代谢综合征对Z综合征的预测价值分析

目的观察代谢综合征对Z综合征的预测价值。方法选择高血压患者96例(男52例,女44例),分别记录性别、年龄、身高、体重、既往病史及服药情况,检测血压、血脂、血糖。所有患者行多导睡眠图监测,呼吸暂停低通气指数(apnea hypopnea index,AHI)≥5次/小时纳入阻塞性睡眠呼吸暂停低通气综合征(obstructive sleep apnea hypopneasyndrome,OSAHS)组,AHI<5次/小时者纳入非OSAHS组。结果年龄40～70岁以及超重或肥胖、难治性高血压、代谢综合征患者患OSAHS风险增加(OR=5.82～30.60,P<0.01),其中代谢综合征的相对危险度最大。OSAHS合并代谢综合征的患者中60.78%为重度OSAHS,OSAHS未合并代谢综合征的患者中20.00%为重度OSAHS。代谢综合征诊断OSAHS的敏感性为91.07%,特异性为75.00%,阳性预测值为83.61%,阴性预测值为85.71%。结论代谢综合征患者患OSAHS的风险较非代谢综合征患者明显增加,且患重度OSAHS的风险有增高趋势。     

妊娠高血压综合征并发妊娠肾病综合征22例临床分析

目的探讨分析妊娠肾病综合征临床特点和治疗方法.方法采用回顾分析方法,对2001年3月-2005年3月收治的22例妊娠高血压综合征(妊高征)并发妊娠肾病综合征与167例妊高征临床资料进行比较.结果妊娠肾病综合征发病孕周(27.8周)早于妊高征(31.4周,P<0.05).妊娠肾病综合征低蛋白血症发病率(100.0%),明显高于妊高征(42.0%,P<0.01).妊娠肾病综合征24 h尿蛋白定量(7.5 g/24 h)明显高于妊高征(4.1 g/24 h,P<0.05).妊娠肾病综合征血清清蛋白浓度(26.9 g/L)明显低于重度妊高征(30.6 g/L,P<0.05).妊娠肾病综合征胆固醇浓度(9.17 mmol/L)明显高于妊高征(7.4 mmol/L,P<0.05).妊娠肾病综合征新生儿出生体重(1 490 g)与妊高征(2 640 g)相比,有统计学意义(P<0.01).结论妊娠肾病综合征发病早,病情重,严重威胁孕产妇和胎儿安全.     

异位促肾上腺皮质激素综合征

<正>概述异位促肾上腺皮质激素(adrenocorticotropic hormone,ACTH)综合征(ectopic ACTH syndrome,EAS)作为库欣综合征的一种特殊类型,是因垂体以外ACTH的肿瘤组织分泌过量具有生物活性的ACTH,刺激肾上腺皮质增生并产生过量皮质类固醇引起的临床综合征,占库欣综合征患者总数的5%～10%^[1]。国外文献报道最多见病因为肺部或支气管肿瘤,约占50%,其次分别为胸腺及胰腺肿瘤,各约占10%,还可有甲状腺髓样癌(medullary thyroid carcinoma,MTC),嗜铬细胞瘤,胃肠道及生殖系统、前列腺等部位的肿瘤^[2]。     

Peutz-Jeghers综合征1例

Peutz-Jeghers综合征(PJS),又名黑色素斑-胃肠多发息肉综合征,以皮肤黏膜色素沉着,全胃肠道多发性息肉为特征,临床上较为少见,发病率约为1/2百万[1].我院收治了1例PJS成人患者.     

血清尿酸水平与代谢综合征相关

目的探讨血清尿酸水平与中国人群代谢综合征的关系。方法入选960例无心血管病症状的普通社区人群,男751人,女209人,平均年龄41.27岁,所有研究对象进行了详细的问卷调查和体检。计算分性别的血清尿酸四分位数,将研究对象分成4组,观察各组代谢综合征发生情况。结果男性中,代谢综合征患者的血清尿酸水平359.5±66.lumol/L,显著高于非代谢综合征患者319.4±66.7umol/L。女性中,代谢综合征患者的血清尿酸水平304.6±60.7umol/L,显著高于非代谢综合征患者242.9.4±52.9umol/L。血清尿酸四分位分组间代谢综合征的发生率分别为5.8%,8.2%,16.7%和24.1%。在校正了年龄、性别、吸烟状况,饮酒状况,糖尿病家族史、血压、血脂、腰围等混杂因素后,血清尿酸高的对象发生代谢综合征的危险是血清尿酸低的2.24（1.05-4.76）倍,P=0.036。结论血清尿酸水平与代谢综合征显著相关。     

血管活性药物治疗肝肾综合征

肝肾综合征(hepatorenal syndrome)是肝硬化后期的常见并发症,也可见于其他严重的肝脏疾病,如急性肝功能衰竭、酒精性肝炎等.肾功能减退为肝肾综合征的主要临床表现之一.临床上将肝肾综合征分为Ⅰ型肝肾综合征和Ⅱ型肝肾综合征两种类型.Ⅰ型肝肾综合征系指患者的血清肌酐水平在2周内升高至221μmol/L(2.5 mg/dL)以上,肾小球滤过率通常低于20 ml/min,进行性少尿,临床表现为严重的肾功能衰竭;而Ⅱ型肝肾综合征主要表现为顽固性腹水,经一段时期后病情可突然恶化,达到Ⅰ型标准.     

Budd—Chiari综合征合并肝肺综合征二例报告

Ｂｕｄｄ -Ｃｈｉａｒｉ综合征系由肝静脉和 /或肝静脉出口以上的下腔静脉 (ｉｎｆｅｒｉｏｒｖｅｎａｃａｖａ ,ＩＶＣ)阻塞所致 ,但Ｂｕｄｄ -Ｃｈｉａｒｉ综合征合并肝肺综合征(Ｈｅｐａｔｏｐｕｌｍｏｎａｒｙｓｙｎｄｒｏｍｅ ,ＨＰＳ)鲜见报道 ,本文报道二例 ,并就ＨＰＳ的发病机制进行探讨。病例 1:患者男 ,31岁 ,农民 ,因双下肢浮肿 3年 ,胸、腹壁静脉曲张 2年 ,劳力性呼吸困难 6月入院。既往无特殊病史和不良嗜好。体查 :一般情况可 ,皮肤巩膜无黄染 ,未见蜘蛛痣和杵状指 ,口唇及甲床微绀 ,腹壁及双侧胸下壁可见显著的静脉曲张 ,血…     

Timothy综合征

1989年,1例反复晕厥的儿童来到了犹他大学Timothy教授的办公室,Timothy教授发现这个孩子不仅有心律失常和心电图中的QT间期延长,还有并指/趾,随后对患儿的临床表现进行了报道,此后,人们以Timothy教授命名了这一疾病,称为＂Timothy综合征（TimothySyndrome,TS）＂,也称为长QT综合征8型。     

POEMS综合征血清血管内皮生长因子检测意义

目的探讨血清血管内皮生长因子(VEGF)在POEMS综合征中的临床意义。方法采用ELISA方法检测POEMS综合征组(36例)、疾病对照组(多发性骨髓瘤29例、格林巴利综合征7例共36例)、正常对照组(36例)的血清VEGF浓度,进行组间比较,并与临床资料进行相关性分析。结果 POEMS综合征组血清VEGF浓度为(982.06±611.49)pg/ml,显著高于疾病对照组[(364.22±264.37)pg/ml]和正常对照组[(217.37±87.74 pg/ml)],P<0.001。VEGF诊断POEMS综合征曲线下面积为0.902,VEGF Cutoff值为360.62 pg/ml,敏感性为0.889,特异性为0.764。POEMS综合征组血清VEGF与肺动脉高压、外周血小板计数相关,与其他临床表现无显著相关性。结论血清VEGF浓度升高是POEMS综合征的一个重要特征,有助于POEMS综合征的诊断;血清VEGF浓度与肺动脉高压有一定相关性,可能是肺动脉高压发病机制之一。     

心脏X综合征与血尿酸水平的相关性研究

目的研究心脏X综合征与血尿酸水平的关系。方法回顾性调查60例心脏X综合征患者、200名健康体检者血尿酸、血糖、血脂等指标水平,同时进行血尿酸与心脏X综合征的相关性、危险因素分析。结果心脏X综合征组尿酸水平为(418.64±46.78)μmol/L、血糖水平为(5.46±1.43)mmol/L与对照组[(336.45±34.98)μmol/L,(5.09±1.01)mmol/L]比较差异有统计学意义(均为P<0.05),两组血脂水平比较差异无统计学意义(P>0.05)。病例对照研究,表明尿酸与心脏X综合征的发生有关联(OR:4.19,95%CI:2.259～7.768,P<0.01)。结论血尿酸水平升高与心脏X综合征的发生具有一定的相关性。     

Long QT syndrome with insulin-dependent diabetes mellitus: contiguous gene syndrome on chromosome 11p

Abstract. Long QT syndrome (Romano-Ward syndrome) and insulin-dependent diabetes mellitus (IDDM) have been documented as being linked with gene(s) on chromosome 11p although concurrence of the two disorders has not been reported. Our case is a 13-year-old boy with Romano-Ward syndrome accompanied by IDDM. The long QT syndrome seemed to be transmitted in an autosomal-dominant mode because the Q-T intervals of his father and paternal grandfather were longer than normal. There was no family member with an abnormally high level of blood glucose except the patient. The human leucocyte antigen (HLA) haplotypes of the patient and the father were DR4/DR9 and DR2/DR9, respectively. This study suggests that in our patient IDDM, as well as Romano-Ward syndrome, is linked with chromosome 11p in the presence of HLA-DR4. The results support the previous study that chromosome 11p encodes a gene implicated in HLA-DR4-dependent diabetes susceptibility.     

Fatal Evans' syndrome after matched unrelated donor transplantation for hyper-IgM syndrome

A 3 and 1/2-yr-old boy underwent matched unrelated stem cell transplantation (SCT) for hyper-IgM syndrome. He developed acute and chronic skin graft-vs.-host disease (GVHD). Ten months following SCT he presented with severe hemolytic anemia and thrombocytopenia (Evans' syndrome). Treatment included high-dose steroids, intravenous immunoglobulins, cyclosporine, mycophenolate mofetil, chemotherapeutic agents (cyclophosphamide, vincristine, VP-16), immunoadsorption, and anti-CD20 and anti-CD52 monoclonal antibodies without response. The patient died 16 months after SCT.     

全身炎症反应与肝肾综合征关系的研究

目的分析肝衰竭患者并发肝肾综合征(HRS)与全身炎症反应(SIRS)的关系。方法在137例肝衰竭患者中,合并HRS68例,单纯肝衰竭69例。收集两组患者Child-Pugh分级、终末期肝病模型(MELD)和SIRS评分。结果 HRS患者SIRS评分为0.70±0.86分,高于对照组0.36±0.54分(P<0.05),白细胞计数为8.96±4.45×109/L,显著高于对照组(5.79±2.40×109/L,P<0.05);HRS患者总胆红素和凝血酶原时间分别为404.5μmol/L和21.7±8.8s,高于对照组(259.0μmol/L和17.6±9.6s,P<0.05),而钠和氯分别为132.6±6.0mmol/L和92.2±9.1mmol/L,显著低于对照组(137.2±3.8mmol/L和99.5±9.3mmol/L,P<0.05)。结论 SIRS与肝衰竭患者并发HRS关系密切。     

异位ACTH综合征6例临床分析

目的提高临床医生对异位ACTH综合征的认识。方法对四川大学华西医院1996—2005年确诊的6例异位ACTH综合征患者的临床资料及诊治过程进行回顾性分析。结果副节瘤1例,神经内分泌癌1例,肺癌4例。临床特征病程15d至2个月,6例患者均有严重低血钾伴碱中毒,5例患者有高血压和双下肢水肿。Cushing综合征表现满月脸4例,淤斑和长胡须2例,多血质、痤疮、色素沉着各1例,血皮质醇(晨起8时)>1173nmol/L,大剂量地塞米松(8mg)抑制试验均不能抑制。结论对病程短,严重低血钾碱中毒,水肿明显而Cushing临床表现不明显者,血皮质醇>1000nmol/L应高度怀疑异位ACTH综合征。寻找病因,尽快手术切除肿瘤,近期预后较好。     

Carvedilol therapy for patients with Eisenmenger syndrome

Background: Eisenmenger syndrome is characterized by the development of pulmonary arterial hypertension with resultant intracardiac right-to-left shunt and hypoxemia in patients with congenital heart defects. In clinical practice, we found that these patients might benefit from carvedilol therapy. Thus, we designed this prospective, open label, observational study to evaluate the efficacy of carvedilol for patients with Eisenmenger syndrome. Methods Twenty patients(17.5 ± 4.8 years) with Eisenmenger syndrome were treated with carvedilol for 6 weeks. The efficacy of carvedilol on 6-minute walking distance, World Health Organization(WHO) functional class, arterial oxygen saturation and right ventricle systolic pressure were assessed.Results At the end of observation, change from baseline in 6-minute walking distance increased 36.35 meters(95% confidence intervals [CI] 25.43 to 47.27 m, P 0.01). WHO functional class was also significantly improved(P 0.05). Change from baseline in right ventricular systolic pressure assessed by echo was reduced by 8.11 mm Hg(95% confidence intervals [CI],-10.78 to-5.44 mm Hg, P 0.05). However, arterial oxygen saturation remained unchanged(87.5 ± 3.02 % at baseline versus 87.80 ± 7.29 % at the end of observation). Conclusions Carvedilol can improve clinical function, symptoms as well as exercise capacity in patients with Eisenmenger syndrome. These findings need to be confirmed in further randomized clinical trials.     

Nephrotic syndrome and acute coronary syndrome in children,teenagers and young adults: Systematic literature review

Myocardial infarction is rare in children, teenagers and young adults (aged < 20 years). The most common aetiologies identified include Kawasaki disease, familial hypercholesterolaemia, collagen vascular disease-induced coronary arteritis, substance abuse (cocaine, glue sniffing), trauma, complications of congenital heart disease surgery, genetic disorders (such as progeria), coronary artery embolism, occult malignancy and several other rare conditions. Nephrotic syndrome is a very rare cause of myocardial infarction, but it is probably underestimated. The purpose of this review was to determine the current state of knowledge on acute coronary syndrome related to nephrotic syndrome. We thus performed a comprehensive structured literature search of the Medline database for articles published between January 1st, 1969 and December 31st, 2021. Myocardial infarction in young adults can be broadly divided into two groups: cases of angiographically normal coronary arteries; and cases of coronary artery disease of varying aetiology. There are several possible mechanisms underlying the association between acute coronary syndrome and nephrotic syndrome: (1) coronary thrombosis related to hypercoagulability and/or platelet hyperactivity; (2) atherosclerosis related to hyperlipidaemia; and (3) drug treatment. All of these mechanisms must be evaluated systematically in the acute phase of disease because they evolve rapidly with the treatment of nephrotic syndrome. In this review, we propose a decision algorithm for the management of acute coronary syndrome in the context of nephrotic syndrome. The final part of the review presents the short- and medium-term therapeutic strategies available. Thromboembolism related to nephrotic syndrome is a rare non-atherosclerotic cause of acute coronary syndrome, and prospective studies are needed to evaluate a systematic approach with personalized therapeutic strategies.     

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文章介绍了重叠综合征的定义、流行病学现状、主要临床特征、诊断方法思路及治疗方法,重点比较重叠综合征与单纯慢性阻塞性肺疾病(COPD)及睡眠呼吸暂停低通气综合征临床表现的异同,有助于加强对重叠综合征的认识。     

Brugada syndrome in a 4-year-old child with Lemierre syndrome—A case report

Brugada syndrome is a rare arrhythmogenic disease with characteristic electrocardiogram (ECG) findings. Fever represents an important triggering factor. We report the case of a 4-year-old Saudi boy who was diagnosed with Lemierre syndrome and subsequently developed Brugada syndrome.     

重叠综合征

慢性阻塞性肺疾病（COPD）是一种以气流受限不完全可逆为特征的常见的慢性呼吸系统疾病。睡眠呼吸暂停低通气综合征（SAHS）也是一种具有潜在危险的常见病。故COPD与SAHS合并存在者即重叠综合征患者的概率相当高。COPD患者睡眠时显著的低氧血症和高碳酸血症发生于快动眼睡眠期。重叠综合征患者较单纯SAHS或COPD患者有更为严重的睡眠有关的低氧血症,表现出更为严重的肺功能损害和肺动脉高压。因此,重叠综合征患者的及时诊断和合理治疗具有重要临床意义。     

Primary fibromyalgia and the chronic fatigue syndrome

Summary Thirty-three primary fibromyalgia patients were investigated for chronic fatigue syndrome symptoms. Significant fatigue was reported by 21/33 patients (63.6%), and patients reported various flulike symptoms, yet only 7/33 patients (21.2%) fulfilled criteria for the chronic fatigue syndrome. Only one patient reported painful lymph glands and four patients reported fever. Thus, symptoms of painful glands or fever might serve as clinical indicators, distinguishing between fibromyalgia and the chronic fatigue syndrome.