Acute immune complex mediated glomerulonephritis in a Chinese girl with Wiskott-Aldrich syndrome variant.
Acute immune complex mediated glomerulonephritis in a Chinese girl with Wiskott-Aldrich syndrome variant.
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一名患有 Wiskott-Aldrich 综合征变异的中国女孩发生急性免疫复合物介导的肾小球肾炎。
DOI：
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发表时间：
1984
期刊：
影响因子：
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通讯作者：
H. Hsu
中科院分区：
文献类型：
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作者：
C. Y. Lin;H. Hsu
本研究报告一位12岁女孩，患有Wiskott-Aldrich综合征变异型，发展为急性肾小球肾炎，无转移因子治疗史及脾切除术控制患者血小板减少症的疗效。患者出现湿疹、严重的血小板减少和免疫缺陷。免疫功能低下主要表现为对非特异性有丝分裂原的迟发型超敏反应和淋巴细胞增殖反应受损，血清IgM降低，同种血凝素降低，反复感染和高IgE。她在入院前一个月出现血尿。对于她严重的血小板减少症，进行了脾切除术，结果证明是有效的。脾切除时，肾活检显示增生性肾小球肾炎，免疫球蛋白(IgA和IgM)和补体C3有粗大颗粒沉积。电子显微镜下可见肾小球内有颗粒状电子致密物沉积，提示为免疫复合体肾炎。
This study presents a 12-year-old girl with Wiskott-Aldrich syndrome variant, who developed acute glomerulonephritis without history of transfer factor therapy and the efficacy of splenectomy for the control of the patient's thrombocytopenia. The patient presented with eczema, severe thrombocytopenia and immunodeficiency. The impaired immunity was featured by impaired delayed hypersensitivity and lymphoproliferative response to nonspecific mitogen, low serum IgM, low isohaemagglutinins, recurrent infections and high IgE. She developed hematuria about one month prior to admission. For her severe thrombocytopenia splenectomy was performed and proved to be effective. At the time of splenectomy, renal biopsy was done and showed proliferative glomerulonephritis with coarse granular deposition of immunoglobulins (IgA and IgM) and C3. Electron microscopy demonstrated granular electron-dense deposits in the glomerulus, indicating an immune complex glomerulonephritis.