酒精性肝病的少见表现：马德龙综合征

doi:10.3969/j.issn.1672-5069.2014.03.017

实用肝脏病杂志 ›› 2014, Vol. 17 ›› Issue (3): 287-291.doi: 10.3969/j.issn.1672-5069.2014.03.017

酒精性肝病的少见表现：马德龙综合征

王菲, 王炳元

530021 南宁市广西医科大学第一附属医院消化内科（王菲）; 中国医科大学附属第一医院消化内科（王炳元）

收稿日期:2013-10-16 出版日期:2014-06-30 发布日期:2016-04-11
通讯作者: 王炳元,E-mail: wangby@medmail.com
作者简介:王菲,女,27岁,硕士研究生,住院医师。主要从事消化系疾病及慢性肝病研究。E-mail：wangfeixhnk@163.com

Infrequent manifestation of alcoholic liver disease:Madelung syndrome

Wang Fei, Wang Bingyuan

Department of Gastroenterology,First Affiliated Hospital,Guangxi Medical University,Nanning 530021,Guangxi Zhuang Autonomous Region,China

Received:2013-10-16 Online:2014-06-30 Published:2016-04-11

摘要/Abstract

摘要： 目的分析马德龙综合征患者的临床特点。方法检索中国知识资源总库（1994~2013年）、万方数据库（2000~2013年）和维普数据库（2002~2013年）有关马德龙综合征的病例报道,共纳入文献61篇,包括资料完整的病例282例,应用SPSS17.0统计软件以及Excel软件进行整理、汇总和分析。结果在282例患者中,男性264例（93.62%）,女性18例（6.38%）,男女比例为14.6∶1;41篇报道资料均完整的患者共62例,男性60例,女性2例。年龄34岁~79岁,平均年龄为（50.04±8.15）岁;个案报道中病程最短3个月,最长20年,平均病程（5.62±4.02）年;有长期大量饮酒史者41例（66.13%）;出现颈部压迫、憋喘、打鼾或睡眠呼吸暂停等上纵隔压迫症状者18例,就诊时查体在腮腺区、颈部、背部发现对称性或绕颈呈环形肿块;在62例个案报道中,58例明确记载有手术切除肿块（包括整形）史。结论马德龙综合征是一种隐匿、缓慢进展的良性肿瘤性疾病,患者多为中年男性,与长期大量酒精摄入密切相关,应引起重视。

关键词: 马德龙综合征, 脂肪瘤病, 酒精性肝病, 脂肪代谢

Abstract: Objective To study the clinical characteristics of patients with Madelung syndrome （MS） in China. Methods We searched China National Knowledge Internet（CNKI）database （1994-2013）,Wanfang database （2000-2013） and VIP Citation database （2002-2013） for articles about MS; Finally,61 articles with 282 cases of MS were included in this study;SPSS17.0 and Excel software were used to analyze the retrieved data. Results In all 282 cases,there were 264 male （93.62%） and 18 female （6.38%） patients,and the male to female ratio was 14.6:1;Among these patients,62 cases from 41 articles had complete case history and clinical data （60 male and 2 female） and the age of patients ranged from 34 to 79 year-old with a average age of （50.04±8.15） year old;In this 62 cases,the course of disease lasted for 3 months and 20 years and the average disease course was （5.62±4.02） years;Forty-one cases （66.13%） had chronic alcoholism; Eighteen cases showed the symptoms of compression of the upper mediastinum,including oppression of the neck, dyspnea,snoring or sleep apnea syndrome;Symmetry or neck-rounded ring-shaped lumps were found in the parotid area,cervical region,and back at physical examination;Fifty-eight （out of 62） cases underwent surgical excision （include plastic surgery）. Conclusion MS is a kind of concealed and slowly progressing benign tumor,mostly affecting middle-aged men with long-term heavy alcohol intake.

Key words: Madelung syndrome, Multiple symmetric lipomatosis, Alcoholic liver disease, Lipometabolism

王菲, 王炳元. 酒精性肝病的少见表现：马德龙综合征[J]. 实用肝脏病杂志, 2014, 17(3): 287-291.

Wang Fei, Wang Bingyuan. Infrequent manifestation of alcoholic liver disease:Madelung syndrome[J]. JOURNAL OF PRACTICAL HEPATOLOGY, 2014, 17(3): 287-291.

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酒精性肝病的少见表现：马德龙综合征

Infrequent manifestation of alcoholic liver disease:Madelung syndrome

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