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首页> 外文期刊>Journal of Nephropathology >Focal segmental glomerulosclerosis in a patient with hypereosinophilic syndrome
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Focal segmental glomerulosclerosis in a patient with hypereosinophilic syndrome

机译:高嗜酸性粒细胞综合征患者的局部节段性肾小球硬化

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Background: Renal complication in hypereosinophilic syndrome (HES) is rare, with literature scarcely reporting association of this syndrome with glomerular involvement. While the direct effect of eosinophilic infiltration in tissues has been linked to histological damage of the HES, other mechanisms may account for renal involvement too. Case Presentation: We present a case of a 17-year-old male patient, with progressive edema, contact reactive erythematous skin lesions, acute kidney injury, nephrotic syndrome and progressive eosinophilia. His bone marrow biopsy revealed moderate hyperplasia with severe eosinophilia and atypical lymphocytes. His renal biopsy revealed glomeruli, enlarged in volume with mesangial expansion and hypercellularity and segmental thickening of capillary loops. Likewise, some glomeruli showed peripheral hyalinosis with synechiae to Bowman’s capsule. Tubules showed cloudy swelling, mild tubular atrophy and hyaline cylinders. Interstitial area showed infiltrated lymphomononuclear cells, focal with no evidence of eosinophils. Blood vessels were unaltered. Immunofluorescence identified glomeruli with granular mesangial IgM deposition. After corticosteroid treatment, eosinophilia and creatinine values regress to normal range. Conclusions: While our case may suggest the coexistence of two unrelated diseases, further studies are required to assess the pathophysiology of glomerular involvement in HES. Given the possibility that mechanisms other than the direct effect of eosinophils are involved in certain patients.
机译:背景:高嗜酸性粒细胞增多症(HES)的肾脏并发症很少见,文献很少报道该综合征与肾小球受累相关。虽然组织中嗜酸性细胞浸润的直接作用与HES的组织学损伤有关,但其他机制也可能导致肾脏受累。病例介绍:我们有一例17岁的男性患者,患有进行性水肿,接触性反应性红斑皮肤病变,急性肾损伤,肾病综合征和进行性嗜酸性粒细胞增多。他的骨髓活检显示中度增生,伴有严重的嗜酸性粒细胞增多和非典型淋巴细胞。他的肾脏活检显示肾小球,肾小球体积增大,肾小球系膜扩张,细胞增生,毛细血管loop节段增厚。同样,一些肾小球在鲍曼氏囊中表现出外周透明性增生和粘连。肾小管显示浑浊肿胀,轻度肾小管萎缩和透明圆筒。间质区域显示浸润的淋巴单核细胞,局灶性无嗜酸性粒细胞的迹象。血管没有改变。免疫荧光鉴定肾小球具有肾小球系膜IgM沉积。皮质类固醇治疗后,嗜酸性粒细胞和肌酐值恢复到正常范围。结论:尽管我们的病例可能提示两种不相关疾病的并存,但仍需进一步研究以评估HES中肾小球受累的病理生理。考虑到某些患者可能存在除嗜酸性粒细胞直接作用以外的其他机制。

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