Reye syndrome and Reye-like syndrome

Reye syndrome (RS) is an acute metabolic encephalopathy and hepatopathy affecting children and adolescents. Outbreaks of RS were common in United States until the early 1980s. However, after the abolition of salicylate (aspirin) therapy for infectious diseases such as influenza or varicella in patients under 18 years of age the incidence decreased. Now classical RS is rare and RS is considered a secondary mitochondrial disease. Reye-like syndrome (RLS), resulting from congenital errors of mitochondrial fatty oxidation, especially medium-chain acyl-CoA dehydrogenase deficiency, has increased due to progress in diagnostic techniques and methods after 1990. Diagnostic differentiation between RS and RLS is difficult because the end results of mitochondrial dysfunction in RS and RLS may be similar.     

Brugada syndrome mimicking acute coronary syndrome

This report describes a 42-year-old man with Brugada syndrome (BRS) mimicking acute coronary syndrome. Chest pain, near-syncope, and electrocardiographic changes were thought initially to be due to ischemia. Cardiac catheterization was performed. The coronary arteries and left ventricular function were normal. Careful review of his electrocardiogram suggested a diagnosis of BRS. BRS may be confused with acute coronary syndrome; early recognition of this syndrome is essential, as implantable cardioverter-defibrillator therapy may be life-saving.     

Wolff-Parkinson-White syndrome concomitant with asymptomatic Brugada syndrome

A 29-year-old man was referred for electrophysiological testing and radiofrequency ablation because of repeated episodes of palpitation over 2 years. A 12-lead electrocardiogram during sinus rhythm showed manifest Wolff-Parkinson-White syndrome and during palpitation showed narrow QRS tachycardia at a rate of 213 beats/min. Following successful radiofrequency ablation of the left anterolateral accessory pathway, sustained atrial fibrillation was induced by atrial extrastimulation. Cibenzoline (2 mg/kg body weight) was injected to terminate atrial fibrillation. ST-T segment elevation in the right precordial leads was observed following cibenzoline administration. Ventricular fibrillation was reproducibly induced by ventricular extrastimuli (S1: 600 ms, S2: 220 ms, S3: 210 ms).     

The leopard syndrome, a cardio-cutaneous syndrome

A patient with the "leopard" syndrome presented with cardiomyopathy and a large arteriovenous shunt of the left renal vessels. The skin manifestations included multiple lentigines, junctional naevi, blue naevi, and one malignant lentigo. This syndrome is a rare entity, but in all patients with multiple lentigines the possibility of associated cardiovascular changes should be considered. In addition, regular monitoring of the pigmented lesions should be performed because of the increased risk of development of malignant melanoma.     

Sweet's syndrome associated with systemic inflammatory response syndrome

Septic shock is characterised by infection causing a systemic inflammatory response, end-organ failure and acute circulatory collapse. Treatment consists of antimicrobial therapy and the supportive management of multi-organ failure. We report a case of what we believed to be septic shock due to pyelonephritis in a patient whose condition continued to deteriorate despite conventional treatment until the diagnosis of Sweet's syndrome was made. Once she was started on high dose steroids, her condition improved and she made a full recovery. We believe this to be the first case of a severe systemic inflammatory response syndrome associated with Sweet's syndrome. Received: 20 January 1998 Accepted: 12 June 1998     

多囊卵巢综合征与代谢综合征关系的研究

目的 探讨多囊卵巢综合征(polycystic ovary syndrome,PCOS)、胰岛素抵抗及代谢综合征(metabolic syndrome,MS)的关系.方法 临床收集101例PCOS患者的血糖、胰岛素、血脂、性激素等指标,按稳态模型(HOMA)计算胰岛素抵抗指数(HOMA-IR),依照4个不同的标准--美国国家胆固醇教育计划成人治疗方案第三次报告(ATPⅢ)(2005年)、国际糖尿病联盟(IDF)(2005年)、中华医学会糖尿病学分会(CDS)(2004年)和<中国成人血脂异常防治指南>制定联合委员会(JCDCG)(2007年),计算PCOS患者中MS的发生率.并将患者分为PCOS伴MS和PCOS不伴MS两组就相关指标进行比较.结果 ①根据ATPⅢ(2005)、IDF(2005)、CDS(2004)、JCDCG(2007)MS诊断标准,PCOS中MS的发病率分别为45.5%、43.6%、31.7%、32.7%.②PCOS伴MS和PCOS不伴MS的患者临床指标的比较:在年龄、腰围、腰臀比(WHR)、收缩压、空腹血糖(FBG)、胰岛分泌指数(HOMA-β)、总胆固醇(TC)、低密度脂蛋白胆固醇(LDL-C)、高密度脂蛋白胆固醇(HDL-C)、黄体激素(LH)、卵泡刺激素(FSH)、雌二醇(E2)、睾酮(T)、泌乳素(PRL)方面,两组差异无统计学意义;在体质量指数(BMI)、舒张压、餐后2小时血糖(PBG)、空腹胰岛素、餐后2小时胰岛素、甘油三酯(TG)、血尿酸方面,PCOS伴MS较PCOS不伴MS显著升高,在HOMA-IR则显著降低,差异有统计学意义.结论 按照MS的4个定义--ATP Ⅲ(2005)、IDF(2005)、CDS(2004)、JCDCG(2007)标准,PCOS患者中均有较高的MS的患病率.PCOS伴MS患者存在较明显的胰岛素抵抗,肥胖尤其是腹型肥胖与MS、胰岛素抵抗密切相关.