Mathur Mohit, Chacko Bobby, Vankalakunti Mahesha, Patil Channappa
Department of Nephrology, Sawai Man Singh Hospital, Jaipur, India.
Department of Nephrology, John Hunter Hospital, NSW, Australia.
Saudi J Kidney Dis Transpl. 2016 Jul-Aug;27(4):805-7. doi: 10.4103/1319-2442.185268.
Fanconi syndrome (FS) in an adult patient is an unusual finding and it merits thorough evaluation. Paraproteinemias are one of the common etiologies in adult FS and need to be ruled out. Among the various forms of renal involvement in multiple myeloma, light chain proximal tubulopathy (LCPT) is the rarest. Usually, it causes proximal tubular dysfunction which is characterized by intracytoplasmic deposition of crystallized, mostly kappa monoclonal light chains in proximal tubules; however, glomerular crystal deposition is unusual. Herein, we are presenting a patient with renal dysfunction and FS. On evaluation, she was found to have multiple myeloma and renal biopsy showed LCPT with extensive crystal deposition in the proximal tubular epithelium along with crystal deposition in the glomerular capillary endothelium. The treatment of the underlying multiple myeloma caused remission of the FS.
成年患者出现范科尼综合征(FS)是一种不常见的情况,值得进行全面评估。副蛋白血症是成年FS的常见病因之一,需要排除。在多发性骨髓瘤的各种肾脏受累形式中,轻链近端肾小管病(LCPT)最为罕见。通常,它会导致近端肾小管功能障碍,其特征是近端肾小管内结晶化的、主要为κ单克隆轻链的胞质内沉积;然而,肾小球晶体沉积并不常见。在此,我们报告一例患有肾功能不全和FS的患者。经评估,发现她患有多发性骨髓瘤,肾活检显示为LCPT,近端肾小管上皮有广泛的晶体沉积,同时肾小球毛细血管内皮也有晶体沉积。潜在的多发性骨髓瘤的治疗使FS得到缓解。