Suppr超能文献

一例多发性骨髓瘤患者伴管状细胞内晶体沉积的范可尼综合征。

A case of Fanconi syndrome accompanied by crystal depositions in tubular cells in a patient with multiple myeloma.

机构信息

Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

Division of Nephrology, Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

出版信息

Kidney Res Clin Pract. 2014 Jun;33(2):112-5. doi: 10.1016/j.krcp.2014.04.002. Epub 2014 Jun 3.

Abstract

Fanconi syndrome (FS) is a rare condition that is characterized by defects in the proximal tubular function. A 48-year-old woman was admitted for evaluation of proteinuria. The patient showed normal anion gap acidosis, normoglycemic glycosuria, hypophosphatemia, and hypouricemia. Thus, her condition was compatible with FS. The M peak was found behind the beta globulin region in urine protein electrophoresis. Upon bone marrow examination, we found that 24% of cells were CD138+ plasma cells with kappa restriction. From a kidney biopsy, we found crystalline inclusions within proximal tubular epithelial cells. Thereafter, she was diagnosed with FS accompanied by multiple myeloma. The patient received chemotherapy and autologous stem cell transplantation, and obtained very good partial hematologic response. However, proximal tubular dysfunction was persistent until 1 year after autologous stem cell transplantation. In short, we report a case of FS accompanied by multiple myeloma, demonstrating crystalline inclusion in proximal tubular cells on kidney biopsy.

摘要

范可尼综合征(FS)是一种罕见的疾病,其特征为近端肾小管功能缺陷。一位 48 岁女性因蛋白尿就诊。患者表现为正常阴离子间隙酸中毒、血糖正常性糖尿、低磷血症和低尿酸血症。因此,她的情况符合 FS。尿蛋白电泳中发现 M 峰位于β球蛋白区后。骨髓检查发现 24%的细胞为 CD138+浆细胞,κ 受限。肾活检发现近端肾小管上皮细胞内有结晶包涵体。此后,她被诊断为 FS 合并多发性骨髓瘤。患者接受了化疗和自体造血干细胞移植,获得了非常好的部分血液学缓解。然而,自体造血干细胞移植后 1 年,近端肾小管功能仍持续异常。总之,我们报告了一例 FS 合并多发性骨髓瘤,肾活检显示近端肾小管细胞内有结晶包涵体。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d46e/4714179/63d2dbc06a6e/gr1.jpg

相似文献

1
A case of Fanconi syndrome accompanied by crystal depositions in tubular cells in a patient with multiple myeloma.
Kidney Res Clin Pract. 2014 Jun;33(2):112-5. doi: 10.1016/j.krcp.2014.04.002. Epub 2014 Jun 3.
4
Fanconi syndrome due to light chain proximal tubulopathy in a patient with multiple myeloma.
Saudi J Kidney Dis Transpl. 2016 Jul-Aug;27(4):805-7. doi: 10.4103/1319-2442.185268.
6
[The 474th case: anemia, ostealgia, proteinuria].
Zhonghua Nei Ke Za Zhi. 2020 Feb 1;59(2):161-164. doi: 10.3760/cma.j.issn.0578-1426.2020.02.014.
7
Specific impairment of proximal tubular cell proliferation by a monoclonal κ light chain responsible for Fanconi syndrome.
Nephrol Dial Transplant. 2012 Dec;27(12):4368-77. doi: 10.1093/ndt/gfs261. Epub 2012 Sep 28.

引用本文的文献

1
Long-term Evolution of Hypophosphatemia and Osteomalacia in a Patient With Multiple Myeloma.
JCEM Case Rep. 2024 Jul 24;2(7):luae137. doi: 10.1210/jcemcr/luae137. eCollection 2024 Jul.
2
Novel mesostructured inclusions in the epidermal lining of ovisacs show optical activity.
PeerJ. 2017 Oct 27;5:e3923. doi: 10.7717/peerj.3923. eCollection 2017.

本文引用的文献

1
The pathogenesis of acute kidney impairment in patients with multiple myeloma.
Adv Chronic Kidney Dis. 2012 Sep;19(5):282-6. doi: 10.1053/j.ackd.2012.04.009.
2
Multiple myeloma.
J Am Soc Nephrol. 2006 Sep;17(9):2533-45. doi: 10.1681/ASN.2006020139. Epub 2006 Aug 2.
3
Multiple myeloma.
N Engl J Med. 2004 Oct 28;351(18):1860-73. doi: 10.1056/NEJMra041875.
4
Renal tubular acidosis: the clinical entity.
J Am Soc Nephrol. 2002 Aug;13(8):2160-70. doi: 10.1097/01.asn.0000023430.92674.e5.
7
Protease resistance and binding of Ig light chains in myeloma-associated tubulopathies.
Kidney Int. 1995 Jul;48(1):72-9. doi: 10.1038/ki.1995.269.
8
The Fanconi syndrome and mechanisms of tubular transport dysfunction.
Kidney Int. 1981 Dec;20(6):705-16. doi: 10.1038/ki.1981.200.
9
Renal tubular acidosis in childhood.
Kidney Int. 1981 Dec;20(6):799-809. doi: 10.1038/ki.1981.213.

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验