Systemic capillary leak syndrome

A man aged 61 had recurrent attacks of severe shock. The episodes were preceded by symptoms such as a runny nose, epigastric discomfort with nausea, vertigo, orthostatism and sometimes light fever. During the attacks there were marked hypotension, a strong rise of the haematocrit, a decrease of the protein and albumin concentrations in the blood and prerenal kidney failure. In addition, there was a paraprotein, type IgG-kappa. The shock every time responded rapidly to intravenous administration of fluid and was followed by a period of substantial polyuria. The pattern was characteristic of systemic capillary leak syndrome, a rare but frequently fatal disease characterized by episodes of unexplained extravasation of plasma. The aetiology and pathogenesis are unknown. Attacks are suppressed by supportive therapy (administration of fluids, inotropics) and future attacks may be prevented by the intake of terbutaline and theophylline. The systemic capillary leak syndrome should be considered in the differential diagnosis of idiopathic and anaphylactic shock.     

Fatal Systemic Capillary Leak Syndrome after SARS-CoV-2Vaccination in Patient with Multiple Myeloma

A young man with smoldering multiple myeloma died of hypotensive shock 2.5 days after severe acute respiratory syndrome coronavirus 2 vaccination. Clinical findings suggested systemic capillary leak syndrome (SCLS); the patient had experienced a previous suspected flare episode. History of SCLS may indicate higher risk for SCLS after receiving this vaccine.     

Fatal Exacerbations of Systemic Capillary Leak Syndrome Complicating Coronavirus Disease

We report 2 fatal exacerbations of systemic capillary leak syndrome (SCLS), also known as Clarkson disease, associated with coronavirus disease (COVID-19) in the United States. One patient carried an established diagnosis of SCLS and the other sought treatment for new-onset hypotensive shock, hemoconcentration, and anasarca, classic symptoms indicative of an SCLS flare. Both patients had only mild-to-moderate symptoms of COVID-19. This clinical picture suggests that these patients succumbed to complications of SCLS induced by infection with severe acute respiratory syndrome coronavirus 2. Persons with known or suspected SCLS may be at increased risk for developing a disease flare in the setting of mild-to-moderate COVID-19 infection.     

埃博拉病毒病的发病机制和诊疗策略

埃博拉病毒是一种强致死性病原体,可引起类似于感染性休克的严重出血热——埃博拉病毒病。埃博拉病毒病在2014年西非地区发生历史上最大的大流行疫情,其临床特点为凝血功能障碍、毛细血管渗漏综合征和休克。迄今为止尚无针对性抗病毒药物治疗。本文从病原学、感染途径、发病机制、临床特征、辅助检查、诊断及治疗特点等方面探讨埃博拉病毒病的机制和诊疗策略。     

Hantavirus infections

Hantaviruses are the causative agents of the zoonotic diseases known as haemorrhagic fever with renal syndrome (HFRS) in Europe and Asia, and hantavirus pulmonary syndrome (HPS) in the Americas. These pathogens are maintained in the wild by rodent reservoirs and are mainly transmitted via the aerosol route. The infection is chronic and apparently asymptomatic in host animals. Whilst HFRS is caused by Hantaan, Seoul, Dobrava and Puumala hantaviruses, HPS is associated with Sin Nombre-like viruses. Common clinical features of HFRS and HPS include fever, myalgia, thrombocytopenia, leukocytosis and a capillary leak syndrome associated with shock in most severe cases. Outbreaks of HFRS and HPS are generally observed during years with dense rodent populations resulting from favourable climatic and environmental conditions. Human activities, such as rodent trapping, farming, cleaning rodent-infested areas, construction work, camping and hunting, are also implicated in the occurrence of hantavirus disease. Prophylactic measures in endemic areas rely essentially on information campaigns and rodent control.     

Comments on Raynaud syndrome based on laser-Doppler studies

The authors have in their care 286 Raynoud syndrome patients. They present their experience in the study of the microcirculation and the treatment of these patients. In 81% (232 patients, 196 women and 36 men) of the cases they did not find any underlying diseases. In the group of the patients with secondary Raynaud's syndrome (35 women and 19 men) underlying diseases, possibly leading to microcirculatory disturbances, could be identified. In the primary Raynaud's syndrome group the main capillary diameter measured by capillary microscopy was 15 +/- 4 micron. In patients with progressive systemic sclerosis the mean diameter was 43 +/- 9 micron. In the group of women with primary Raynaud's syndrome the authors performed laser Doppler measurements of the nailfold microcirculation. During the reactive hyperaemia test basal values (18.2 +/- 6.2 Perfusin Units, P. U.) and reactive hyperaemia values (a mean of 180% growth compared to the basal values) were similar to that perfusion values referred in the literature. In the progressive systemic sclerosis group the basal flow (9.3 +/- 3.3 P. U.) and the reactive hyperaemia (a mean of 20% increase compared to basal values) were significantly lower. Concerning the fact that the progression of Raynaud's syndrome into systemic autoimmune disease--mainly to progressive systemic sclerosis may be predicted, it is important to follow up carefully all patients with Raynaud's syndrome.     

Generalized mastocytosis; to be considered following resuscitation

This case report describes a male patient aged 44 with recurrent hypotensive shock and severe pulmonary obstruction. The diagnosis of systemic mastocytosis was suggested by the presence of small pigmented maculae (urticaria pigmentosa) and intolerance to NSAIDs. The diagnosis was confirmed by microscopic investigation of the pigmented maculae and bone. The patient was advised to avoid certain foods and drugs and has so far been free of symptoms. In patients with hypotensive shock and (or) severe pulmonary obstruction without a clear cause, systemic mastocytosis should be included in the differential diagnosis.     

感染性休克死亡率和危险因素分析

目的分析感染性休克的死亡率及其危险因素。方法回顾总结2002年1月-2005年10月110例感染性休克患者临床资料,采用单因素方差分析和多因素Logistic回归对死亡因素进行分析。结果110例感染性休克患者死亡率为67.2%,全身炎症反应综合征(SIRS)评分、器官衰竭个数是感染性休克死亡危险因素,器官衰竭个数是强危险因素(OR=55.184)。结论感染性休克病死率极高,防治器官功能衰竭是改善预后的关键。     

全身炎症反应综合征的分子发病机制及其治疗研究

全身炎症反应综合征(SIRS)是机体对感染性或非感染性因素产生的反应,是严重感染、败血症和休克发展到多器官功能不全(MODS)的共同通路。在其发生发展过程中,存在着多种调节因子的相互作用以及促炎反应和抗炎反应的平衡和失衡,深入研究其分子机制,将为SIRS的临床治疗提供新的途径。此文综述了SIRS的病理生理过程及分子发病机制,涉及到炎症细胞激活、细胞因子产生及其主要的信号转导通路等,在此基础上,扼要介绍了生物反应调节治疗方面的一些研究进展。     

Toxic shock syndrome: broadening the differential diagnosis

BACKGROUND: Toxic shock syndrome is a rare but potentially fatal toxin-mediated febrile illness. Although classically associated with tampon use, it is now known that many nonmenstrual conditions are related to this syndrome. Serious morbidity and mortality can occur if this syndrome is not promptly recognized. METHODS: MEDLINE was searched from 1978 to the present using the phrase "toxic shock syndrome." Case reports and articles related to tampon-associated toxic shock syndrome were excluded from the literature review except when defining toxic shock syndrome or discussing the cause of the syndrome. A case of nonmenstrual toxic shock syndrome associated with an intrauterine device and a review of the definition, cause, diagnostic criteria, and management are reported. RESULTS AND CONCLUSIONS: Toxic shock syndrome can mimic many common diseases. Because it can be associated with a number of nonmenstrual-related conditions, patients with unexplained fever and rash and a toxic condition out of proportion to local findings should have the diagnosis of toxic shock syndrome in their differential diagnosis. Early recognition and aggressive management can decrease the overall morbidity and mortality.     

生脉注射液对失血性休克促炎因子TNF-a和IL-6影响的研究进展

休克时组织细胞的低灌注可诱导细胞因子过度释放导致全身炎症反应综合征(SIRS),因此,在失血性休克早期采取有效措施抑制细胞因子的产生释放及进行有效的抗炎治疗,对遏制失血性休克向不可逆方向转化具有重要意义。中药生脉注射液在失血性休克的治疗中具有明显作用,本文通过文献复习,对失血性休克复苏过程中血浆中两个代表性细胞因子TNF-a、IL-6含量变化进行归纳综述,为临床对生脉注射液的进一步研究提供参考。     

自发性脑脊液漏致原发性低颅压综合症

[目的]探讨自发性脑脊液漏与原发性低颅压综合症的相互关系,其临床特点、诊断及治疗。[方法]分析了两例以原发性低颅压综合症为临床表现的自发性脑脊液鼻漏患者的临床资料,并复习这两类疾病的相关文献。[结果]两例患者均突发起病,表现为额颞部剧痛后出现恶心、呕吐及体位性头痛,用力及咳嗽时症状加重,平卧时缓解。腰穿颅内压力为0,脑脊液中红细胞、微量蛋白增加。脑池核素显像两例均发现脑脊液鼻漏,其中1例伴有颈胸段脑脊液漏。[结论]自发性脑脊液鼻漏是原发性低颅压综合症的致病因素之一,脑池核素显像可以明确诊断,补液治疗可以获得临床治愈。     

连续肾替代治疗在外科严重脓毒症并发毛细血管渗漏综合征中的治疗价值

目的 探讨连续肾替代治疗在外科严重脓毒症毛细血管渗漏中的临床治疗价值.方法 38例脓毒症患者按治疗方案不同分为实验组和对照组,对照组常规综合治疗,实验组在常规综合治疗的基础上加连续肾替代治疗.分别监测两组患者治疗后（0、12、24、48、72 h）红细胞计数、血红蛋白含量、血小板计数、白细胞计数、红细胞压积、血浆白蛋白含量变化.中心静脉压（CVP）、有创动脉压（ABP）、每小时尿量变化、氧合指数情况.血清IL-6、IL-8、TNF-α水平变化.结果 实验组血小板计数、红细胞压积、血浆白蛋白含量明显高于对照组[72 h：（211.75±45.23）×109 vs（135.67±41.45）×109;0.43±0.05 vs 0.35±0.04;（48.60±4.76）g/L vs（41.17±4.64）g/L,P〈0.01],白细胞计数低于对照组[72 h：（7.58±2.31）×109 vs（13.77±2.67）×109,P〈0.01],有创动脉压（ABP）、氧合指数P02／Fi02、每小时尿量变化高于对照组[72 h：（94.25±8.60）mmHg vs（84.22±7.37）mmHg;345.25±35.21 vs 304.22±38.74;（80.15±14.54）ml vs（62.72±12.33）ml,P〈0.01],血清IL-6、IL-8、TNF-α水平显著低于对照组（72 h：249.55±99.60 vs 368.83±97.11;600.75±98.31 vs 718.94±92.00;665.35±138.44 vs 843.22±123.95,P〈0.01,P〈0.05）.结论 连续肾替代治疗是治疗外科严重脓毒症毛细血管渗漏的有效治疗方法,明显改善患者病情.     

Is the primary antiphospholipid syndrome a forerunner of SLE?

INTRODUCTION: Antiphospholipid syndrome is a multi-organ autoimmune disorder, characterized by arterial and venous thrombotic events, and a well-defined group of recurrent foetal wasteage due to pathologic antibodies against phospholipids and protein co-factors. Antiphospholipid antibodies can be formed in primary antiphospholipid syndrome, but also in other conditions, most often in systemic lupus erythematosus. This may modify the outcome of lupus increasing the risk for thrombotic complications. Less is known about the outcome in primary antiphospholipid syndrome, whether it may precede the development of systemic lupus. AIMS: Authors hereby describe the case of four patients with primary antiphospholipid syndrome in whom the disease progressed to systemic lupus erythematosus. RESULTS: Lupus followed the primary antiphospholipid syndrome within around a three-year period. It was indicated by the appearance of different antinuclear autoantibodies and clinical complications, such as polyarthritis, nephritis and hematologic disturbances. All of the patients presented cerebrovascular accident as the thrombotic manifestation. All but one, were around forty years old, and had a milder form of lupus. Symptoms of the antiphospholipid syndrome determined the outcome. On the other hand, a typical lupus developed in the youngest patient. CONCLUSIONS: According to present cases antiphospholipid syndrome may be considered as the initiative phase of SLE, but APS being a separate entity also may associate to lupus. Present observations indicate the importance of follow-up the patients with APS by immunologic respect. Future prospective, multi-centre studies with larger number of cases are needed to provide further evidence on the fact that patients with APS may acquire other autoimmune disorder.     

Immune-modulatory actions of arginine in the critically ill

Current trials of immune-enhancing diets suggest several beneficial clinical effects. These products are associated with a reduction in infectious risk, ventilator days, ICU and hospital stay. However, methodological weaknesses limit the inferences we can make from these studies. Furthermore, improvements in outcomes were largely seen in surgical patients and in patients who tolerated critical amounts of formula. We propose that the beneficial findings cannot easily be extrapolated to other patient populations since there is suggestion from clinical trials that the sickest patients, especially those with severest appearances of sepsis, shock and organ failure may not benefit or may even be harmed. In these conditions we hypothesize that systemic inflammation might be undesirably intensified by immune-enhancing nutrients like arginine in critically ill patients. In this paper, we review the purported effects of arginine on the immune system and organ function to understand the scientific rationale for its inclusion into enteral feeding products. We conclude that patients with the most severe appearances of the systemic inflammatory response syndrome should not receive immune-enhancing substrates which may aggravate systemic inflammation and worsen clinical outcomes.     

Toxic shock syndrome. I. Clinical exclusion of other syndromes by strict and screening definitions

Several clinical definitions of toxic shock syndrome have been proposed and used in extensive epidemiologic and clinical studies. Most of these definitions suggest (but usually do not require) that there be sufficient laboratory studies to exclude other potentially similar syndromes. Simplified definitions which broaden the spectrum of toxic shock syndrome illness have also been proposed but not validated. In this study, clinical findings of consecutive hospitalized patients were compared: nine with toxic shock syndrome (confirmed by a modification of the collaborative strict case definition) and 120 with potentially similar diagnoses (bacteremia with shock, meningococcemia, Staphylococcus aureus bacteremia, scarlet fever, toxic epidermal necrolysis, acute rheumatic fever, leptospirosis, Rocky Mountain spotted fever, rubeola, Kawasaki syndrome, erythema multiforme, and Stevens-Johnson syndrome). None of the 120 controls satisfied the clinical criteria of the modified strict definition of toxic shock syndrome, demonstrating its exclusionary properties even in the absence of additional laboratory data. A "simplified" screening definition was constructed which might be applied early in illness (i.e., at admission) and this definition distinguished all the patients with toxic shock syndrome from all but three (2.5%) of the 117 analyzable patients with other mucocutaneous or potential infectious shock syndromes. Applied prospectively in the state of Colorado passive/active reporting system, the screening definition identified 24 potential toxic shock syndrome cases of which 19 (76%) eventually were confirmed as toxic shock syndrome. Before being adopted and widely used, clinical syndrome definitions should be documented to exclude other potentially overlapping syndromes or should require additional mandatory exclusionary laboratory data.     

浅谈感染性休克血管活性药物的运用

休克的微循环学说问世以来，对感染性休克的救治无疑起了非常积极的指导作用。然而就感染性休克微循环障碍各期、特别是微血管扩张期（即淤血性缺氧期；高排低阻型休克；暖休克）如何运用血管活性药尚无一致意见。本文就此从微循环障碍理论本身谈了一些初浅认识以期斧正。     

Predictive factors of recurrent hemorrhage in duodenal ulcer: report of 208 cases

The aim of this study was to evaluate clinical, biological and endoscopic predictive factors of recurrent bleeding. Two hundred and eight patients (169 males/39 females) with a bleeding from duodenal ulcers, confirmed by an endoscopy, were included in a retrospective study performed over 3 years. Forrest classification was used to describe endoscopic stigmata of bleeding. Those who are Forrest la and lb were directly operated. Twenty two patients (10,5%) had rebled. Based on bivariate analysis, the predictive factors of recurrent bleeding were the site of ulcer, a shock at admission, ulcer size D 2.0 cm and the presence of stigmata of bleeding (Forrest II) p<0,05. Only endoscopic stigmata of bleeding and shock were predictive factors of recurrent bleeding in multivariate analysis. Mortality with 2.4% of rate, was strongly linked with recurrent bleeding in elderly and ill people.     

新生儿毛细血管渗漏综合征的早期预测与药物治疗

毛细血管渗漏综合征(CLS)是NICU常见的危重症之一,死亡率高,其临床表现复杂,分期界限模糊,常因其他合并症而易被忽视。目前尚无明确的诊断标准,主要根据临床表现及实验室检查来诊断。治疗原则主要包括控制原发疾病及液体复苏等,常用药物包括抗生素、羟乙基淀粉、白蛋白、血制品、血管活性药等。了解其发病机制及临床表现,通过早期预测,早期干预,对提高CLS患儿生存率和预后有重要意义。