Can a Single Glomerulus Morphology Implicate Successful Therapy?

Mohamed O. Ezwaie, Sabah M. Elbarasi, Huda A. Shawish
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Abstract

Recurrent gross hematuria of glomerular origin is frequently encountered in clinical practice, and in absence of specific serological marker, renal biopsy is mandatory to address the definitive diagnosis, and set out an appropriate therapeutic protocol. Technical deficiencies associated with practice of renal biopsy are frequently encountered, as inadequate number of glomeruli or poor immunofluorescence staining of kidney biopsy specimen; however, these deficiencies can be offset by detailed electron microscopy analysis of a single abnormal glom. We present a single middle-aged Libyan woman, with a rare glomerular disease, related to abnormal activation of alternative complement pathway, where renal biopsy report was initially not adequate and lacking immunohistochemistry workup. However, electron microscopy reports a characteristic abnormal glomerular deposit, coupled with clinical and biochemical data that guided our therapeutic protocol. In a middle-aged female who presented with recurrent gross hematuria and nephrotic range proteinuria, we should suspect a glomerular pathology. Further to immunoglobulin A nephropathy or lupus nephritis, particularly in presence of complement abnormalities and negative serology for glomerulopathy-related autoantibodies, dense deposit disease and C3 glomerulonephritis that are rare complement mediated glomerulopathy should be considered as a seronegative lupus nephritis-equivalent, in terms of their membranoproliferative features on light microscopy, and when setting out appropriate therapeutic protocol. Patient and family counseling for C3 glomerulopathy is essential because this type of glomerulopathy has a recurrence rate after kidney transplant.
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单一肾小球形态是否与治疗成功有关?
在临床实践中经常遇到肾小球来源的复发性肉眼血尿,在缺乏特定血清学标志物的情况下,必须进行肾活检以确定明确的诊断,并制定适当的治疗方案。经常遇到与肾活检实践相关的技术缺陷,如肾小球数量不足或肾活检标本免疫荧光染色差;然而,这些缺陷可以通过对单个异常光斑进行详细的电子显微镜分析来弥补。我们报告了一位利比亚中年妇女,患有罕见的肾小球疾病,与替代补体途径异常激活有关,其肾脏活检报告最初不充分且缺乏免疫组织化学检查。然而,电子显微镜报告了特征性的异常肾小球沉积,再加上临床和生化数据,指导了我们的治疗方案。在一个中年女性谁提出复发大体血尿和肾病范围蛋白尿,我们应该怀疑肾小球病理。此外,免疫球蛋白A肾病或狼疮性肾炎,特别是存在补体异常和肾小球相关自身抗体血清学阴性的情况下,致密沉积病和C3肾小球肾炎是罕见的补体介导的肾小球病,在光镜下其膜增生性特征和制定适当的治疗方案时,应被视为血清阴性狼疮性肾炎。C3肾小球病的患者和家属咨询是必要的,因为这种类型的肾小球病在肾移植后有复发率。
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