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首页> 外文期刊>Case Reports in Nephrology and Dialysis >Proliferative Glomerulonephritis with Monoclonal Immunoglobulin G Lambda Deposits: Report of the First Pediatric Case
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Proliferative Glomerulonephritis with Monoclonal Immunoglobulin G Lambda Deposits: Report of the First Pediatric Case

机译：增生性肾小球肾炎合并单克隆免疫球蛋白Gλ沉积：首例儿科病例报告

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摘要

Proliferative glomerulonephritis with monoclonal immunoglobulin G deposits (PGNMID) is a recently described, uncommon renal disorder which is considered a monoclonal gammopathy of renal significance. Although some patients will have a detectable monoclonal spike, overt hematologic malignancy is found in only a minority. Most patients with PGNMID are over the age of 50 years, and to our knowledge no cases have been reported in children or adolescents. Renal biopsy shows variable histologic patterns by light microscopy, with membranoproliferative and membranous patterns being most common. Immunofluorescence microscopy demonstrates restriction to a single immunoglobulin G heavy chain isotype and a single light chain subtype. Electron microscopy reveals granular, unorganized deposits. We report a rare pediatric case which occurred in a 17-year-old female. The rarity of this entity in the adult population has not permitted a standard treatment regimen to be established. Our adolescent patient was treated with multiple treatment regimens including prednisone, mycophenolate mofetil, rituximab, bortezomib, and daratumumab. Our case demonstrates that awareness of this disorder by pediatric nephrologists and pathologists is vital to guide accurate disease classification, prognosis, and treatment.
机译：具有单克隆免疫球蛋白G沉积物（PGNMID）的增生性肾小球肾炎是最近描述的一种罕见的肾脏疾病，被认为具有肾脏意义。尽管有些患者会有可检测到的单克隆刺突，但只有少数人发现明显的血液系统恶性肿瘤。多数PGNMID患者年龄超过50岁，据我们所知，尚无儿童或青少年的病例报告。肾活检通过光学显微镜显示可变的组织学模式，其中膜增生和膜模式最为常见。免疫荧光显微镜显示对单个免疫球蛋白G重链同种型和单个轻链亚型的限制。电子显微镜显示颗粒状，无组织的沉积物。我们报告了一个罕见的儿科病例，该病例发生在一名17岁的女性中。该实体在成年人群中的稀有性不允许建立标准的治疗方案。我们的青春期患者接受了多种治疗方案的治疗，其中包括泼尼松，霉酚酸酯，利妥昔单抗，硼替佐米和达拉妥单抗。我们的案例表明，儿科肾脏病医生和病理学家对这种疾病的认识对于指导准确的疾病分类，预后和治疗至关重要。

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