Clinicopathological characteristics and long-term prognosis of monoclonal immunoglobulin light chain associated Fanconi syndrome.
Clinicopathological characteristics and long-term prognosis of monoclonal immunoglobulin light chain associated Fanconi syndrome.
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单克隆免疫球蛋白轻链相关范可尼综合征的临床病理特征及长期预后
DOI：
10.1177/2040620720983127
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发表时间：
2021
影响因子：
3.4
通讯作者：
Chen L
中科院分区：
文献类型：
--
作者：
Chen Z;Li J;Shi X;Wang Y;Xia P;Ye W;Ye W;Qin Y;Li H;Li M;Li X;Wen Y;Chen L
单克隆免疫球蛋白轻链相关范可尼综合征（LC - FS）是一种累及近端肾小管的罕见疾病。由于大多数已报道的病例来自西方国家，我们旨在分析亚洲LC - FS的临床病理特征及其对化疗的治疗反应。
对单中心的26例LC - FS患者进行了回顾性研究。
确诊时，26例亚洲LC - FS患者的平均年龄为54.7±14.7岁，女性占57.7%。他们表现出不同程度的近端肾小管功能障碍，其中正常血糖性糖尿（88.0%）、高磷酸盐尿（84.2%）和氨基酸尿（84.0%）是最常见的特征。平均估算肾小球滤过率（eGFR）为（68.0±26.4）ml/min/1.73 m²。化疗后，58.3%的病例实现了肾脏反应，同时伴有血液学反应，66.7%的病例获得了肾小管反应。在3年的随访期间，具有肾脏意义的单克隆丙种球蛋白病患者的eGFR水平显著下降，其中很少有人（21.4%）接受了化疗。
亚洲LC - FS患者有轻度肾功能障碍。化疗可改善肾脏和肾小管功能，这可能与血液学反应有关。
Monoclonal immunoglobulin light chain associated Fanconi syndrome (LC-FS) is a rare disease that involves proximal tubules. As most of the reported cases came from western countries, we aimed to analyze the clinicopathological characteristics of Asian LC-FS and its treatment responses to chemotherapy. A total of 26 LC-FS patients in a single-center were retrospectively studied. At diagnosis, the mean age of the 26 Asian LC-FS patients was 54.7 ± 14.7 years, with females accounting for 57.7%. They presented with different degrees of proximal tubular dysfunctions with normoglycemic glycosuria (88.0%), hyperphosphaturia (84.2%) and aminoaciduria (84.0%) as the most common features. The mean estimated glomerular filtration rate (eGFR) was (68.0 ± 26.4) ml/min per 1.73 m2. After chemotherapy, renal response was achieved in 58.3% cases, which was accompanied by hematological response, and tubular response was acquired in 66.7% cases. During 3 years of follow-up, the eGFR levels significantly decreased in the monoclonal gammopathy of renal significance patients, few of whom (21.4%) had received chemotherapy. Asian LC-FS patients had mild renal function disorder. The chemotherapy could improve both renal and tubular functions, which may be related to the hematological response.
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影响因子：
4.2
作者：
Ram, Rapur;Swarnalatha, Gudithi;Dakshinamurty, Kaligotla Venkata
通讯作者：
Dakshinamurty, Kaligotla Venkata
影响因子：
5
作者：
Shi, Xiaoxia;Chen, Zhixin;Chen, Limeng
通讯作者：
Chen, Limeng
影响因子：
12.8
作者：
Gertz, Morie A.
通讯作者：
Gertz, Morie A.
影响因子：
13.2
作者：
Bridoux, F;Sirac, C;Touchard, G
通讯作者：
Touchard, G
影响因子：
20.3
作者：
Leung, Nelson;Bridoux, Frank;Kyle, Robert A.
通讯作者：
Kyle, Robert A.