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I型马德隆病快速进展型病例,双侧腮腺及小唾液腺受累。

Rapidly progressive case of type I Madelung disease with bilateral parotid and minor salivary glands involvement.

作者信息

Hoxha M, Cakoni R, Basho M

机构信息

Department of Allergology, 'Mother Teresa' UHC, Tirane, Albania.

Department of Endocrinology, 'Mother Teresa' UHC, Tirane, Albania.

出版信息

Br J Biomed Sci. 2020 Jan;77(1):48-52. doi: 10.1080/09674845.2019.1656497. Epub 2019 Sep 6.

Abstract

Madelung disease has been literally classified as a rare disease, more common in Mediterranean population. It is characterized by the presence of multiple symmetrical, non-capsulated storages of adipose tissue. We present a 56 year old woman with a sudden unexplained progression of swelling in the neck and upper thorax region associated with severe dyspnea. She was admitted with angioedema in the Allergology Department. Radiology images identified a bilateral adipose degeneration of the parotid and minor salivary glands. The diefinitive diagnosis concluded was Type 1 Madelung Disease. The decision was to keep the patient under periodic observation and if any deterioration takes place, surgical intervention will be taken under consideration. Despite non previous findings of Madelung disease in our population this case presented a challenge of emergency diagnosis.

摘要

马德隆病实际上已被归类为罕见疾病,在地中海人群中更为常见。其特征是存在多个对称的、无包膜的脂肪组织蓄积。我们报告一名56岁女性,颈部和上胸部区域突然出现不明原因的肿胀并伴有严重呼吸困难。她因血管性水肿入住过敏科。放射学影像显示腮腺和小唾液腺双侧脂肪变性。最终确诊为1型马德隆病。决定对患者进行定期观察,若病情出现任何恶化,将考虑采取手术干预措施。尽管我们人群中此前未发现马德隆病病例,但该病例带来了紧急诊断的挑战。

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