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一项单中心病例系列研究,涉及8例因单克隆丙种球蛋白病继发获得性范科尼综合征这一罕见浆细胞异常增生症的患者。

A single-center case series of eight patients with the rare plasma cell dyscrasia of acquired Fanconi syndrome secondary to monoclonal gammopathy.

作者信息

Liu Yang, Zhu Tienan, Xu Lingling, Qin Yan, Zhuang Junling

机构信息

a Department of Hematology , Peking Union Medical College Hospital, Peking Union Medical College , Beijing , China.

b Department of Endocrinology , Peking Union Medical College Hospital, Peking Union Medical College , Beijing , China.

出版信息

Leuk Lymphoma. 2015;56(11):3124-8. doi: 10.3109/10428194.2015.1025392. Epub 2015 Apr 7.

Abstract

Acquired Fanconi syndrome (FS) is a rare presentation of monoclonal gammopathy. We retrospectively summarized the cases of eight patients with FS secondary to monoclonal gammopathy at Peking Union Medical College Hospital (PUMCH) from January 2007 to April 2014. All patients had generalized or partial impairment of proximal renal tubular function. Six patients were diagnosed with monoclonal gammopathy of undetermined significance (MGUS), and two were diagnosed with multiple myeloma (MM). Although chemotherapy was administered to the two patients with MM and to one patient with MGUS, decreased paraprotein levels did not lead to improvements in metabolic abnormalities. All patients received continuous supplementation with deficient nutrients, leading to marked remission of bone pain and improved quality of life. Although renal function in most patients gradually declined, none of the patients developed end-stage renal disease (ESRD) during an average follow-up time of 31 months, and no case of MGUS has yet transformed into MM.

摘要

获得性范科尼综合征(FS)是单克隆丙种球蛋白病的一种罕见表现。我们回顾性总结了2007年1月至2014年4月在北京协和医院(PUMCH)诊断为继发于单克隆丙种球蛋白病的8例FS患者的病例。所有患者均有近端肾小管功能的全面或部分损害。6例患者被诊断为意义未明的单克隆丙种球蛋白病(MGUS),2例被诊断为多发性骨髓瘤(MM)。尽管对2例MM患者和1例MGUS患者进行了化疗,但副蛋白水平的降低并未导致代谢异常的改善。所有患者均接受了持续的营养缺乏补充治疗,骨痛明显缓解,生活质量得到改善。尽管大多数患者的肾功能逐渐下降,但在平均31个月的随访时间内,没有患者发展为终末期肾病(ESRD),也没有MGUS病例转化为MM。

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