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肾软斑病的重新评估。

Renal malacoplakia reappraised.

作者信息

Dobyan D C, Truong L D, Eknoyan G

机构信息

Department of Medicine, Baylor College of Medicine, Houston, TX 77030.

出版信息

Am J Kidney Dis. 1993 Aug;22(2):243-52. doi: 10.1016/s0272-6386(12)70313-x.

Abstract

Renal malacoplakia is an unusual form of chronic tubulointerstitial nephritis with a predilection for patients with recurrent urinary tract infections and an immunocompromised state. Its characteristic feature is the parenchymal infiltration by macrophages with ample cytoplasm containing abundant periodic acid-Schiff-positive granules, known as von Hansemann cells, and the presence of diagnostic extracytoplasmic or intracytoplasmic calcospherites, known as Michaelis-Gutmann bodies. Renal malacoplakia is more common in women (female to male ratio 3:1), in whom the lesions develop in a younger age group (third to fifth decade) than in men (over 50 years). Enlarged kidneys in the presence of persistent urinary infection should lead to the consideration of this entity. The diagnosis can only be established by pathologic examination of renal tissue. Renal biopsy early in the course of the disease is essential since in an increasing number of cases medical therapy has resulted in resolution of the disease process and preservation of renal function.

摘要

肾软斑病是一种罕见的慢性肾小管间质性肾炎,好发于复发性尿路感染和免疫功能低下的患者。其特征性表现为巨噬细胞浸润实质,巨噬细胞胞质丰富,含有大量过碘酸希夫染色阳性颗粒,即所谓的冯·汉瑟曼细胞,以及存在具有诊断意义的胞外或胞内钙化小体,即米氏小体。肾软斑病在女性中更为常见(男女比例为3:1),女性病变发生的年龄组较男性年轻(第三至第五个十年)(男性发病年龄超过50岁)。在持续存在尿路感染的情况下肾脏增大应考虑到该病。诊断只能通过肾组织病理检查来确立。在疾病早期进行肾活检至关重要,因为在越来越多的病例中,药物治疗已使疾病过程得到缓解并保留了肾功能。

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