Sakaue S, Nakata K, Nagata H, Yamaguchi J
Fourth Department of Internal Medicine, Obihiro Kousei Hospital.
Rinsho Ketsueki. 1994 Aug;35(8):786-91.
Fanconi syndrome is characterized by some absorptive defects of proximal renal tubules. It is observed either primarily, or secondarily to various diseases. A case of multiple myeloma complicated by Fanconi syndrome is reported. The patient was a 62-year-old female. The serum total protein was elevated, and monoclonal IgG-kappa protein was found on serum immunoelectrophoresis. Urinary Bence Jones protein was positive. Bone marrow aspiration disclosed increased dysplastic plasma cells, which led to the diagnosis of multiple myeloma. A diagnosis of Fanconi syndrome was based on renal glycosuria, hypophosphatemia, hypokalemia, and panaminoaciduria due to abnormal excretion from the kidney, and metabolic acidosis. After chemotherapy for multiple myeloma, serum IgG level and urine sugar decreased, and serum potassium level was corrected.
范科尼综合征的特征是近端肾小管存在一些吸收缺陷。它可原发性出现,也可继发于各种疾病。本文报告了一例并发范科尼综合征的多发性骨髓瘤病例。患者为一名62岁女性。血清总蛋白升高,血清免疫电泳发现单克隆IgG-κ蛋白。尿本-周蛋白呈阳性。骨髓穿刺显示发育异常的浆细胞增多,从而诊断为多发性骨髓瘤。范科尼综合征的诊断基于肾性糖尿、低磷血症、低钾血症以及由于肾脏排泄异常导致的全氨基酸尿症,还有代谢性酸中毒。多发性骨髓瘤化疗后,血清IgG水平和尿糖降低,血清钾水平得到纠正。