Li Runze, Wang Chuanxiao, Bu Quandong, Pu Wenshen, Zhou Bin, Che Lin, Zhang Hui, Xu Yan, Luan Hong
Department of Medicine, Qingdao University, Qingdao, People's Republic of China.
Department of Endocrine, Affiliated Hospital of Qingdao University, Qingdao, People's Republic of China.
Diabetes Metab Syndr Obes. 2022 Aug 6;15:2365-2375. doi: 10.2147/DMSO.S363836. eCollection 2022.
Madelung's disease (MD) is a rare disease characterized by the deposition of unencapsulated fat masses on the face, neck, chest, back and other areas of patients. The aim of the study was to analyze the clinical characteristics, comorbidities and treatment of MD in Chinese populations.
We retrospectively reviewed the medical records of 54 patients who were diagnosed with MD at the Affiliated Hospital of Qingdao University and Qingdao Municipal Hospital from January 2005 to February 2021 and collected the subjects' demographic information, clinical indicators, location of fat deposits, treatment, complications and prognostic data.
Among 54 MD patients in the study, only 1 (1.85%) was female, and the subjects had an average age of 56.65 ± 7.93 years. More than 70% of patients had a history of long-term smoking or/and alcohol abuse. In our study, type I accounted for approximately 61.11% of cases according to Donhauser's classification, and almost all patients had neck fat deposition. MD patients often have multiple comorbidities across several systems, such as the endocrine, digestive, circulatory, urinary, and neurological systems. Among these, endocrine system diseases were the most common comorbidities in our study, accounting for 81.48%. Notably, up to 20.37% of cases were complicated with cancer, especially digestive system tumors. More than 70% of the patients received surgical treatment, and nearly 40% experienced postoperative recurrence.
Considering that MD patients often have comorbidities of multiple systems and that a small number of cases are even complicated by cancer, we recommend that clinicians comprehensively assess a patient's condition and complications, advocate that patients quit consuming alcohol and smoking as soon as possible, establish healthy dietary and living habits, and formulate individualized and comprehensive diagnosis and treatment plans.
马德隆氏病（MD）是一种罕见疾病，其特征为患者面部、颈部、胸部、背部及其他部位出现无包膜脂肪团沉积。本研究旨在分析中国人群中MD的临床特征、合并症及治疗情况。
我们回顾性分析了2005年1月至2021年2月期间在青岛大学附属医院和青岛市市立医院被诊断为MD的54例患者的病历，收集了受试者的人口统计学信息、临床指标、脂肪沉积部位、治疗情况、并发症及预后数据。
本研究的54例MD患者中，仅1例（1.85%）为女性，受试者平均年龄为56.65±7.93岁。超过70%的患者有长期吸烟或/和酗酒史。根据Donhauser分类，在我们的研究中I型约占病例的61.11%，几乎所有患者都有颈部脂肪沉积。MD患者常伴有多个系统的多种合并症，如内分泌、消化、循环、泌尿和神经系统。其中，内分泌系统疾病是我们研究中最常见的合并症，占81.48%。值得注意的是，高达20.37%的病例并发癌症，尤其是消化系统肿瘤。超过70%的患者接受了手术治疗，近40%的患者术后复发。
鉴于MD患者常伴有多个系统的合并症，且少数病例甚至并发癌症，我们建议临床医生全面评估患者的病情及并发症，倡导患者尽快戒酒和戒烟，建立健康的饮食和生活习惯，并制定个性化的综合诊疗方案。
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