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炎症性肌纤维母细胞瘤浸润导致的肾衰竭,对皮质类固醇治疗有反应。

Renal failure resulting from infiltration by inflammatory myofibroblastic tumor responsive to corticosteroid therapy.

作者信息

Williams M E, Longmaid H E, Trey G, Federman M, Crosson A W

机构信息

Renal Unit, Joslin Diabetes Center, 1 Joslin Pl, Boston, MA 02215, USA.

出版信息

Am J Kidney Dis. 1998 Jun;31(6):E5. doi: 10.1053/ajkd.1998.v31.pm10074585.

DOI:10.1053/ajkd.1998.v31.pm10074585
PMID:10074585
Abstract

Inflammatory myofibroblastic tumor (IMT) is a rare disease of inflammatory and spindle cell proliferative lesions in multiple sites. Most frequently reported in the lungs, a variety of extrapulmonary sites have been described. We report the first case of IMT causing renal failure by massive bilateral renal infiltration. Renal function continued to deteriorate (peak serum creatinine level, 8.4 mg/dL) despite placement of a ureteral stent. The kidneys were diffusely enlarged on computed tomographic (CT) scan. Renal biopsy showed myofibroblastic proliferation and inflammatory infiltration. Renal failure responded favorably over weeks to corticosteroid therapy (serum creatinine level, 2.8 mg/dL) with a marked reduction in bilateral renal enlargement by CT scan.

摘要

炎性肌纤维母细胞瘤(IMT)是一种罕见的、多部位发生的炎性和梭形细胞增殖性病变疾病。最常见于肺部,也有多种肺外部位的报道。我们报告首例因双侧肾脏大量浸润导致肾衰竭的IMT病例。尽管放置了输尿管支架,肾功能仍持续恶化(血清肌酐峰值水平为8.4mg/dL)。计算机断层扫描(CT)显示双肾弥漫性肿大。肾活检显示肌纤维母细胞增殖和炎性浸润。数周的皮质类固醇治疗使肾衰竭得到良好改善(血清肌酐水平为2.8mg/dL),CT扫描显示双侧肾脏肿大明显减轻。

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