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继发于κ轻链骨髓瘤的成人范科尼综合征:骨髓瘤治疗后肾小管功能的改善

Adult Fanconi syndrome secondary to kappa-light chain myeloma: improvement of tubular functions after treatment for myeloma.

作者信息

Uchida S, Matsuda O, Yokota T, Takemura T, Ando R, Kanemitsu H, Hamaguchi H, Miyake S, Marumo F

机构信息

Musashino Red Cross Hospital, Department of Internal Medicine, Tokyo, Japan.

出版信息

Nephron. 1990;55(3):332-5. doi: 10.1159/000185986.

Abstract

A 66-year-old man with kappa-light chain multiple myeloma had adult Fanconi syndrome. Renal tubular transport abnormalities consisted of renal tubular acidosis, renal glycosuria, aminoaciduria, phosphaturia and renal hypouricemia. After therapy for multiple myeloma, urinary Bence Jones protein became undetectable, and all these renal tubular abnormalities except urate wasting were corrected. Histological examination revealed electron-dense tubular and rod-like deposits in proximal tubular epithelium. This clinical observation suggests that the renal tubular transport defects were secondary to the myeloma process, possibly due to Bence Jones proteinuria.

摘要

一名66岁的kappa轻链多发性骨髓瘤男性患者患有成人范可尼综合征。肾小管转运异常包括肾小管酸中毒、肾性糖尿、氨基酸尿、磷酸盐尿和肾性低尿酸血症。多发性骨髓瘤治疗后,尿本-周蛋白检测不到,除尿酸排泄增加外的所有这些肾小管异常均得到纠正。组织学检查显示近端肾小管上皮中有电子致密的管状和棒状沉积物。这一临床观察表明,肾小管转运缺陷是骨髓瘤病程的继发性表现,可能是由于本-周蛋白尿所致。

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