Shear Syndrome: The Worst Case Scenario of Crush Syndrome

Shear syndrome is described as a complication of crush syndrome. In addition to compression of and injury to the electrode, complete transection occurs. In this case, the free end migrated to the pulmonary artery with the potential for further complications.     

Update on Clinical Features and Brain Abnormalities in Neurogenetics Syndromes

Neuroimaging methods represent a critical tool in efforts to join the study of the neurobiology of genes with the neurobiology of behaviour, and to understand the neurodevelopmental pathways that give rise to cognitive and behavioural impairments. This article reviews the clinical features and highlights studies with a focus on the relevant gene–brain–behaviour connections observed in neurogenetic syndromes, such as Williams, Rett, Fragile X, Prader‐Willi, Angelman, Down and velocardiofacial (22q11.2 deletion) syndromes. The relationship of altered brain regions and activation patterns are discussed for each syndrome, as well as the clinical, cognitive and behavioural correlates of these neuroimaging findings.     

A Case of Delayed Presentation of Thigh Compartment Syndrome

Background

Thigh compartment syndrome is a rare and devastating process. It generally occurs within hours to days of a traumatic event, although cases have been reported nearly 2 weeks after the initial event.

Objectives

To evaluate the literature describing the timing between inciting event and presentation of thigh compartment syndromes, with a focus on delayed presentations of this rare condition. To describe the unique properties of thigh compartments, and finally, to review the anatomy and techniques needed to measure the compartment pressures of the thigh.

Case Report

A case of a 54-year-old man is presented. He sustained trauma to his thigh 17 days prior to presenting to our ED with severe, sudden-onset pain in his right thigh. Compartment pressures were measured and confirmed the diagnosis of compartment syndrome caused by two large intramuscular hematomas. No other contributing events were identified.

Conclusions

Compartment syndrome in the thigh should be considered in patients with a concerning examination and a history of recent trauma. This particular case represents the longest reported time between injury and development of a thigh compartment syndrome.     

原发性肾病综合征合并布加综合征16例

目的：探讨原发性肾病综合征合并布加综合征的病因、发病情况、临床特点及治疗方案。方法：对2001—2004年16例原发性肾病综合征合并布加综合征的临床资料进行回顾性分析。结果：原发性肾病综合征合并布加综合征为血栓形成所致，起病急，表现为肝脾肿大、进行性顽固性腹水、上腹痛、消化道出血、水肿等。积极治疗原发病，溶栓抗凝治疗能促进腹水消退，防治肝肾功能衰竭，防治血管狭窄，缩短病程。介入治疗尤其局部溶栓术在短期内更能明显改善患者临床症状和实验室指标。结论：临床上应提高对本综合征的认识，对疑似病例应尽早做相关影像学检查以明确诊断，并根据病情制定恰当的治疗方案。     

高嗜酸性粒细胞综合征的临床及实验室特征研究

目的 探讨高嗜酸性粒细胞综合征(HES)各亚型的临床及实验室特点,尤其是骨髓及外周血涂片中嗜酸性粒细胞的形态学特征.方法 回顾性分析该院2017-2020年63例HES患者的临床和实验室资料,复检外周血和骨髓涂片中嗜酸性粒细胞的形态学特征.结果 63例H ES患者中有反应性HES患者54例,特发性HES患者9例.反应性...     

Intermittent Preexcitation: Marked Enhancement of Anterograde Conduction in the Atrioventricular Accessory Pathway with Isoproterenol

A 29-year-old man presented with intermittent preexcitation and exertional syncope. Electrophysiological evaluation in the control state demonstrated a single, left posterior free-wall atrioventricular accessory pathway. The anterograde and retrograde effective refractory periods and block cycle lengths were long in the control state. No tachycardias were induced during programmed electrical stimulation. After intravenous administration of isoproterenol, anterograde conduction of the accessory pathway was markedly enhanced (block cycle length shortened 45% to less than 240 ms) and rapid antidromic reciprocating tachycardia (CL = 250 ms) associated with syncope was observed. Following successful surgical dissection of the accessory pathway the patient has been without tachycardia or exertional symptoms. We conclude that, under the influence of exercise or emotion, patients with intermittent preexcitation may be at risk for serious arrhythmias.     

Transvaginal sonography of postabortal (Redo) syndrome

Acute hematometra, also termed the postabortal syndrome or redo syndrome, is a rare immediate complication of suction curettage characterized by severe lower abdominal cramping in association with an enlarged and markedly tender uterus. We describe the transvaginal sonographic features of this syndrome.     

女性更年期综合征现状及影响因素分析

目的调查女性更年期综合征的现状,探讨其影响因素。方法采用一般情况调查表、改良更年期Kupermann评分量表和症状自评量表、艾森克个性问卷,对833例更年期女性进行调查,采用χ2检验、皮尔逊相关性分析与多元回归分析进行统计处理。结果本组女性更年期综合征发生率为77.6%,城市、已婚、补充豆浆/牛奶女性组群更年期症状评分显著性低于来自农村、没有补充豆浆/牛奶的女性组群(P0.01);更年期症状评分与自评症状量表总分、神经质维度得分呈正相关(相关系数为0.432、0.344,P0.01),与内外向维度得分呈显负相关(相关系数为-0.155,P0.01);经多元线性回归分析,自评症状量表总分、神经质维度得分、内外向维度得分进入回归方程(回归系数分别是0.752、0.302、-0.149,P0.01或P0.05)。结论女性更年期症状与城乡、年龄、长期补充豆浆、心理症状及人格特质有关。     

SAPHO综合征一例报道并文献复习

目的引起国内临床医生对SAPHO综合征的重视，提高诊治水平。方法报道1例SAPHO综合征并对国外SAPHO综合征的文献进行复习。结果本例患者为19岁男孩，既有面部及胸背部痤疮和双侧腋窝脓疱病皮肤改变，又有双侧锁骨内段骨炎、骨肥大、胸锁关节和胸骨柄体关节炎的影像学表现。结论SAPHO综合征少见，若皮肤痤疮和（或）脓庖病伴有骨关节炎性改变时应考虑到本病。     

Parameter signature of a reel problem

Clinics involved in follow-up of cardiac implantable electronic devices, especially implantable cardiac defibrillators (ICDs), increasing rely upon remote monitoring. This case describes the parameter signature characteristic of an uncommon but well-described syndrome. Analysis of the nature and timing of the sequence of abnormalities contained in the archived device data, all of which is available for review via remote monitoring, reveals the cause for failure of this primary prevention ICD system.     

代谢综合征的诊断问题

代谢综合征是一组复杂的代谢紊乱症候群,是导致糖尿病、心脑血管疾病的危险因素的聚集。本文重点探讨代谢综合征(metabolic syndrome,MS)的定义及诊断。     

Emergency department presentation of a rare neurological disorder

The treatment and disposition of patients with neurological disorders is commonplace in the emergency setting, but atypical and uncommon presentations can prove to be especially challenging. In this article we discuss the case of a 31-year-old woman who presented with a rare disorder known as opsoclonus myoclonus syndrome (OMS). OMS is characterized by involuntary, multidirectional movement of the eyes, myoclonus, and truncal ataxia. The etiology is thought to be autoimmune, and is most commonly associated with encephalitis or paraneoplastic syndrome. After an 8-day hospital course, which included several different treatment modalities including plasmapheresis, the patient was discharged after making a complete recovery. Unusual presentations such as the one described in this article illustrate the point that it is crucial to have a systematic approach that can be applied to identify and treat potentially life-threatening neurological conditions.     

True Incidence of Pacemaker Syndrome

HELDMAN, D., ET AL.: True Incidence of Pacemaker Syndrome. Although the purported incidence of pacemaker syndrome according to the literature is only 5%–15%, this is based on a series of patients with VVI pacing. Increasing numbers of studies are being reported in which patients prefer the dual chamber mode despite little benefit being demonstrated on objective testing, suggesting that pacemaker syndrome may be more common than is generally reported. This study was designed to evaluate the reported symptoms in a series of patients programmed to both the VVI and one or more dual chamber modes. Forty unselected patients with dual chamber pacemakers were entered into a blind, randomized trial comparing the symptoms associated with VVI pacing to those associated with dual chamber pacing. Patients were randomized to either WI or dual chamber pacing. At the end of 1 week, questionnaires rating 16 different symptoms were completed. Blood pressure, LV function, presence of ventriculoatrial conduction, and ability to override the pacemaker were evaluated. The pacemaker was then programmed to the other mode. Overall, 12 of 16 symptoms were significantly worse in the VVI as compared to dual chamber mode. The most highly significant (p < 0.005) were shortness of breath, dizziness, fatigue, pulsations in the neck or abdomen, cough, and apprehension. Pacemaker syndrome was clinically recognized in 83% of patients paced in the WI mode with 65% of patients experiencing moderate to severe symptoms. There were no readily identified clinical, hemodynamic, or electrophysiological parameters that predicted which patients would develop pacemaker syndrome. Thus, when patients have an opportunity to experience both pacing modes in close proximity to one another, there is a high incidence of pacemaker syndrome in the VVI mode.     

Superior mesenteric artery syndrome: Diagnosis and management

Superior mesenteric artery (SMA) syndrome (also known as Wilkie's syndrome, cast syndrome, or aorto-mesenteric compass syndrome) is an obstruction of the duodenum caused by extrinsic compression between the SMA and the aorta. The median age of patients is 23 years old (range 0-91 years old) and predominant in females over males with a ratio of 3:2. The symptoms are variable, consisting of postprandial abdominal pain, nausea and vomiting, early satiety, anorexia, and weight loss and can mimic anorexia nervosa or functional dyspepsia. Because recurrent vomiting leads to aspiration pneumonia or respiratory depression via metabolic alkalosis, early diagnosis is required. The useful diagnostic modalities are computed tomography as a standard tool and ultrasonography, which has advantages in safety and capability of real-time assessments of SMA mobility and duodenum passage. The initial treatment is usually conservative, including postural change, gastroduodenal decompression, and nutrient management (success rates: 70%-80%). If conservative therapy fails, surgical treatment (i.e., laparoscopic duodenojejunostomy) is recommended (success rates: 80%-100%).     

Different definition of microvascular angina

We sometimes encounter patients with microvascular angina (MVA), a disease characterized by anginal pain without abnormal coronary arteriographic findings or coronary spasm. More than 40 years have passed since MVA was first confirmed. The terms ‘syndrome X’, ‘cardiac syndrome X’ and ‘microvascular dysfunction’ have also been used to describe conditions similar to MVA, but all with slightly different definitions. The cause of MVA seems almost certain to be organic and functional abnormalities of the small arteries of the heart. Patients with MVA are likely to suffer from endothelial dysfunction and other microvascular abnormalities of both the coronary and peripheral arteries. The major treatment of MVA has been medication, most often calcium channel blockers. The prognosis of MVA is generally excellent, although symptoms remain in many studies. Some MVA patients with accompanying hypertensive heart disease have gone on to develop progressive left ventricular dysfunction, with poor prognosis. The different definitions applied to the terms used to describe this condition, what we refer to here as MVA, can confound issues involved in diagnosis, prognosis and proper treatment. Therefore, it is extremely important to distinguish primary MVA without underlying heart disease from secondary MVA to explore the disease mechanism and examine the clinical characteristics. It is more than 40 years since Likoff first confirmed this disease; therefore, all researchers know that strict diagnostic criteria for MVA should be immediately established.     

类Gitelman综合征发病机制的研究进展

Gitelman-like综合征与Gitelman综合征(GS)的临床表现一样,是以低钾性碱中毒和低镁血症为特征。Gitelman 综合征是一种由于编码位于肾远曲小管的噻嗪类利尿剂敏感的钠氯共转运蛋白基因突变的低钾失盐性肾小管疾病,又名家族性低钾低镁血症。但仍有10%左右的临床诊断为GS,却未能检测到致病疾病的突变。专家们对于临床表现相似的、但未检测到基因致病突变的这这些患者定义为Gitelman-like 综合征,这些患者往往存在非遗传因素或线粒体DNA的突变如异亮氨酸和苯丙氨酸的突变。     

Noninvasive Investigation of Pericarotid Syndrome: Role of MR Angiography in the Diagnosis of Internal Carotid Dissection

A 52-year-old man presented with unilateral left periorbital and frontotemporal pain associated with a partial ipsilateral Horner's syndrome of the postganglionic type and representing a pericarotid syndrome. MRI demonstrated a perivascular subacute hematoma at the level of the cervical portion of the left internal carotid artery with a markedly reduced flow-void signal. MR angiography confirmed the narrowed lumen of the dissected cervical internal carotid artery. There was also a right-sided precavernous carotid aneurysm. Three months later the left-sided pain had subsided, with complete resolution of the hematoma and incomplete restoration of the left carotid lumen seen on MR angiography. Dissection of the carotid wall may cause the oculosympathetic paralysis by producing a lesion of the superior cervical ganglion, the internal carotid nerve, or the perivascular sympathetic plexus. Whereas in pericarotid syndrome the most common cause is cervical carotid dissection, Raeder's syndrome additionally involving parasellar cranial nerves, may be caused by any paracavernous/cavernous lesion, including neoplasms and intracranial carotid aneurysms. The clinical distinction is useful to determine the appropriate diagnostic investigation, in view of the different pathoanatomical localization and different disease spectrum. As demonstrated in the present case, the combination of MRI and MR angiography is a reliable noninvasive tool to investigate the differential diagnosis of pericarotid syndrome, accurately depicting occlusive, stenotic or aneurysmal lesions of the carotid artery. We suggest that intraarterial angiography is no longer necessary.     

变应性肉芽肿性血管炎11例临床分析

【目的】分析变应性肉茅肿性血管炎(CSS)的临床表现．以便提高临床诊断水平【方法】11例确诊CSS患者．分析其既往痛史、临床表现、实验室检查、活检病理及治疗情况【结果】11例患者中发生哮喘10例;10例出现皮肤损伤;分别有1例和2例出现周围神经和中枢神经病变：消化道症状者1例．包括腹痛、腹泻和脓血便：肾脏损害者4例;心脏受累者6例全部病例周围血嗜酸粒细胞(EOS)均增高．有1例活捡病理支持CSS诊断。所有患者均用糖皮质激素和免疫抑制制治疗。【结论】CSS的临床表现多种多样．对于有系统性表现．特别是血嗜酸细胞升高者应提高警惕。