Bilawal Abbas , Fiza Shafi , Muhammad Usama bin Shabbir , Sijel Husseini
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引用次数: 0
摘要
肾病综合征(NS)是指尿液中蛋白质排泄过多,称为蛋白尿,而循环白蛋白缺乏,称为低白蛋白血症。肾病综合征的蛋白尿值为每小时 40 毫克/米^2 以上,白蛋白的临界值为 30 克/升以下。一种罕见的血管炎称为显微镜下多血管炎,主要累及肾脏和肺部。我们有一个 17 岁女孩的病例,她的肾脏和中枢神经系统均受累,这是显微镜下多血管炎重叠背景下的一种表现。肾活检和核周抗中性粒细胞胞浆抗体(p-ANCA)提示她患有显微镜下多血管炎。在接受环磷酰胺和脉冲类固醇治疗后,患者的症状缓解并出院。为了帮助早期发现和治疗疾病,本病例强调了在诊断过程中考虑 ANCA 相关血管炎的重要性,并突出了肾病-肾炎重叠的概念。
Overlap of nephrotic syndrome with nephritic syndrome and its relation to microscopic polyangiitis in a seventeen-year-old young female
The medical condition termed as nephrotic syndrome (NS) is defined by excessive excretion of proteins in the urine, called proteinuria, and lack of circulating albumin, called hypoalbuminemia. The value for proteinuria in NS is more than 40 mg/m^2 per hour, and the cut-off value for albumin is less than 30 g/L. A rare form of vasculitis, called microscopic polyangiitis mainly affects the kidneys and lungs. We have an instance of a seventeen-year-old girl with the involvement of the renal and central nervous systems as a manifestation of microscopic polyangiitis on the background of overlap. Renal biopsies and perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) suggested microscopic polyangiitis. After receiving cyclophosphamide and pulse steroids, the patient's symptoms resolved, and the patient was discharged. To help with early disease detection and treatment, this case emphasizes the importance of considering ANCA-associated vasculitis during diagnostic processes and highlights the concept of nephrotic-nephritic overlap.