轻链近端肾小管病中肾小管肌酐分泌丧失作为肾小管近端细胞功能障碍的唯一征象：一例报告
Loss of tubular creatinine secretion as the only sign of tubular proximal cell dysfunction in light chain proximal tubulopathy: A case report.
作者信息
Stehlé Thomas, Vignon Marguerite, Flamant Martin, Figueres Marie-Lucile, Rabant Marion, Rodenas Anita, Noël Laure-Hélène, Arnulf Bertrand, Vidal-Petiot Emmanuelle
机构信息
aDepartment of Renal Physiology, DHU Fire, Bichat Hospital bDepartment of Clinical Immunology, Saint-Louis Hospital, Assistance Publique des Hôpitaux de Paris cParis Diderot University dINSERM 1149, Center of Research on Inflammation eDepartment of Pathology, Necker Hospital, Assistance Publique des Hôpitaux de Paris fParis Descartes University gDepartment of Pathology, Tenon Hospital, Assistance Publique des Hôpitaux de Paris, Paris, France.
出版信息
Medicine (Baltimore). 2016 Jun;95(26):e3815. doi: 10.1097/MD.0000000000003815.
Light chain proximal tubulopathy (LCPT) is a rare disease, characterized by cytoplasmic inclusions of light chain (usually kappa) immunoglobulins. Clinical presentation is usually a Fanconi syndrome. The proximal tubular dysfunction can be incomplete, and exceptional cases of LCPT without any tubular dysfunction have even been described. Here, we report a case of LCPT in which the only sign of proximal tubulopathy is the absence of secretion of creatinine, as assessed by the simultaneous measurement of renal clearance of creatinine and CrEDTA. The loss of tubular creatinine secretion as a sign of tubular proximal cell dysfunction ought to be identified in patients with light chain proximal tubulopathy as it leads to a clinically relevant underestimation of GFR by the creatinine-derived equations. The prevalence and prognostic significance of this particular proximal tubular damage in LCPT remain to be determined.
轻链近端肾小管病（LCPT）是一种罕见疾病，其特征为轻链（通常为κ链）免疫球蛋白的胞质内包涵体。临床表现通常为范科尼综合征。近端肾小管功能障碍可能不完全，甚至有文献描述过无任何肾小管功能障碍的LCPT特殊病例。在此，我们报告一例LCPT病例，通过同时测量肌酐和铬标记乙二胺四乙酸（CrEDTA）的肾脏清除率评估，近端肾小管病的唯一迹象是肌酐分泌缺失。在轻链近端肾小管病患者中，应识别出肾小管肌酐分泌丧失这一近端肾小管细胞功能障碍的迹象，因为它会导致基于肌酐的公式在临床上对肾小球滤过率（GFR）的低估。这种LCPT中特定近端肾小管损伤的患病率和预后意义仍有待确定。
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