马德隆病——临床实践中的挑战

M. Alexandru, Micu C Mihaela
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引用次数: 0

摘要

多发性对称脂肪瘤病(MSL)或马德隆病是一种罕见的脂质代谢疾病,其特征是在解剖区域对称生长,有时大量未包裹的脂肪沉积(脂肪瘤),其典型部位包括颈部、枕区、上臂、面部、腹部和背部,更不寻常的是累及阴囊和舌头。在这篇文章中,我们介绍了急性少关节发作的痛风患者的诊断和治疗挑战,导致重要的功能丧失,酒精性肝病可能演变为肝硬化,心脏和肾衰竭,双侧腮腺区域肿胀和马德隆病。识别和筛查与马德隆病有关的潜在并发症具有重要的实际意义,因为治疗重点应相应地分层。
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Madelung Disease-a Challenge in Clinical Practice
Multiple Symmetric Lipomatosis (MSL) or Madelung’s disease is a rare disorder of the lipid metabolism characterized by symmetric growth of sometimes massive unencapsulated fatty deposits (lipomas) in anatomical regions ranging from typical sites such as neck, occipital area, upper arms, face, abdomen and back to the more unusual involvement of the scrotum and the tongue. In this article we present the diagnostic and therapeutic challenges in a patient developing an acute oligoarticular attack of gout generating important functional incapacity, alcoholic liver disease potentially evolving to liver cirrhosis, heart and kidney failure, bilateral parotid gland region swelling and Madelung disease. The recognition and screening for potential complications related to the Madelung disease is of great practical importance because treatment priorities should be stratified accordingly.
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