Xamoterol in the treatment of orthostatic hypotension associated with multiple system atrophy (Shy-Drager syndrome)

The effect of xamoterol on the orthostatic hypotension associated with Shy-Drager syndrome was investigated in three patients. Intra-arterial blood pressure was measured during a control period and during treatment with xamoterol, both in a cardiovascular investigation laboratory and for 24 h of unrestricted activity using portable apparatus. Xamoterol lessened the total number of symptomatic episodes of orthostatic hypotension by 67 per cent. Average untreated 24-h intra-arterial blood pressure was 132/78 mmHg; during treatment with xamoterol it rose to 138/90 mmHg. However episodes of severe hypertension (defined as a systolic intra-arterial blood pressure above 200 mmHg) were more frequent with xamoterol. Although xamoterol attenuated orthostatic hypotension, careful monitoring of ambulatory blood pressure may be necessary, particularly at the start of treatment, because of the development of severe supine hypertension. Intravenous test doses of xamoterol did not predict either the attenuation of orthostatic hypotension or the development of supine hypertension in all patients.     

Successful Treatment of Severe Orthostatic Hypotension with Erythropoietin

KAWAKAMI, K., et al .: Successful Treatment of Severe Orthostatic Hypotension with Erythropoietin. A 71-year-old man, who was diagnosed with familial amyloidosis type I, was admitted for treatment of severe orthostatic hypotension associated with recurrent syncopal attacks. Head-up tilt testing demonstrated severe orthostatic hypotension (114/72 mmHg in the supine position and 62/34 mmHg in the upright position) with syncope or presyncope. Oral midodorine and fludrocortisone therapies failed to prevent his symptoms. After administration of subcutaneous erythropoietin, his blood pressure drop in the upright position was decreased and symptoms disappeared unassociated with improvement of anemia. Although previous reports have shown that the mechanism by which erythropoietin improves orthostatic hypotension is related to improvement in anemia, other mechanisms may also play a role. (PACE 2003; 26[Pt. I]:105–107)     

老年人体位性低血压

体位性低血压（OH）在老年人群中较为常见。随年龄增长,大血管弹性纤维减少,交感神经反射增强,可使老年人血压升高。长期偏高的血压及老年人易合并糖尿病、脑卒中等多种疾病,不仅损害压力感受器的敏感度,还会影响血管和心室的顺应性[1],当体位突然发生变化或服降压药以后,在血压突然下降的同时,各组织器官缺血的危险性也大大增加。     

Asymptomatic Brugada Syndrome Associated with Postural Orthostatic Tachycardia Syndrome:

Autonomic imbalance may work as a modifying factor for initiating lethal arrhythmia in patients with Brugada syndrome. A 26-year-old man with episodes of near syncope was given a diagnosis of an autonomic disorder, postural orthostatic tachycardia syndrome (POTS). The patient spontaneously showed typical Brugada-type ECG, and ventricular fibrillation was induced by programmed electrical stimulation, which allowed the further diagnosis of Brugada syndrome. Although it seems that Brugada syndrome is asymptomatic, its uncommon association of POTS may increase the risk for future arrhythmic events in this patient.     

脊髓损伤后的体位性低血压

体位性低血压是指在直立体位下的血压的下降。体位性低血压常发生在高位脊髓损伤的患者中,影响患者的训练和康复。但到目前为止,其发生原因尚未完全明确。本文主要就脊髓损伤后体位性低血压的可能原因;典型症状、诊断和测量;治疗和预防进行综述。     

Orthostatic Hypotension in Children with Acute Febrile Illness

Background

Children presenting to the Pediatric Emergency Department (PED) with fever often describe symptoms such as lightheadedness, dizziness, fatigue, and weakness, and may appear pale. They may also present with a chief complaint of syncope. Such symptoms may result from orthostatic hypotension.

Objective

To determine whether children with an acute febrile illness have a higher incidence of orthostatic hypotension compared to afebrile children.

Methods

A prospective cohort study was conducted at the PED at Assaf Harofeh Medical Center, a university-affiliated hospital in Israel. Eighty children aged 4–18 years were recruited. Thirty-nine had fever (>38 °C for 6–48 h) and 41 were afebrile. All subjects had their blood pressure measured in the supine position (after 5 min of rest) and again after standing for 3 min. The main outcome measure was orthostatic hypotension, that is, a reduction of systolic blood pressure of at least 20 mm Hg, or a fall in diastolic blood pressure of at least 10 mm Hg within 3 min of standing.

Results

There were no differences between the groups in gender, age, height, or weight. Orthostatic hypotension was found in 10/39 (25.6%) of febrile children and in 2/41 (5%) of afebrile children (p = 0.012).

Conclusions

The incidence of orthostatic hypotension among febrile children in the PED is high, and may explain common symptoms such as dizziness or syncope. Such patients should be instructed to drink properly and to avoid rapid changes in body posture.     

直立性低血压老年人在硬膜外麻醉期间低血压的临床分析

目的:研究直立性低血压的老年人在硬膜外麻醉期间低血压的发生率及血流动力学的变化,初步探讨其血压变化的机制.方法:将70例拟行下腹部手术的老年人分为直立性低血压组(Ⅰ组)19例和对照组(Ⅱ组)51例.观察两组病人在行硬膜外麻醉后(阻滞平面控制在胸6以下)低血压的发生率;比较两组病人在麻醉前及低血压发生时的中心静脉压(CVP)、心输出量(CO)、总外周阻力(TPR)及心率(HR).结果:硬膜外麻醉后,Ⅰ组低血压发生率(100%)比Ⅱ组(71%)高(P<0.01);低血压发生时,与麻醉前比较,两组病人均见CO降低(Ⅰ组P<0.01,Ⅱ组P<0.05)及CVP下降(Ⅰ组P<0.001,Ⅱ组P<0.01);且Ⅰ组CO和CVP的降低更为明显(P<0.01).结论:直立性低血压老年人在硬膜外麻醉期间更易于出现低血压,其主要原因可能与直立性低血压老年人CO的减少更为剧烈有关.     

多系统萎缩患者的临床特点分析

目的 研究不同亚型多系统萎缩(multiple system atrophy,MSA)患者的临床特点.方法 回顾分析2009年1月-2011年1月收治的105例"很可能的"MSA患者的临床资料,包括发病年龄、首发症状、临床表现、治疗反应性等.结果 105例MSA患者中,男57例,女48例,发病年龄58岁.以小脑性共济失...     

The Treatment of Idiopathic Orthostatic Hypotension: a Combined Fludrocortisone and Flurbiprofen Regime

We report our experience in the management of idiopathic orthostatichypotension using the prostaglandin synthetase inhibitor, flurbiprofen. In five subjects with proven autonomic faihire the effect onblood pressure of the addition of flurbiprofen (or fludrocortisone)to the previous drug regime was assessed. Both lying and standingblood pressure were seen to rise with fludrocortisone treatmentalthough the change in standing blood pressure was not statisticallysignificant. Flurbiprofen, in contrast, produced no change inlying blood pressure but a significant rise in standing bloodpressure. Digital blood flow measurements were made on threesubjects before and after flurbiprofen and a dose-related reductionin post-occlusive reactive hyperaemia was demonstrated suggestingan effect of this drug on precapillary smooth muscle tonus.Two patients failed to maintain a long-term response to treatmentwith flurbiprofen and fludrocortisone but have been helped bythe addition of i) ephedrine or ii) tyramine and monoamlne oxldaseinhibitor (phenelzme). We recommend a stepwlse approach to treatment in this conditioncommencing with flurbiprofen and adding fludrocortisone laterif necessary. This approach would appear to offer the maximumbenefit with a minimum of side effects.     

体位性低血压对老年维持性血液透析患者预后的影响

目的探讨体位性低血压(OH)对老年维持性血液透析患者预后的影响。方法选取我院2012年1月~2014年8月入院治疗的50例老年维持性血液透析患者做为研究对象。分析研究体位性低血压对患者预后及死亡的影响。结果50例患者中,18例(占36%)患者存在不同程度的OH。随访2~145w,共有5例死亡。应用多变量COX比例危险率回归模型研究发现患者年龄、有无OH及脑血管病变是影响患者预后的重要因素,差异具统计学意义(P0.05)。结论老年维持性血液透析患者发生OH现象后可成为预示患者死亡的独立影响因素。     

Shy-Drager综合征九例误诊漏诊原因分析

目的:探讨Shy-Drager综合征(SDS)的误诊原因,总结其临床特点并提出防范误诊措施.方法:回顾分析9例SDS误诊病例的临床资料.结果:9例均为男性,均表现为直立性低血压、尿便障碍、阳痿、泌汗异常等,亦有锥体系统、锥体外系、小脑症状.病初误诊为椎底动脉供血不足4例,诊断前列腺增生漏诊SDS 3例,误诊为抑郁症和植物神经功能紊乱各1例.7例MRI检查示脑干、小脑萎缩.根据临床表现及医技检查结果确诊为SDS.结论:SDS临床少见,早期误诊率高,需全面认识其临床特点,详细询问病史,细致查体,及时完善MRI检查有助早期诊断.     

老年原发性低颅压综合征诊治体会(附46例报告)

[目的]探讨老年原发性低颅压综合征(PIH)的临床特点,提高对该病的诊治水平,减少误诊发生.[方法]对近15年在本院住院确诊为PIH的46例老年患者的临床资料及诊治过程进行回顾性分析,并结合文献对此类患者的临床特点、发病机制、诊断、鉴别诊断以及治疗进行探讨.[结果]46例PIH均表现为直立性头痛,且脑脊液压力≤60 mmH2O,结合脑脊液常规生化检查及头颅影像学检查可确诊;通过改变体位、饮食、静脉补液等处理后,46例患者头痛症状均能有效缓解;其中13例患者治疗1周内症状消失出院,23例患者治疗1～2周疗效显著出院,10例患者治疗3周左右痊愈出院.所有患者出院前经复查腰穿脑脊液压力恢复正常,所有患者随访半年时间均无复发.[结论]针对头痛的老年人,临床医生必须警惕老年PIH,通过详细的病史采集及辅助检查并排除其他明确原因有助于减少该病的漏诊及误诊;一旦确诊则可通过综合应用各种治疗手段,有效地纠正病因、缓解症状及改善预后.     

张波应用温阳通脉方治疗多系统萎缩临床经验

张波主任认为,多系统萎缩基本病机为脾肾阳虚、气血不足,临床多以温肾健脾为治则,方选温阳通脉,并根据临床随证加减。     

多系统萎缩的泌尿生殖系功能障碍5例报道

目的认识多系统萎缩患者的泌尿生殖系功能障碍特点及影像尿动力学检查的意义。方法报告5例多系统萎缩病例。结果多系统萎缩在泌尿生殖功能障碍上表现为排尿困难、夜间尿频、尿急阳痿等。特征性尿动力学检查结果是残余尿大于100 ml,逼尿肌-尿道外括约肌协同失调,膀胱测压早期出现膀胱颈开放,括约肌肌电图改变有出现自发电位,运动单位电位(MUPs)持续时间超过13 ms,多相波大于60%,影像学检查有不同程度的脑萎缩,脑桥小脑呈“十”字退变。结论本病是一种以神经系统多部位进行性萎缩所致的临床病变,而其中一些萎缩部位在控制泌尿生殖系功能方面起着重要的作用。泌尿生殖系改变与膀胱出口梗阻病变相似,如进行合理的辅助检查,可提高早期多系统萎缩诊断,避免误诊和不必要的外科治疗。     

磁共振成像在多系统萎缩诊断与鉴别诊断中的应用价值

目的:探讨磁共振成像(MRI)在诊断多系统萎缩(MSA)及鉴别MSA与帕金森病(PD)中的应用价值。方法:对26例MSA患者(MSA组)、20例PD患者(PD组)及18例健康对照者(对照组)进行MRI检查,观察脑实质萎缩、脑桥十字征、壳核外侧缘裂隙征、MCP高信号、壳核低信号,并测量所有受试者的MCP宽度。结果:所有MSA患者均可见不同程度的小脑、脑桥、延髓、壳核萎缩,四脑室及桥延池扩大,其中15例(57.7%)可见脑桥十字征,6例(23.1%)可见T2像轴位MCP高信号,8例(30.8%)可见裂隙征,4例(15.4%)可见T2像壳核低信号。MSA组MCP宽度(6.4±1.4)mm明显小于PD组(9.2±1.1)mm与对照组(9.4±0.8)mm(P均<0.01),PD组MCP宽度与对照组差异无统计学意义。15例MRI有脑桥十字征的MSA患者MCP宽度平均为(5.3±1.0)mm,11例MRI无脑桥十字征的MSA患者MCP宽度平均为(7.2±1.6)mm,两者比较差异有统计学意义(P<0.05)。结论:MRI有助于MSA的诊断;MRI测定MCP宽度可作为鉴别MSA与PD的一个指标。     

A Systematic Review of the Management of Orthostatic Hypotension After Spinal Cord Injury

Krassioukov A, Eng JJ, Warburton DE, Teasell R, Spinal Cord Injury Rehabilitation Evidence Research Team. A systematic review of the management of orthostatic hypotension after spinal cord injury.

Objective

To review systematically the evidence for the management of orthostatic hypotension (OH) in patients with spinal cord injuries (SCIs).

Data Sources

A key word literature search was conducted of original and review articles as well as practice guidelines using Medline, CINAHL, EMBASE, and PsycInfo, and manual searches of retrieved articles from 1950 to July 2008, to identify literature evaluating the effectiveness of currently used treatments for OH.

Study Selection

Included randomized controlled trials (RCTs), prospective cohort studies, case-control studies, pre-post studies, and case reports that assessed pharmacologic and nonpharmacologic intervention for the management of OH in patients with SCI.

Data Extraction

Two independent reviewers evaluated the quality of each study, using the Physiotherapy Evidence Database score for RCTs and the Downs and Black scale for all other studies. Study results were tabulated and levels of evidence assigned.

Data Synthesis

A total of 8 pharmacologic and 21 nonpharmacologic studies were identified that met the criteria. Of these 26 studies (some include both pharmacologic and nonpharmacologic interventions), only 1 pharmacologic RCT was identified (low-quality RCT producing level 2 evidence), in which midodrine was found to be effective in the management of OH after SCI. Functional electrical stimulation was one of the only nonpharmacologic interventions with some evidence (level 2) to support its utility.

Conclusions

Although a wide array of physical and pharmacologic measures are recommended for the management of OH in the general population, very few have been evaluated for use in SCI. Further research needs to quantify the efficacy of treatment for OH in subjects with SCI, especially of the many other pharmacologic interventions that have been shown to be effective in non-SCI conditions.     

Assessment and Treatment of Self-Injurious Behavior Associated with Donnai-Barrow Syndrome

Donnai and Barrow (American Journal of Medical Genetics, 68, 441–444, 1993) reported multiple participants who shared a variety of specific physical and neurological anomalies. Relatively few cases have been reported since then and few of those have progressed to an age such that the developmental progression of the disorder can be ascertained. We describe one participant with Donnai-Barrow syndrome who engaged in repetitive self-injurious behavior (SIB), which heretofore has not be described in this population. Direct observations of the participant’s SIB were conducted across a range of contexts, and the behavior was successfully decreased using a combination of procedures. This case is discussed within the context of the behavioral phenotype of Donnai-Barrow syndrome.