Immunotactoid glomerulopathy in a child with Down syndrome
Immunotactoid glomerulopathy in a child with Down syndrome
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唐氏综合症儿童的免疫类肾小球病
DOI：
10.1007/bf00861584
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发表时间：
1993
影响因子：
3
通讯作者：
H. Shigematsu
中科院分区：
文献类型：
--
作者：
T. Takemura;K. Yoshioka;N. Akano;I. Michihata;M. Okada;S. Maki;H. Shigematsu
一名患有唐氏综合症(21-三体)的9岁女孩在6岁时被发现有蛋白尿和镜检血尿。在接下来的三年中，尽管血压和肾功能保持正常，但蛋白尿逐渐增加。患者没有表现出潜在的全身性疾病、单克隆性丙种球蛋白病、冷球蛋白血症或浆细胞瘤的组织学证据。经皮肾活检显示免疫乳样肾小球病变(纤维性肾小球肾炎)，以肾小球基底膜增厚、系膜弥漫性扩张和大小不等的酸性希夫阳性结节为特征，其免疫球蛋白、轻链(κ和λ)和补体成分(C3、C4、C1q)沿系膜内的肾小球毛细血管呈强阳性。刚果红染色和淀粉样硫黄素T染色均为阴性。肾小球系膜区和肾小球基底膜内均可见直径15-17 nm的纤维(大于淀粉样纤维)。我们不知道以前有免疫乳素样肾小球病变和一例染色体异常的患者的报道。
A 9-year-old girl with Down (21-trisomy) syndrome was found to have proteinuria and microscopic haematuria at age 6 years. Proteinuria gradually increased during the next 3 years, although blood pressure and renal function remained normal. The patient exhibited no underlying systemic diseases, monoclonal gammopathy, cryoglobulinaemia or histological evidence of plasmacytoma. A percutaneous renal biopsy revealed immunotactoid glomerulopathy (fibrillary glomerulonephritis) characterized by thickening of the glomerular basement membrane, diffuse mesangial expansion and various-sized acid-Schiff-positive nodules that were intensely positive for IgG, light chains (κ and λ) and complement components (C3, C4, C1q) along the glomerular capillaries in the mesangium. Congo red dye and amyloid thioflavine T staining were negative. Fibrils (15–17 nm in diameter — larger than amyloid fibrils) were present in the mesangial area and within the glomerular basement membrane. We are not aware of a previous report of immunotactoid glomerulopathy and a patient with chromosomal abnormalities.