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[马德隆病的诊断与治疗]

[Diagnosis and treatment of Madelung's disease].

作者信息

Chen Xing-Min, Li Wu-Yi, Ni Dao-Feng, Wei Bo-Jun, Xu Chun-Xiao, Gao Zhi-Qiang, He Lin, Chen Yong-Ning

机构信息

Department of Otorhinolaryngology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing 100730, China.

出版信息

Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi. 2006 Jul;41(7):524-7.

PMID:17007380
Abstract

OBJECTIVE

To review the characteristics of Madelung's disease which is rare and unfamiliar to clinicians and to find the method of diagnosis and treatment.

METHODS

Detailed clinical data of 7 patients with Madelung's disease were reviewed and analyzed. And related literatures were discussed together.

RESULTS

All of 7 patients have excessive subcutaneous fat deposit predominantly around neck. One patients had the complication of central and peripheral neuropathy, One patients had the complication of glucose intolerance, One patients had the complication of chronic hepatopathy and hyperuricaemia. These 3 patients were associated with sleep apnea syndrome simultaneously. Another one patient was accompanied by autonomic nervous system disease only. Total neck lipectomy and abstinence from alcohol were performed on 5 patients. No recurrence was seen during a follow-up of 6 months to 5 years. One patient received partial lipectomy twice and relapsed again. All pathological results were nonencapsulated fat. One patient refused treatment. Six men patients were alcohol abusers.

CONCLUSIONS

Madelung's disease is characterized by massive accumulation of nonencapsulated subcutaneous fat mainly located symmetrically in the neck. Chronic alcoholism may be a major risk factor. It may be associated with some internal diseases. Total neck lipectomy to improve figuration and relieve pressure and abstinence from alcohol may be the main effective therapy.

摘要

目的

回顾马德隆氏病的特征,该病较为罕见,临床医生对此并不熟悉,并探寻其诊断和治疗方法。

方法

回顾并分析7例马德隆氏病患者的详细临床资料,并结合相关文献进行讨论。

结果

7例患者均有主要位于颈部周围的皮下脂肪过度沉积。1例患者出现中枢和周围神经病变并发症,1例患者出现葡萄糖耐量异常并发症,1例患者出现慢性肝病和高尿酸血症并发症。这3例患者同时合并睡眠呼吸暂停综合征。另1例患者仅伴有自主神经系统疾病。5例患者接受了全颈部脂肪切除术并戒酒。在6个月至5年的随访期间未见复发。1例患者接受了两次部分脂肪切除术,再次复发。所有病理结果均为非包膜脂肪。1例患者拒绝治疗。6例男性患者为酗酒者。

结论

马德隆氏病的特征是主要对称位于颈部的非包膜皮下脂肪大量堆积。慢性酒精中毒可能是主要危险因素。它可能与一些内科疾病有关。全颈部脂肪切除术以改善外形和减轻压迫并戒酒可能是主要的有效治疗方法。

相似文献

1
[Diagnosis and treatment of Madelung's disease].[马德隆病的诊断与治疗]
Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi. 2006 Jul;41(7):524-7.
2
Launois-Bensaude syndrome (Madelung's disease).
Dermatol Online J. 2001 Dec;7(2):9.
3
[Giant Madelung's disease. Report of a case and review of the literature].[巨大型马德隆病。1例报告并文献复习]
HNO. 2002 Dec;50(12):1075-8. doi: 10.1007/s00106-002-0629-5.
4
[Madelung's lipomatosis of the neck--a sonographic diagnosis?].[颈部马德隆脂肪过多症——超声诊断?]
HNO. 1989 Mar;37(3):117-9.
5
[Madelung's syndrome].[马德隆氏综合征]
Vestn Dermatol Venerol. 1989(9):4-6.
6
Benign symmetric lipomatosis of the tongue in Madelung's disease.马德隆病中的舌部良性对称性脂肪瘤病
J Craniomaxillofac Surg. 2006 Dec;34(8):489-93. doi: 10.1016/j.jcms.2006.06.003. Epub 2006 Dec 8.
7
[Madelung's lipomatosis of the neck--expression of an alcohol-induced endocrine disorder?].[颈部马德隆脂肪过多症——酒精性内分泌紊乱的表现?]
HNO. 1986 Nov;34(11):474-6.
8
Benign symmetric lipomatosis (Madelung's disease): case reports and current management.良性对称性脂肪瘤病(马德隆病):病例报告与当前治疗方法
Aesthetic Plast Surg. 2004 Mar-Apr;28(2):108-12; discussion 113. doi: 10.1007/s00266-004-3123-5. Epub 2004 May 28.
9
[Neurologic disorders in patients with Madelung's lipomatosis].[马德隆脂肪过多症患者的神经系统疾病]
Khirurgiia (Mosk). 1995(3):42-3.
10
[Madelung's disease].[马德隆病]
Wiad Lek. 2009;62(3):163-7.

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Life (Basel). 2024 Apr 17;14(4):521. doi: 10.3390/life14040521.
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Rare adipose disorders (RADs) masquerading as obesity.罕见脂肪组织疾病伪装成肥胖。
Acta Pharmacol Sin. 2012 Feb;33(2):155-72. doi: 10.1038/aps.2011.153.