Susac's syndrome or catastrophic antiphospholipid syndrome?

Susac's syndrome is a microangiopathic disorder of unknown pathogenesis presenting with encephalopathy, hearing loss and branch retinal artery occlusions. The term 'catastrophic' antiphospholipid syndrome (APS) is used to define a subset of the APS characterized by thrombotic microangiopathy with clinical evidence of three or more organ involvement developed in a short period of time. We describe a patient with typical features of Susac's syndrome, that appeared in less than a week, in whom aPL were detected, thus fulfilling criteria for 'probable' catastrophic APS.     

Metabolic syndrome

Corresponding author:Charles Shaeffer,MD,Desert Cardiology Center,Eisenhower Medical Center,39000 Bob Hope Drive,Rancho Mirage,CA92270-3221,USATel:760-346-0182;E-mail:sewfari @ earthlink.net     

Hepatorenal syndrome

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Economy-class syndrome or Immobile Traveler's syndrome?

Economy class syndrome describes the situation of patients who suffer pulmonary embolism following prolonged immobility in a long distance flight. The objective of the present study was to analyze whether there is a risk of pulmonary thromboembolism after a long overland journey. The study consecutively enrolled 100 patients diagnosed with pulmonary thromboembolism. Nine of them had previously undertaken a long journey (>5 h), 6 of which had been over land. Consequently, we think that the preventative measures recommended for airline passengers should be extended to users of other means of transport.     

Acute coronary syndrome or apical ballooning syndrome?

Apical ballooning syndrome (ABS) is uniquely characterized by the acute onset of transient extensive kinesis of the apical and mid portions of the left ventricle without significant epicardial coronary artery stenosis, accompanied by chest symptoms and electrocardiogram changes similar to those of acute coronary syndrome. We report a case of ABS with severe coronary artery stenosis presenting as acute coronary syndrome after emotional stress. ABS should be considered a cause of left ventricular wall motion abnormalities even if a coronary arteriogram shows severe coronary artery stenosis.     

Neuro-Behçet syndrome presenting as acute meningeal syndrome

Beh?et's disease is a systemic vasculitic disease characterized mainly by recurrent oral and genital aphthous ulcers, uveitis and skin findings. Central nervous system involvement is a serious manifestation. A case of acute meningeal syndrome secondary to Beh?et's disease is presented. Beh?et's disease should always be considered in differential diagnosis of acute meningeal syndrome without signs of viral infection, particularly in the context of multisystem manifestations.     

Irrita blebowel syndrome

IBS是胃肠病门诊最常见的疾病 ,目前的诊断主要依靠罗马标准 ,根据这一标准 ,病人须有腹部不适或疼痛 ,以及肠道功能异常。在美国IBS的发病率为 10 -2 0 % ,其中 75 %为女性。多数IBS病人为轻度 ,对本病能够耐受 ,对安慰和生活改变等治疗反应良好 ;约 2 5 %为中度 ,可明显影响日常生活 ,经一般胃肠病对症治疗效果欠佳 ,用心理治疗常可得到缓解 ;另外 ,5 %为重症病人 ,各种治疗效果均差。IBS的发生涉及肠道的运动和感觉异常 ,疼痛阈值降低 ;30 %的IBS病人有急性结肠感染史 ,机理尚不清楚 ,可能通过心理 神经 免疫因素而导致IBS的发生 ;脑肠轴也参与IBS的发生。作者主要用胃肠道和脑相互作用这一循环模式来认识IBS的身心异常 ,焦虑和抑郁在IBS病人中很常见。IBS的治疗应考虑其身心异常这一特点 ,轻度病人通过饮食调整、肠道扩容或动力药治疗 ,也可用抗胆碱和抗抑郁药 ,但应注意它们的副作用 ;认知治疗也是有效的方法之一。作者采用心理和胃肠道结合疗法 ,强调家庭的作用 ,因为IBS和家庭成员间的关系可相互影响。同时心理和消化专家一起对病人咨询 ,这样有利于对病人进行IBS的心理和躯体两方面的特点的解释     

Guillain-Barré syndrome

Guillain-Barré syndrome consists of at least four subtypes of acute peripheral neuropathy. Major advances have been made in understanding the mechanisms of some of the subtypes. The histological appearance of the acute inflammatory demyelinating polyradiculoneuropathy (AIDP) subtype resembles experimental autoimmune neuritis, which is predominantly caused by T cells directed against peptides from the myelin proteins P0, P2, and PMP22. The role of T-cell-mediated immunity in AIDP remains unclear and there is evidence for the involvement of antibodies and complement. Strong evidence now exists that axonal subtypes of Guillain-Barré syndrome, acute motor axonal neuropathy (AMAN), and acute motor and sensory axonal neuropathy (AMSAN), are caused by antibodies to gangliosides on the axolemma that target macrophages to invade the axon at the node of Ranvier. About a quarter of patients with Guillain-Barré syndrome have had a recent Campylobacter jejuni infection, and axonal forms of the disease are especially common in these people. The lipo-oligosaccharide from the C jejuni bacterial wall contains ganglioside-like structures and its injection into rabbits induces a neuropathy that resembles acute motor axonal neuropathy. Antibodies to GM1, GM1b, GD1a, and GalNac-GD1a are in particular implicated in acute motor axonal neuropathy and, with the exception of GalNacGD1a, in acute motor and sensory axonal neuropathy. The Fisher's syndrome subtype is especially associated with antibodies to GQ1b, and similar cross-reactivity with ganglioside structures in the wall of C jejuni has been discovered. Anti-GQ1b antibodies have been shown to damage the motor nerve terminal in vitro by a complement-mediated mechanism. Results of international randomised trials have shown equivalent efficacy of both plasma exchange and intravenous immunoglobulin, but not corticosteroids, in hastening recovery from Guillain-Barré syndrome. Further research is needed to discover treatments to prevent 20% of patients from being left with persistent and significant disability.     

Primary antiphospholipid syndrome with and without Sneddon��s syndrome

The main objective of this study was to compare clinical and laboratory data obtained from patients with primary antiphospholipid syndrome (PAPS) with and without Sneddon’s syndrome (SS). A transverse study with 54 (85.2% female) PAPS patients (Sapporo criteria) was performed. Demographic, drug use, and antiphospholipid antibodies data were evaluated, as well as clinical and laboratory findings of SS. Patients were subdivided into one of two groups: PAPS with SS and PAPS without SS. Both groups were similar with respect to age (p = 0.05), gender (p = 0.34), race (p = 0.31), weight (p = 0.93), height (p = 0.27), and body mass index (p = 0.75); however, the SS group exhibited higher disease duration (96.0 ± 54.9 vs. 55.2 ± 52.0 months, p = 0.01). By definition, all PAPS with SS patients suffer from stroke, an arterial event; the frequency of stroke events (28.5 vs. 7.5%, p = 0.04), as well as of limb ischemia (100 vs. 30.0%, p < 0.0001) was higher in this group than in the PAPS without SS group. On the other hand, patients in the PAPS without SS group had more venous events, such as deep venous thrombosis, than those in the PAPS with SS group (80.0 vs. 50.0%, p = 0.03). In conclusion, an understanding of the relationship between APS and SS is important in order to identify a subgroup for which more rigorous accompaniment and therapy may be necessary.     

Lemierre’s syndrome

Lemierre’s syndrome is characterized by an oropharyngeal infection followed by internal jugular vein septic thrombophlebitis and metastatic emboli, most often to the lungs and joints. The syndrome is most commonly associated with the anaerobic gram-negative rod Fusobacterium necrophorum. Diagnosis is established with evidence of metastatic infection and internal jugular vein thrombophlebitis. CT is considered the diagnostic procedure of choice. Treatment should include an extended course of a b-lactamase-resistant antibiotic and surgical drainage of any purulent fluid collection. Anticoagulation remains controversial, and ligation of the internal jugular vein is reserved for patients with persistent sepsis and recurrent emboli. With appropriate therapy, mortality is 4% to 12%; but mortality is increased when therapy is delayed.     

Behçet syndrome

PURPOSE OF REVIEW: Interest in Beh?et syndrome (BS), although the condition is rare in many parts of the world, is increasing as judged by the growing number of related publications and scientific meetings. RECENT FINDINGS: Recent work reconfirmed the following without further insight into their meaning in pathogenesis: males have a more severe course; one third of the patients have a thrombophilia; papulopustular lesions and arthritis go together; gammadelta T are increased; and HLA B51 is still the main genetic association. Presence of increased levels of antibodies to Saccharomyces cerevisiae and the proposal of alpha-enolase as the target antigen of antiendothelial cell antibodies, known to be present also in BS, are observations that might help in delineating the unknown pathogenesis. For management, preliminary yet promising experience with the use of anti-tumor necrosis factor agents is being reported. SUMMARY When compared with 2 decades ago, we know considerably more and can do more about BS.     

Coronary subclavian steal syndrome

Coronary subclavian steal syndrome arises when a stenosis of the subclavian artery results in reduced antegrade or retrograde flow in an internal mammary artery with result- ant coronary ischemia. This occurs in patients who have previously undergone surgical coronary revascularization utilizing an internal mammary artery graft. This syndrome     

Sjögren's syndrome

Sj?gren's syndrome is an autoimmune disease characterized by inflammation of the exocrine glands, leading to impaired function. Here, I review the relatively short history of the syndrome and explain why it is frequently underdiagnosed, undertreated and under-researched. Attempts to provide classification criteria have culminated in the revised American-European Consensus Criteria, which provide a sound basis for both clinical management and research. The recognition that Sj?gren's syndrome is a disease of considerable morbidity has led to a more aggressive approach to therapy ranging from topical therapies to systemic treatment with secretagogues such as pilocarpine and cemiveline, and immunomodulatory drugs such as hydroxychloroquine and interferon-alpha. The central role of the glandular epithelial cell is identified as the key to understanding the pathogenesis of the disease. Hypofunction rather than destruction of these cells is now regarded as the main mechanism of secretory failure in Sj?gren's syndrome.