Takayasu's arteritis on steroid therapy. Seven years follow-up

The authors report a 7 year follow-up of Takayasu's arteritis (TA) type III, group 1, in a young Italian woman. At diagnosis, at the age of 25, the echotomographic and angiographic studies showed narrow subclavian arteries, narrow abdominal aorta (diameter of 0.6-0.8 cm) below the renal arteries, stenotic left common carotid and renal arteries, and occluded upper mesenteric artery. With steroid therapy, (prednisone 50 mg/day per os), the erythrocyte sedimentation rate (ESR) normalized within 12 days. With a maintenance dosage of 7.5 mg/day per os, the patient achieved remission as documented by the absence of symptoms, the persistent normalization of ESR, and the improving of the diameter of the abdominal aorta (1.3-1.4 cm). On steroid therapy, the patient had a normal pregnancy and delivered a healthy baby girl. The disease has been stable for seven years. Recently, diabetes mellitus occurred and it has been treated with insulin therapy. The rising of ESR after tapering of steroid therapy (prednisone 5 mg per os on alternate days) suggests an alternative treatment with a cytotoxic agent.     

Widespread thrombosis and necrotizing arteritis of the liver in a 69-year-old woman with symptoms simulating those of temporal arteritis

Autopsy findings of widespread thrombosis and necrotizing arteritis of the liver in a 69-year-old woman who had clinical symptoms suggesting those of temporal (giant cell) arteritis were presented. The lesion of the temporal artery was not of temporal arteritis but of thrombosis, part of widespread thrombosis, which occurred in the small arteries having hypertensive arteriolopathy. This case also had an arteritis of the liver resembling polyarteritis nodosa (PN). The pathologic processes demonstrated in this case suggested that the temporal arteritis-like symptoms may be brought about by occlusion of the temporal artery. Temporal arteritis is a nonfatal and self-limited disease and little information has been obtained from autopsy material. Accordingly, it is necessary to collect autopsy cases of temporal arteritis to investigate this disease through observations of whole bodies. Recently we examined a case which appeared clinically having symptoms of temporal arteritis, but the autopsy revealed that the symptoms were not brought about by temporal arteritis but by thrombosis of the temporal artery. It is the purpose of this paper to present this case and to discuss some problems concerning genesis of symptoms of temporal arteritis.     

Coronary artery disease in Takayasu's disease. Report of an operative case]

A 39-years-old woman with Takayasu's arteritis confirmed by histological examination was admitted in our hospital because of angina pectoris. Selective coronary arteriograms reveled a 90% isolated stenosis in the right coronary ostium. The patient underwent a right coronary artery bypass grafting using the saphenious vein. The post operative clinical course was uncomplicated. On follow-up over 14 months the patient has been completely free from anginal chest-pain.     

Takayasu arteritis: a chronic vasculitis that is rare in children

Takayasu arteritis was diagnosed in two children, a 15-year-old girl and a 10-year-old boy. The girl had suffered from fatigue, malaise, abdominal pain and weight loss for several months, but no clear cause could be found. A few weeks later, when a blood pressure of 222/155 mmHg was measured, the possibility of renal artery stenosis was considered and imaging studies revealed indications for Takayasu's disease. The patient was given methylprednisolone followed by a combination of prednisone and, initially, cyclophosphamide, later methotrexate. This resulted in a clinical remission of the inflammatory process. The boy presented with increasing fatigue and variable episodes of fever. After 3 years, sarcoidosis or Castleman's disease were considered. Imaging studies revealed aortic stenosis. He underwent stenting of the involved vessel segment. Takayasu arteritis is a chronic vasculitis of unknown origin, affecting mainly the aorta and its main branches. As a result of the inflammation, stenosis, occlusion or dilatation of the involved vessels may occur and cause a wide range of symptoms. Especially in the early phase, the symptoms often are non-specific. One should look for hypertension, blood pressure differences between the two arms, decreased peripheral pulsation or bruits over the aorta and its major branches. Radiological examination may consist ofangiography, magnetic resonance imaging or CT-scans. Treatment consists of corticosteroids and other immunosuppressants, such as cyclophosphamide, methotrexate, azathioprine, and antagonists of tumour-necrosis factor alpha. In addition, balloon dilatation or stenting is often necessary.     

儿童大动脉炎35例临床分析

目的:探讨儿童大动脉炎的临床特点,提高对该病的认识。方法:研究对象为1990年1月～2008年1月在吉林大学第一医院儿科住院并确诊为儿童多发性大动脉炎的患儿共35例,对其城乡分布、临床表现、病情活动性指标以及病情严重性等进行回顾性分析并与文献报道过的成人多发性大动脉炎进行比较。结果:35例患儿中男18例,女17例,发病年龄4～15岁,平均为(11.5±2.2)岁,确诊时病程7天～48个月,其中位数时间为2个月。临床分型以Ⅱ型为最多占57.1%,首发症状以头痛(51.4%)、抽搐(37.1%)等较为多见;在体征方面高血压94.3%、血管杂音61.4%、无脉或脉弱74.1%,共有22例患儿心脏受累所占的比例约为62.8%;仅有不到一半的患儿出现炎性指标升高,就诊时共有30例患儿处于病情活动期占85.7%,有94%以上的患儿出现一种或一种以上的并发症。结论:与文献报道中的成人发病情况比较,儿童多发性大动脉炎并非少见而且较成人大动脉炎起病急、病情重、预后差,在临床工作中应注意监测血压,以减少漏诊及误诊的几率。     

剖宫产瘢痕妊娠41例临床分析

目的探讨剖宫产瘢痕妊娠的发病机制、早期诊断以及治疗方法。方法回顾分析41例剖宫产瘢痕妊娠患者的临床资料,包括患者的发病年龄,孕次及产次,剖宫产手术史及发病至剖宫产术的间隔时间,首发症状及辅助检查的结果,治疗方法及结局。结果 41例患者中2例自然流产,39例患者行双侧子宫动脉化疗栓塞术,32例患者因β-HCG下降不满意或超声提示剖宫产切口瘢痕处仍有妊娠组织或异常团块而行清宫术。结论随着剖宫产瘢痕妊娠病例增多,为避免其引起严重的并发症,早期诊断并作出正确的治疗至关重要。CSP的治疗尚无统一的标准,子宫动脉化疗栓塞术是非常安全、有效的选择。     

Surgical Repair of Abdominal Aortic and Renal Artery Aneurysms in Takayasu's Arteritis

Takayasu''s arteritis is a large vessel vasculitis that can be a challenging diagnosis to make and has a varied clinical presentation. Management largely depends on affected vessel disease severity and individual patient considerations. The diagnosis must be considered in a young patient with large vessel aneurysms. We present a case of a 30 year-old woman of Pacific Islander descent who presented to the Tripler Army medical Center Vascular Surgery Department in Honolulu, Hawai‘i seeking repair of her abdominal aortic and renal artery aneurysms prior to conception.A 30 year-old Pacific Islander woman with a history of a saccular abdominal aortic aneurysm and renal artery aneurysms presented to our clinic seeking vascular surgery consultation prior to a planned pregnancy. She had a renal artery stent placed at an outside institution for hypertension. She met the diagnosis of Takayasu''s arteritis by Sharma''s criteria. Physical exam was significant for a palpable, pulsatile, abdominal mass and CT angiography revealed a saccular irregular-appearing infra-renal abdominal aortic aneurysm, extending to the aortic bifurcation, with a maximum diameter of 3.3 cm. A right renal artery aneurysm was also identified proximally, contiguous with the aorta, with a maximal transverse diameter of 1.7 cm. The patient underwent a supraceliac bypass to the right renal artery with a 7mm Dacron graft, as well as excision of the right renal artery aneurysm. The abdominal aortic aneurysm was replaced using a Hemashield Dacron bifurcated 14mm x 7mm bypass graft. Intraoperative measurements of the renal artery aneurysm were 1.5 x 1.5 cm and the saccular appearing distal abdominal aortic aneurysm measured 3.6 x 3.3cm. The patient was discharged from the hospital 7 days post-operatively. At 1-year follow up, CT scan of the abdominal aorta revealed the repair was without any evidence of aneurysm formation, anastomotic pseudoaneurysm formation, or areas of stenosis. She has remained normotensive with a normal serum creatinine 18 months after her repair. She has since delivered her second child.It is rare for Takayasu''s arteritis to present with aneurysmal disease. It is much more common to present with stenosis or occlusion. It has yet to be proven that Takayasu''s truly has a higher incidence in those of Asian descent. Takayasu''s can be a difficult diagnosis to make but can be aided with the use of Sharma''s criteria. Our particular patient posed unique considerations on the effects of the physiology of pregnancy on her aneurysms and repair.Managing the active phases of disease is imperative, and though medical management is first line, surgical intervention may be necessary. Surgical intervention should be performed in a quiescent period of disease if possible given that biological inflammation at the time of intervention increases the complication rate. Repair of aneurysmal disease in a young female should also be considered prior to pregnancy.     

Clinical thinking and decision making in practice. An elderly patient with vertigo and high sedimentation rate

A 77-year-old woman was admitted because of progressive vertigo, nausea and a dysarthric speech disorder. The patient's history of diabetes mellitus, hypertension and hypercholesterolaemia, and the finding of murmurs over peripheral arteries at physical examination led to a presumptive diagnosis of cerebellar ischaemia in the context of generalized atherosclerosis. However, the diagnosis was revised when bilateral cerebellar infarction was demonstrated radiologically, and a biopsy of a temporal artery revealed giant cell arteritis. Despite treatment with prednisone (60 mg daily) the patient's neurological condition deteriorated, and she succumbed several months later to pneumonia. The case illustrates the pitfalls in the diagnostic approach of elderly patients with multiple pathology and it also emphasizes that in an elderly person with high erythrocyte sedimentation rate (> 100 mm in the first hour) temporal arteritis should be ruled out as soon as possible to prevent further neurological damage.     

Susac syndrome: neurologic, psychiatric, ophthalmologic otorhinolaryngologic and neuroradiologic features of a rare autoimmune disease

Encephalopathy, recurrent occlusion of retinal arteries and hearing loss comprise the clinical picture of Susac's syndrome. The correct diagnosis is frequently missed because of incomplete clinical signs or negligence of previous symptoms. Early diagnosis and treatment can halt the progression and prevent permanent disability. METHODS: Here, we describe a Hungarian case and review the clinical characteristics, diagnostic procedures and current concepts of therapy. RESULTS: A 30-year-old female was admitted to our neurology department because of change in her personality, apathy, and difficulty in concentration. Brain MRI indicated multiple hyperintense T2-weighted lesions including cerebellum and corpus callosum. Protein content of the CSF was markedly elevated. The recurrent bilateral loss of vision and hearing along with migraine in her previous 2,5-year-long medical history suggested Susac's syndrome. Fundoscopy and fluorescein angiography indicated multiple occlusions of the retinal arteries, audiography revealed bilateral hearing loss. Systemic autoimmune and connective tissue diseases and thrombophilia were excluded. The markedly elevated protein in the cerebrospinal fluid supported Susac's syndrome. Chronic treatment with methylprednisolone resulted in remission of clinical signs. DISCUSSION: Consideration of multiple clinical signs is an important key to the diagnosis of rare clinical entities like Susac's syndrome.     

Experience with intra-arterial thrombolysis in patients with acute stroke

The authors report on their results with local intraarterial thrombolysis in four patients. PATIENTS: Three patients suffered from vertebrobasilar artery occlusion, and one from carotid-T occlusion. All were treated with local intraarterial thrombolysis by a joint team of neurologist, neuroanaesthesist and interventional neuroradiologist in the stroke-department of St. Stephen Municipal Hospital in collaboration with National Institute of Neurosurgery. Selection of patients suitable for successful intraarterial thrombolysis has to be done in the neurological department with help of ultrasound and neurological examination and CT scan. Patients were transferred to the therapeutic angiosuit and interventional treatment was initiated immediately upon establishing the diagnosis. At least one million units of urokinase was infused directly into the embolus in each case. RESULTS: Complete recanalisation was achieved in three of the four cases. That was associated with full recovery in two patients, in whom good collateral circulation and no signs of atherosclerotic lesions were found prior to treatment. Another patients has died despite of full recanalisation of the basilar artery. One patient survived after partial recanalisation of bilateral vertebral artery occlusion with mild residual tetraparesis. CONCLUSION: Local intraarterial thrombolysis is effective and is associated with good clinical outcome in properly selected patients. Careful and accurate ultrasound diagnostics of the site of occlusion plays a significant role in proper patient selection. Although difficult, acute intraarterial thrombolysis can be organized in a timely fashion even utilizing the required facilities in two different institutions, providing that all efforts is being made by each party for the fast diagnosis and treatment initiation.     

探讨多层螺旋CT在肺动脉栓塞中的临床应用

目的分析多层螺旋CT在肺动脉栓塞患者临床诊断中的应用价值。方法选取2010年1月—2013年12月期间我院收治的74例肺部动脉栓塞患者作为观察对象,所有患者均经16层CT配合MPB、MIP及VR进行高效处理进行诊断,回顾性分析本组患者的影像学情况。结果本组74例患者经多层螺旋CT检查后,本组74例患者中,病变共受累肺动脉485支,其中显示栓塞左/右肺动脉10支,亚段肺动脉207支,叶肺动脉117支,段肺动脉151支。结论多层螺旋CT在PE的临床诊断中具有无创、高效、准确率高等特点,尤其是对亚段PE的敏感性非常高,可以作为今后临床中关于PE的首选诊断方法。     

多发大性动脉炎1例报告

多发性大动脉炎是一病因尚不明确的少见动脉性疾病,本文我科就收治的一例患者结合文献报道进行讨论。     

Increase of norepinephrine-induced endothelium-dependent relaxation of pulmonary artery in rats after chronic exposure to cold

The present study was designed to determine whether norepinephrine (NE) mediate endothelium-dependent relaxations in arteries of the pulmonary vasculature of cold-acclimated rats. Twenty male Sprague-Dawley rats comprising two groups (Cold-acclimated for 12 weeks at 6°C, CA; Warm-acclimated for 12 weeks at 24 °C, WA) were used. After anesthesia, the pulmonary artery (4 mm long) was isolated. Pulmonary artery with and without endothelium were suspended for isometric force measurements in a buffered salt solution. The doseresponse relations for the vascular responses to the isolated pulmonary artery to norepinephrine (NE), phenylephrine (PE) and acetylcholine (Ach) were determined and compared in the CA group and the WA group. In the CA group, the vascular sensitivities to NE and PE-induced contraction in the pulmonary artery was significantly lowered than that in the WA group. NE and PE-induced contractions were significandy greater in endotheliumdenuded compared with endothelium-intact arteries. These differences of contraction responses to NE and PE between arteries with widiout endothelium were significantly greater in the CA group than in the WA group. There was no significant difference between the pulmonary arterial response to Ach in the CA group and that in the WA group. Our data suggest that chronic exposure to cold show decreased NE and PE-induced contraction responses in isolated pulmonary arteries and may decrease NE-induced contraction responses due to enhancing NE-induced endodielium derived relaxing factor release via up-regulating endothelial α-adrenoceptors.     

肺隔离症的外科诊治临床分析

目的 总结肺隔离症的临床特点、诊断及外科治疗经验.方法 回顾性分析2000年7月至2009年1月经手术治疗15例肺隔离症患者的临床资料.结果 肺隔离症的术前诊断率为93.33%(14/15);术前均行胸部CT平扫+增强,其中9例加行血管三维成像.12例叶内型隔离肺行肺叶切除术,3例叶外型隔离肺行局部切除术.术中发现异常供血动脉14例来源于胸主动脉,1例来源于腹腔干.全部患者手术顺利,无围手术期死亡.结论 肺隔离症的误诊率较高,胸部增强CT薄层扫描+血管三维成像有利于肺隔离症的诊断.手术是治疗肺隔离症安全、有效的方法.     

二维及彩色多普勒超声对多发性大动脉炎的诊断价值

[目的]探讨多发性大动脉炎的二维及彩色多普勒超声诊断价值。[方法]回顾性分析52例多发性大动脉炎患者周围血管的二维及彩色多普勒超声表现。[结果]多发性大动脉炎累及多支大血管,以头臂动脉最多,受累血管声像图表现为对称的管壁增厚、管腔狭窄或闭塞,彩色多普勒超声显示相应血流动力学改变。[结论]二维及彩色多普勒超声检查病变段血管的形态学及血流动力学改变,可作为临床诊断多发性大动脉炎的常规方法。     

彩色多普勒超声评价血流参数指标在诊断移植肾动脉狭窄中的价值

目的：探讨彩色多普勒超声显像对肾移植术后动脉并发症的诊断价值。方法：应用彩色多普勒技术（CDFI）发现了20例发生移植肾动脉狭窄患者,并接受了动脉造影检查,证实有移植肾动脉狭窄,其中2例为重度狭窄导致闭塞。20例患者测量5项多普勒血流参数,肾动脉主干、叶间动脉收缩期峰值流速、加速时间和阻力指数,并进行对比分析。结果：移植肾动脉血管造影显示,13例狭窄部位发生在吻合口附近,5例发生在肾动脉主干,2例移植肾动脉闭塞。移植肾动脉收缩期最大血流速度（PSV）≥2.0 m/s,小叶间动脉加速时间（AT）延长≥0.10 s,阻力指数〈0.07,敏感性分别为100%,90%,54%。2例移植肾动脉闭塞无或稀少血流信号。结论：移植肾动脉吻合口及肾动脉主干收缩期最大血流速度（PSV）,小叶间加速时间（AT）在诊断移植肾动脉狭窄上敏感性较高;彩超显示移植肾内部血流信号消失或肾内仅显示稀少血流信号是移植肾动脉闭塞重要的诊断依据。     

Bilateral leg edema,pulmonary hypertension,and obstructive sleep apnea: a cross-sectional study

This study was undertaken to clarify whether pulmonary hypertension is a useful marker for underlying obstructive sleep apnea in patients with edema. Twenty-eight ambulatory adults with bilateral leg edema and a normal echocardiogram were enrolled. Sixteen subjects had pulmonary hypertension, and 12 subjects had normal pulmonary artery pressures. Spirometry, pulse oximetry on room air, and polysomnography were obtained for each subject. Ten of 16 (63%) pulmonary hypertension subjects and 9 of 12 (75%) nonpulmonary hypertension subjects had obstructive sleep apnea (P =.48). Eleven of 16 (69%) pulmonary hypertension subjects and 11 of 12 (92%) nonpulmonary hypertension subjects were obese (P =.20). If these results are generalizable, obstructive sleep apnea is frequently associated with bilateral leg edema and obesity, regardless of the presence of pulmonary hypertension. Thus, especially in obese patients, bilateral leg edema may be a useful clinical marker for underlying obstructive sleep apnea.     

多层螺旋CT肺动脉造影在肺小动脉栓塞诊断中的价值

目的:探讨多层螺旋CT肺动脉造影(MSCTPA)在肺小动脉栓塞诊断中的价值。方法:回顾性分析12例肺小动脉栓塞患者的临床资料及MSCTPA表现。结果:12例患者肺栓塞均位于肺动脉段及亚段水平,受累肺动脉段及亚段126支,MSCTPA显示肺小动脉栓塞的征象为肺小动脉内的大小不等的充盈缺损所致的狭窄及闭塞改变,其中中心性充盈缺损44支,附壁性充盈缺损51支,闭塞31支。结论:MSCTPA是准确、快捷、无创的影像诊断方法,可以作为诊断肺小动脉栓塞的首选检查方法。     

布托啡诺对脂多糖诱导离体兔肺动脉反应性变化的影响

目的 探讨阿片受体激动剂布托啡诺对正常及内毒素休克时肺动脉血管反应性的影响,从而指导临床用药.方法 应用血管环张力检测技术,观察布托啡诺对正常及脂多糖（lipopolysaccharide,LPS 4 μg/ml,4 h）孵育的肺动脉血管环反应性的影响.结果 在正常肺动脉组,布托啡诺终浓度（0.1 μmol/L,1 μmol/L,10μmol/L,100 μmol/L）使苯肾上腺素（Phenylephrine,PE）预收缩的肺动脉呈剂量依赖性舒张,在LPS孵育的肺动脉组,布托啡诺终浓度（0.1 μmol/L,1μmol/L,10μmol/L,100 μmol/L）也使PE预收缩的肺动脉呈剂量依赖性舒张反应,但其舒张作用与正常组比较明显加强.结论 布托啡诺对血管具有直接的舒张反应,并且LPS可以增强布托啡诺对肺动脉的最大舒张反应.     

A population-based case-control study of temporal arteritis: evidence for an association between temporal arteritis and degenerative vascular disease?

The first population-based incident case-control study of temporal arteritis (TA) in the US was conducted using the unique data resources of the Rochester Epidemiology Project. During the period 1950-1985, 88 newly diagnosed cases of biopsy-proven TA were identified among residents of Olmsted County, Minnesota. Cases were each matched to four Olmsted County community controls on age, sex and duration of community medical record. Odds ratios (OR) were calculated for marital status, education, Quetelet index, pregnancy, age at menopause, thyroid disease, diabetes, smoking, hypertension, angina, myocardial infarction, peripheral vascular disease, and stroke. Multivariable conditional logistic regression analysis identified statistically significant adjusted OR for smoking (2.3, 95% CI = 1.3-4.1). Elevated ORs which were not statistically significant were noted for angina, myocardial infarction, and peripheral vascular disease. These data suggest that TA and arteriosclerosis may share a common causal pathway. Alternatively, histopathological misclassification of temporal artery biopsies may have resulted in the observed association. Due to the limited power of this population-based study, multicentre collaboration should be encouraged to more precisely define the epidemiology of TA.