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Antibody-mediated rejection with the presence of glomerular crescents in a pediatric kidney transplant recipient: A case report.
Pediatric Transplantation ( IF 1.2 ) Pub Date : 2020-05-21 , DOI: 10.1111/petr.13722
Vasiliki Karava 1 , Hara Gakiopoulou 2 , Argyroula Zampetoglou 1 , Smaragdi Marinaki 3 , Sofia Havaki 4 , Maria Bitsori 5 , Constantinos J Stefanidis 1 , Andromach Mitsioni 1
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Glomerular crescents in kidney transplantation are indicative of severe glomerular injury and constitute a hallmark of RPGN. Their concurrence with ABMR has been rarely described only in adult patients. We report a case of 10‐year‐old boy with compound heterozygous Fin‐major Finnish‐type congenital nephrotic syndrome, who had received a deceased‐donor kidney transplant 5 years before onset of acute kidney injury and nephrotic range proteinuria without hematuria. Kidney allograft biopsy illustrated 6 glomeruli with global sclerosis and 6 with remarkable circumferential or segmental cellular crescents. Negative glomerular immunofluorescence for immune‐complex deposits and the absence of serum ANCA eliminated the presence of immune‐mediated and ANCA‐positive pauci‐immune crescentic glomerulonephritis. Diagnosis of ABMR was based on the high levels of HLA class II DSA and the histological evidence of glomerulitis, peritubular capillaritis, and acute tubular injury with positive linear peritubular capillary C4d staining. The patient despite plasmapheresis and enhanced immunosuppressive treatment progressed to end‐stage renal disease. We conclude that glomerular crescents may represent a finding of AMBR and possibly a marker of poor allograft prognosis in pediatric patients.

中文翻译:

小儿肾脏移植受者中肾小球新月体的抗体介导排斥反应:一例。

肾移植中的肾小球新月形表明严重的肾小球损伤,构成了RPGN的标志。仅在成年患者中很少描述他们对ABMR的同意。我们报道了一个10岁男孩,患有复合杂合的芬兰主要芬兰型先天性肾病综合症,该男孩在急性肾损伤和无血尿的肾病范围蛋白尿发作前5年接受了死者肾脏移植。肾脏同种异体移植活检显示6例肾小球整体硬化,6例外周或节段细胞新月明显。免疫复合物沉积的阴性肾小球免疫荧光和血清ANCA的缺乏消除了免疫介导的和ANCA阳性的弱免疫性新月形肾小球肾炎的存在。ABMR的诊断基于高水平的HLA II类DSA以及肾小球炎,小管周毛细血管炎和线性小管周围毛细血管C4d阳性染色引起的急性小管损伤的组织学证据。尽管血浆清除和增强的免疫抑制治疗,患者仍进展为终末期肾脏疾病。我们得出的结论是,肾小球新月体可能代表了AMBR的发现,并可能是小儿患者同种异体移植预后不良的标志。
更新日期:2020-07-29
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