Recklinghausen's neurofibromatosis associated with membranous nephropathy.
Recklinghausen's neurofibromatosis associated with membranous nephropathy.
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DOI：
10.1159/000187543
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发表时间：
1993
期刊：
影响因子：
2.5
通讯作者：
T. Kokubo;Y. Hiki;A. Horii;Y. Kobayashi
中科院分区：
文献类型：
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作者：
T. Kokubo;Y. Hiki;A. Horii;Y. Kobayashi
Yutaka小林，医学博士，北里大学医学院医学系，1 - 15 - 1，北里，相模原228（日本）尊敬的先生，我们在这里提出一个罕见的病例雷克林豪森神经纤维瘤病相关的膜性肾病。临床病理结果的详细评价进行了描述。一位68岁的日本女性因全身水肿和严重蛋白尿于1990年5月30日入住北里大学医院。自1964年以来，她有雷克林豪森神经纤维瘤病的病史。1989年4月首次发现蛋白尿，此后一直持续。1989年12月，出现全身水肿。入院时，体格检查显示血压为140/80 mm Hg和全身水肿。有多个皮下神经纤维瘤覆盖整个皮肤表面，涉及右膝和腋窝的咖啡Au Lait斑点。实验室检查结果如下：尿蛋白3 - 4 g/天，沉淀红细胞阴性，白细胞计数6，900/μ l，正常分类，红细胞计数351 × 10V μ l，血红蛋白10.6 g/dl，血小板31.3 × 10V μ l，ESR 59 mm/h，血清总蛋白4.7 g/dl，白蛋白3.1 g/dl，总胆固醇404 mg/dl，尿素氮22 mg/dl，肌酐0.8 mg/dl，IgG 380 mg/dl，IgA 77 mg/dl，IgM 131 mg/dl，CH 50 33 U/ml，C3 56 mg/dl，C415 mg/dl，ANA阴性，RA试验阴性，循环免疫复合物阴性，Hb抗原阴性，梅毒反应阴性，Ccr 62 1/天。智力调查未发现异常。在住院第21天进行肾活检。组织学检查结果与膜性肾病一致。活检标本包含10个肾小球，显示毛细血管壁弥漫性中度增厚。银浸渍显示毛细血管壁弥漫性空泡化，并存在从基底膜延伸的垂直突起。显著的细胞增殖是不显著的。其中2个肾小球显示节段性硬化，伴泡沫细胞。局部观察到肾小管变性和萎缩。在某些部位还观察到间质纤维化和单核细胞浸润。间质性泡沫细胞也可见。电镜下可见弥漫性上皮下及膜内致密沉积物。肾小球基底膜增厚，有宽棘状突起形成，部分部位膜内可见透明沉积物。新生肾小球基底膜物质也可见。完全没有观察到上皮下和系膜致密沉积物。免疫组织化学，IgG和C3
Yutaka Kobayashi, MD, Department of Medicine, School of Medicine, Kitasato University, 1-15-1, Kitasato, Sagamihara 228 (Japan) Dear Sir, We present here a rare case of Recklinghausen’s neurofibromatosis associated with membranous nephropathy. A detailed evaluation of the clinicopathological findings is described. A 68-year-old Japanese woman was admitted to Kitasato Universitiy Hospital on May 30,1990, because of general edema and severe proteinuria. She had a history of Recklinghausen’s neurofibromatosis since 1964. Her proteinuria was first pointed out in April 1989 and continued since then. In December 1989, general edema developed. On admission, physical examination revealed a blood pressure of 140/80 mm Hg and general edema. There were multiple subcutaneous neurofibromas covering the entire skin surface involving cafe au lait spots on the right knee and the axilla. Laboratory findings were as follows: urinary protein 3-4 g/day, sediment RBC negative, WBC count 6,900/μl with normal differentials, RBC count 351 × lOVμl, hemoglobin 10.6 g/dl, platelets 31.3 × lOVμl, ESR 59 mm/h, serum total protein 4.7 g/dl, albumin 3.1 g/dl, total cholesterol 404 mg/dl, urea nitrogen 22 mg/dl, crea-tinine 0.8 mg/dl, IgG 380 mg/dl, IgA 77 mg/ dl, IgM 131 mg/dl, CH50 33 U/ml, C3 56 mg/ dl, C415 mg/dl, ANA negative, RAtest negative, circulating immune complexes negative, Hbs-antigen negative, syphilis reaction negative, Ccr 62 1/day. Malignanacy survey revealed no abnormality. Renal biopsy was performed on the 21st hospital day. The histological findings were compatible with membranous nephropathy. The biopsy specimen contained 10 glomeruli showing a diffuse moderate thickening of the capillary walls. Silver impregnation revealed diffuse vacuolization of the capillary walls and the presence of perpendicular projections extending from the basement membrane. Significant cellular proliferations were unremarkable. Two of the glomeruli showed segmental sclerotic changes with some foam cells. Tubular degeneration and atrophy were focally observed. Interstitial fibrosis and mononuclear cell infiltration were also noted in some parts. Interstitial foam cells were also seen. Ultrastructurally, diffuse subepithelial and intramembranous dense deposits were observed. The glomerular basement membrane was thickened with wide spike formation and included intramembranous lucent deposits in some parts. Newly formed glomerular basement membrane materials were also seen. Subendothelial and mesangial dense deposits were not observed at all. Im-munohistochemically, IgG and C3