Cronkhite-Canada综合征1例

Cronkhite-Canada综合征(Cronkhite-Canada syndrome,CCS)非常罕见,病因不明,以胃肠道多发性息肉和外胚层三联征两大症候群为主.主要表现为慢性腹泻、腹部不适、毛发脱落、色素沉着、指(趾)甲萎缩等.本文报道CCS 1例,通过病史及内镜检查结果并结合相关文献对该病进行分析讨论,探讨CCS的临床特征,提高对该病的认识.     

Cronkhite-Canada综合征1例报道

Cronkhite-Canada综合征(Cronkhite-Canada syndrome,CCS)发病罕见,病因不明,是以胃肠道多发息肉和外胚层三联征两大症候群为主,临床表现为慢性腹泻、腹痛、脱发、皮肤色素沉着、指(趾)甲萎缩脱落等。本文报道CCS 1例。     

Cronkhite-Canada综合征1例

Cronkhite-Canada 综合征(Cronkhite-Canada syndrome, CCS),又称息肉-色素沉着-脱发-指(趾)甲营养不良综合征.该病最早在1955 年由Cronkhite和Canada首先报道[1], 1966年命名为Cronkhite-Canada syndrome[2],是临床上一类非...     

Cronkhite-Canada综合征国内文献复习

胃肠道息肉-色素沉着-秃发-指(趾)甲萎缩综合征(Cronkhite-Canada syndrome，CCS)是由Cronkhite和Canada于1955年首先报道的一组临床综合征。国内1985年首次报告本病，现就山西医科大学第二医院收治的1例患者及国内发表有关文献24篇加以总结分析。     

Cronkhite-Canada综合征临床特征及发病机制初探

Cronkhite-Canada综合征(CCS)又称息肉-色素沉着-脱发-爪甲营养不良综合征(polyposispig-mentation-alopecia-onycholrophia syndrome),是临床上极为罕见的一类非遗传性疾病。目前关于CCS的发病机制知之甚少,有研究表明炎症免疫反应可能是CCS主导因素,RiegertJohnson等[1]和Sweetser等[2]先后通过免疫组织化学(免疫     

中西医结合诊治Cronkhite-Canada综合征1例并文献复习

Cronkhite-Canada综合征(Cronkhite-Canada syndrome, CCS)一种临床罕见的非遗传性疾病，以胃肠道多发息肉和外胚层三联征为主要特征，临床症见消化道症状、皮肤色素沉着、脱发、爪甲脱落等。西医治疗CCS缺乏明确有效的治疗手段，多以经验性对症治疗为主。中医根据辨证论治，针对本例患者，辨证为脾虚夹痰热瘀毒滞肠证，治以健脾和胃、祛邪化积，先后予以口服清营汤和乌梅汤，经治疗2周后，患者好转出院。本文探讨中西医结合诊治1例CCS并文献复习，以供临床医师参考。     

Cronkhite-Canada综合征1例及国内文献复习

Cronkhite-Canada综合征(Cronkhite-Canadasyndrome,CCS)在国内又被称为息肉病-色素沉着-秃发-指甲萎缩综合征,临床较罕见。现报告我院1例并复习国内文献15例如下。病例报告该患者男性,70岁。1990年出现反复腹胀。1995年无明显诱因双手指甲全部脱落,后再次长出,但指甲增厚,无光     

Cronkhite-Canada综合征合并桥本甲状腺炎1例

Cronkhite-Canada综合征(CCS)是一种罕见的非遗传性疾病, 主要表现为胃肠道弥漫性息肉病和外胚层发育不良(如脱发、指甲萎缩和皮肤色素沉着), 以及味觉减退、腹泻、体重减轻、贫血、低蛋白血症等, 目前治疗策略尚未统一。此病确切病因尚不明确, 可能与自身免疫功能紊乱有关, 常合并其他自身免疫病, 但全球范围内相关报道仍不多见。现报告1例CCS合并幽门螺杆菌感染、桥本甲状腺炎、原发性甲状腺功能减退症患者, 针对疾病发展特点、潜在机制和诊疗体会进行分析总结, 供临床参考。     

Cronkhite—Canada综合征

本综合征于1955年由Cromkhite和Cana-da最先报告,故目前国际上统一称为Cronkhite-Canada syndrome(简称CCS).CCS的临床特征是患者有胃肠道多发性息肉、重度腹泻、低蛋白血症,同时伴有外胚层某些器官的发育障碍或退化提前,如毛发脱落、皮肤色素沉着和指(趾)甲萎缩等,国内学者通常称之为胃肠道息肉病-秃发-色素沉着-指甲萎缩综合征或多发性消化道息肉综合征.     

Cronkhite-Canada综合征1例临床病理分析

Cronkhite-Canada综合征(Cronkhite-Canada syndrome,CCS)是一种罕见的非遗传性疾病,以外胚层异常伴弥漫性息肉病为特征。现报道1例CCS。     

12例心电图异常患者的传导系统病理改变观察

目的探讨心脏传导系统（CCS）病变与心律失常及心电图ST-T改变的关系。方法对12例有临床心电图异常改变的心脏标本作CCS组织学观察。结果（1）12例心电图异常者均见CCS有病变,包括有CCS脂肪浸润和（或）纤维化、淀粉样变、炎症、发育异常及出血等。（2）组织学显示急性炎症性改变者的心电图改变主要有：窦性心动过速、频发多源性室性早搏。（3）组织学显示慢性退行性改变者的心电图改变复杂多样：包括慢-快综合征、窦性心动过缓并室内分支传导阻滞、房室传导阻滞及心室内传导阻滞。（4）CCS病变可有心电图ST-T改变。结论CCS病变是心律失常及心电图ST-T改变的病理基础之一。     

Cronkhite-Canada综合征2例报告并国内外文献评价

目的总结Cronkhite—Canada综合征（CCS）的临床表现、消化道息肉特点、病理改变及诊断治疗，以提高临床医生对本病的认识。方法对我院诊断明确的2例CCS病例及1990年以来72篇文献报告的31例病例的临床资料回顾性研究。结果符合CCS诊断标准的入组病例33例，男20例，女13例；年龄17～77岁，中位年龄51岁。临床表现为脱发、指（趾）甲改变、皮肤色素沉着、腹部不适及低蛋白血症。全部患者均有消化道多发息肉，累及胃、小肠、大肠。息肉病理无特异性，其中8例癌变。治疗方法包括激素治疗17例，内镜下息肉摘除2例，因癌变、消化道梗阻及蛋白丢失性肠病手术11例（3例同时激素治疗）和其他治疗6例，其中1年内激素治疗有效14例，最长1例随访7年无复发。结论Cronkhite—Canada综合征（CCS）是以消化道多发息肉伴外胚层改变为主要临床特点的综合征，诊断较困难，激素治疗能改善症状，长期疗效仍待进一步研究。     

幽门螺杆菌培养上清液诱发小鼠胃粘膜组织损伤的研究

通过动物实验证实幽门螺杆菌(Hp)的细胞毒素对小鼠胃粘膜具有损害作用。用不同剂量的产毒 Hp 菌株(NCTC11637)的培养上清液灌服 BALB/C 小鼠,观察胃粘膜普通病理及超微结构的改变,并与用非产毒 Hp 菌株(来自于临床分离株)的培养上清液及生理盐水灌服过的鼠胃粘膜进行比较。结果表明:Hp 的细胞毒素可以对小鼠胃粘膜产生明显的损害,但并不能引起明显的炎症反应,特别是多形核细胞的浸润。而对照组(用生理盐水)以及无毒素组对小鼠胃粘膜则无明显损害。提示细胞毒素在导致胃部疾病方面起重要作用。     

Unique properties of the ATP-sensitive K⁺ channel in the mouse ventricular cardiac conduction system

Background- The specialized cardiac conduction system (CCS) expresses a unique complement of ion channels that confer a specific electrophysiological profile. ATP-sensitive potassium (K(ATP)) channels in these myocytes have not been systemically investigated. Methods and Results- We recorded K(ATP) channels in isolated CCS myocytes using Cntn2-EGFP reporter mice. The CCS K(ATP) channels were less sensitive to inhibitory cytosolic ATP compared with ventricular channels and more strongly activated by MgADP. They also had a smaller slope conductance. The 2 types of channels had similar intraburst open and closed times, but the CCS K(ATP) channel had a prolonged interburst closed time. CCS K(ATP) channels were strongly activated by diazoxide and less by levcromakalim, whereas the ventricular K(ATP) channel had a reverse pharmacological profile. CCS myocytes express elevated levels of Kir6.1 but reduced Kir6.2 and SUR2A mRNA compared with ventricular myocytes (SUR1 expression was negligible). SUR2B mRNA expression was higher in CCS myocytes relative to SUR2A. Canine Purkinje fibers expressed higher levels of Kir6.1 and SUR2B protein relative to the ventricle. Numeric simulation predicts a high sensitivity of the Purkinje action potential to changes in ATP:ADP ratio. Cardiac conduction time was prolonged by low-flow ischemia in isolated, perfused mouse hearts, which was prevented by glibenclamide. Conclusions- These data imply a differential electrophysiological response (and possible contribution to arrhythmias) of the ventricular CCS to K(ATP) channel opening during periods of ischemia.     

脑心综合征患者的心电图及心肌损伤标志物的变化

目的探讨脑卒中患者发生脑心综合征时,心电图(ECG)与心肌酶谱、肌钙蛋白-T(CTnT)等心肌损伤标志物的变化及意义。方法检测发生脑心综合征的135例脑卒中患者的ECG、心肌酶谱与CTnT,并对不同损伤部位、不同病种的临床资料进行分析。结果脑卒中后ECG改变在病后1周内发生率84.4%,其与脑损害部位有关,靠近基底节及丘脑的部位病变时其心电图异常率高;丘脑损伤以早搏多见,延髓损伤以心动过缓多见,而基底节区则以快速型心律失常多见;心肌酶谱异常者CK-MB升高为55.5%,肌钙蛋白T 24 h内升高者46.7%。结论脑心综合征表现为早期心电图异常与心肌损伤标志物的升高。     

Cronkhite-Canada syndrome with colon cancer, portal thrombosis, high titer of antinuclear antibodies, and membranous glomerulonephritis

A 64-year-old man, who came to us with diarrhea, presented with ectodermal changes such as hyperpigmentation, alopecia, and onychatrophy, and was affected by polyposis in the colorectum and stomach. The polyps were histologically consistent with those in Cronkhite-Canada syndrome (CCS). Interestingly, the patient also had colon cancer, as well as portal thrombosis and a high concentration of antinuclear antibody. Treatment with prednisolone ameliorated the symptoms and the gastrointestinal polyposis, while the cancer was successfully treated with a hemicolectomy. Six months after the surgery, the patient developed nephropathy, with nephrotic-range proteinuria, without recurrence of the cancer. The biopsied renal specimen showed membranous glomerulonephritis. This is a rare case of CCS associated with various complications such as colon cancer, portal vein thrombosis, a high titer of antinuclear antibodies, and membranous glomerulonephritis. Although the pathogenesis of CCS is essentially unknown, these complications might have been indicative of an underlying immunological abnormality.     

A Case of Cronkhite–Canada Syndrome markedly improved with mesalazine therapy

We report a 50‐year‐old Japanese woman with typical clinical manifestations of Cronkhite–Canada Syndrome (CCS) and possible novel treatment modality for this disease. The patient was diagnosed as CCS based on the presence of several clinical manifestations, such as a diffuse alopecia, nail deformities, hypogeusia, pigmentation of skin, and abdominal discomfort combined with diarrhea and wasting. In addition, she also had multiple polypoid lesions in the gastrointestinal (GI) tract. She was first treated with hyperalimentation and corticosteroid. While this combination therapy seemed to reduce several clinical manifestations, abdominal symptoms and diarrhea recurred with the beginning of oral nutrition. Endoscopy and histology showed that inflammatory changes remained, especially in the lower intestine. Therefore, mesalazine was started. A few days after this therapy, her clinical symptoms disappeared and the polypoid lesions in the large bowel completely resolved. It was therefore possible to restart oral nutrition. We predict that the administration of mesalazine might be one of the useful therapies for CCS.     

Impact of clinical presentation and pretest likelihood on the relation between calcium score and computed tomographic coronary angiography

The purpose of the present study was to assess the impact of clinical presentation and pretest likelihood on the relation between coronary calcium score (CCS) and computed tomographic coronary angiography (CTA) to determine the role of CCS as a gatekeeper to CTA in patients presenting with chest pain. In 576 patients with suspected coronary artery disease (CAD), CCS and CTA were performed. CCS was categorized as 0, 1 to 400, and >400. On CT angiogram the presence of significant CAD (≥50% luminal narrowing) was determined. Significant CAD was observed in 14 of 242 patients (5.8%) with CCS 0, in 94 of 260 patients (36.2%) with CCS 1 to 400, and in 60 of 74 patients (81.1%) with CCS >400. In patients with CCS 0, prevalence of significant CAD increased from 3.9% to 4.1% and 14.3% in nonanginal, atypical, and typical chest pain, respectively, and from 3.4% to 3.9% and 27.3% with a low, intermediate, and high pretest likelihood, respectively. In patients with CCS 1 to 400, prevalence of significant CAD increased from 27.4% to 34.7% and 51.7% in nonanginal, atypical, and typical chest pain, respectively, and from 15.4% to 35.6% and 50% in low, intermediate, and high pretest likelihood, respectively. In patients with CCS >400, prevalence of significant CAD on CT angiogram remained high (>72%) regardless of clinical presentation and pretest likelihood. In conclusion, the relation between CCS and CTA is influenced by clinical presentation and pretest likelihood. These factors should be taken into account when using CCS as a gatekeeper for CTA.