in HHT patients with chest radiograph (CXR) and oxygen shunt study has not been validated and is thought to be insensitive. We hypothesized that agitated saline contrast echocardiography (ECHO) would be a useful screening test for PAVMs. Methods and results A total of 106 sequential HHT patients underwent screening for PAVMs with ECHO in a prospective study. If the test was positive, or if the CXR or shunt study suggested PAVMs, pulmonary angiography was performed. A positive ECHO was defined as appearance of bubbles in the left atrium after injection of agitated saline solution. A positive shunt study was defined as a partial pressure of oxygen in arterial blood <500 mm Hg while breathing 100% oxygen. The mean age was 41 years (range 15-80 years);66% were female. Forty-four patients had positive ECHO. Forty-one of the 44 patients underwent angiography. Three patients declined further testing. Thirty-three of the 41 patients who underwent angiography were diagnosed with PAVMs. Of the 62 patients with a negative ECHO, 18 underwent angiography because of either a shunt study or CXR that was suggestive of PAVMs. Of these 18 patients, 2 had PAVMs. In the total population of 106 patients, 35 (33%) had PAVMs. ECHO was the only positive screening test in 11 of 35 (31%) patients. The diagnosis of PAVMs in these 11 patients would have otherwise been missed. Conclusions ECHO is a useful screening tool for PAVMs in HHT.

关键词：造影剂/投药和剂量诊断鉴别超声心动描记术/方法心房/超声检查注射静脉内肺/血液供给肺/超声检查观察者偏差前瞻性研究肺动脉/畸形肺动脉/放射摄影术肺动脉/超声检查肺静脉/畸形肺静脉/放射摄影术肺静脉/超声检查氯化钠/投药和剂量氯化钠/诊断应用毛细血管扩张遗传性出血性/并发症毛细血管扩张遗传性出血性/超声检查女(雌)性人类男(雄)性中年人

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A treatment algorithm for the management of epistaxis in hereditary hemorrhagic telangiectasia

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AMERICAN JOURNAL OF RHINOLOGY 1999年第4期13卷 319-322页

作者： Lund, VJ Howard, DJ Inst Laryngol & Otol London WC1X 8DA England

We examined the management options used for epistaxis of varying severity in patients with hereditary, hemorrhagic telangiectasia (HHT) to develop a treatment algorithm. Fifty patients with HHT were studied from 1991 to 1997 to consider various treatment options and their relative efficacy. There were 28 females and 22 men;their ages ranged from 15 to 85. Follow-up ranged from 6 months to 6 years. A wide variety of treatments had been used (including various forms of cautery, septal surgery, topical and systemic hormones, antifibrinolytic therapy, arterial ligation, laser therapy, and closure of the nostrils). Treatments used during the follow-up period were argon laser therapy (40), systemic estrogen or progesterone (17), septodermoplasty (12), or closure of the nostrils (14). Patients were divided into those with mild to moderate disease (requiring one or no blood transfusions during the course of their illness) and those with severe disease (requiring more than one blood transfusion). Using this broad categorization, those with mild to moderate disease did well with sequential argon laser therapy with or without the adjunct of septodermoplasty or hormone therapy. Those with severe disease did not find argon laser therapy beneficial but found their epistaxis completely slopped with closure of the nasal cavities. Based on these findings, a simple treatment algorithm defines the optimum treatment options for epistaxis in this difficult-to-treat group of patients.

关键词：算法抗纤维蛋白溶解药/治疗应用动脉/外科学输血烧灼术队列研究鼻出血/药物疗法鼻出血/外科学鼻出血/治疗雌二醇同源物/治疗应用炔雌醇/治疗应用随访研究激光凝固术结扎术甲羟孕酮/治疗应用鼻腔/外科学鼻中隔/外科学鼻窦/血液供给孕酮同源物/治疗应用毛细血管扩张遗传性出血性/并发症青少年成年人老年人老年人 80以上女(雌)性人类男(雄)性中年人

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遗传性出血性毛细血管扩张症累及肝脏一例

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中国医师杂志 2011年第4期13卷 575-576页

作者：夏喜斌罗建光谭长连中南大学湘雅二医院放射科长沙410011

患者，女，21岁，因反复呕血并黑便半年余，加重半月于2010年8月27日入院。患者半年前无明显诱因出现呕血、黑便，在当地医院诊断“上消化道出血”予以抗炎、制酸、止血等对症治疗后，症状稍缓解，半月前上述症状加重，于对症治疗症状... 详细信息

患者，女，21岁，因反复呕血并黑便半年余，加重半月于2010年8月27日入院。患者半年前无明显诱因出现呕血、黑便，在当地医院诊断“上消化道出血”予以抗炎、制酸、止血等对症治疗后，症状稍缓解，半月前上述症状加重，于对症治疗症状无明显缓解。家族史：患者一姐姐因反复鼻出血，全身多处毛细血管扩张，有胃出血病史。查体：神志清，前胸、背部，肘关节等部位可见成簇的鲜红色和紫红色斑点，压之不完全褪色。影像表现：腹部CT及CTA表现：CT增强扫描见门静咏和肝静脉早显（图1），门静脉增宽，

关键词：毛细血管扩张遗传性出血性/并发症肝疾病/病因学病例报告

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遗传性出血性毛细血管扩张症伴肺动静脉瘘一例

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中华儿科杂志 1981年第4期19卷 236-237页

作者：史延芳中国医科大学附属第三医院儿科

遗传性出血性毛细血管扩张症(Osler-Ren-du-Weber 氏病)比较少见,且易误诊。国内从1961年到1978年报告38例,除皮肤和粘膜毛细血管扩张外,主要症状为鼻衄和/或消化道出血,仅2例出现血尿,2例伴侏儒症及肝硬

关键词：病例报告动静脉瘘/并发症肺疾病/并发症毛细血管扩张遗传性出血性/并发症儿童人类

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HEREDITARY HEMORRHAGIC TELANGIECTASIA WITH GINGIVAL LESION - REVIEW AND CASE-REPORTS

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JOURNAL OF PERIODONTOLOGY 1976年第5期47卷 295-298页

作者： EVERETT, FG HAHN, CR UNIV OREGON HLTH SCI CTRSCH DENTDEPT PERIODONTOLPORTLANDOR 97201 UNIV OREGON HLTH SCI CTRSCH MEDDEPT DERMATOLPORTLANDOR 97201

A case of hereditary, hemorrhagic telangiectasia is described which has been under observation for more than 8 years. The clinical findings are discussed; light microscopic and electron microscopic findings as well as current research for a causative mechanism and recommended therapy are reviewed. The patient exhibited gingival telangiectatic participation. Another, even more severe case of this disease showed repeated, severe hemorrhage from mucosal lesions in the region of the incisive papilla, severe internal bleeding from the nasal mucosa and the gut and several punctate gingival telangiectases. The occurance of gingival lesions may not be as rare as thought. Such lesions may simply be rather inconspicuous.

关键词：牙龈疾病/病因学牙龈疾病/病理学口腔疾病/病因学口腔疾病/病理学毛细血管扩张遗传性出血性/并发症毛细血管扩张遗传性出血性/病理学女(雌)性人类男(雄)性中年人

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Clinical diagnosis: Intrahepatic arteriovenous fistula associated with hereditary hemorrhagic telangiectasia

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ANNALS OF NUCLEAR MEDICINE 2002年第7期16卷 490-490页

作者： Suga, K Yamaguchi Univ Sch Med Dept Radiol Yamaguchi Japan

Abstract not available.

关键词：动静脉瘘/病因学动静脉瘘/放射性核素显像肝动脉/畸形肝动脉/放射性核素显像肝静脉/畸形肝静脉/放射性核素显像有机锝化合物/诊断应用植酸/诊断应用放射性核素血管显像术/方法放射性药物/诊断应用毛细血管扩张遗传性出血性/并发症毛细血管扩张遗传性出血性/放射性核素显像成年人女(雌)性人类

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