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胶原纤维性肾小球病。

Collagenofibrotic Glomerulopathy.

机构信息

Department of Nephrology, Yokkaichi Hazu Medical Center, Japan.

Department of Cardiology and Nephrology, Mie University Graduate School of Medicine, Japan.

出版信息

Intern Med. 2021 Mar 15;60(6):911-915. doi: 10.2169/internalmedicine.6090-20. Epub 2020 Oct 14.

DOI:10.2169/internalmedicine.6090-20
PMID:33055489
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8024945/
Abstract

Collagenofibrotic glomerulopathy or LMX1B-associated nephropathy is a rare disease in which type III collagen accumulates in the glomeruli. We herein report a 64-year-old Japanese woman with an elevated serum creatinine level and persistent proteinuria for 7 years. An electron microscopic study using tannic acid showed curved and frayed collagen fibers within mesangial and subendothelial regions compatible with type III collagen depositions. The distribution of type IV collagen α1-6 chains was normal. Since no pathogenic mutations were identified in the LMX1B gene, she was diagnosed with collagenofibrotic glomerulopathy and treated with angiotensin II receptor blocker and calcium antagonist to control her blood pressure.

摘要

胶原纤维性肾小球病或 LMX1B 相关肾病是一种罕见疾病,其特征是 III 型胶原在肾小球中蓄积。本文报道了一例 64 岁日本女性,其血清肌酐水平升高且蛋白尿持续 7 年。使用鞣酸的电子显微镜研究显示,在系膜区和内皮下区存在弯曲和磨损的胶原纤维,与 III 型胶原沉积相符。IV 型胶原 α1-6 链的分布正常。由于在 LMX1B 基因中未发现致病突变,因此诊断为胶原纤维性肾小球病,并使用血管紧张素 II 受体阻滞剂和钙通道阻滞剂控制血压。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6e4a/8024945/7f7a70a38daa/1349-7235-60-0911-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6e4a/8024945/9a145bb03f34/1349-7235-60-0911-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6e4a/8024945/29dc2975afec/1349-7235-60-0911-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6e4a/8024945/90d3b4ba3fff/1349-7235-60-0911-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6e4a/8024945/7f7a70a38daa/1349-7235-60-0911-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6e4a/8024945/9a145bb03f34/1349-7235-60-0911-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6e4a/8024945/29dc2975afec/1349-7235-60-0911-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6e4a/8024945/90d3b4ba3fff/1349-7235-60-0911-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6e4a/8024945/7f7a70a38daa/1349-7235-60-0911-g004.jpg

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1
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2
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本文引用的文献

1
LMX1B-Associated Nephropathy With Type III Collagen Deposition in the Glomerular and Tubular Basement Membranes.肾小球和肾小管基底膜中 III 型胶原沉积相关的 LMX1B 相关性肾病。
Am J Kidney Dis. 2018 Aug;72(2):296-301. doi: 10.1053/j.ajkd.2017.09.023. Epub 2017 Dec 12.
2
Collagenofibrotic (Collagen Type III) glomerulopathy in association with diabetic nephropathy.胶原纤维性(Ⅲ型胶原)肾小球病合并糖尿病肾病
Saudi J Kidney Dis Transpl. 2017 Jul-Aug;28(4):898-905.
3
Two brothers with collagenofibrotic glomerulopathy.两名患有胶原纤维性肾小球病的兄弟。
CEN Case Rep. 2015 May;4(1):85-89. doi: 10.1007/s13730-014-0145-y. Epub 2014 Sep 18.
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Spectrum of LMX1B mutations: from nail-patella syndrome to isolated nephropathy.LMX1B 基因突变谱:从指甲髌骨综合征到单纯肾病。
Pediatr Nephrol. 2017 Oct;32(10):1845-1850. doi: 10.1007/s00467-016-3462-x. Epub 2016 Jul 23.
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Marked elevation of serum hyaluronan levels in collagenofibrotic glomerulopathy.胶原纤维性肾小球病中血清透明质酸水平显著升高。
Intern Med. 2014;53(16):1801-4. doi: 10.2169/internalmedicine.53.2412. Epub 2014 Aug 15.
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LMX1B mutation with residual transcriptional activity as a cause of isolated glomerulopathy.LMX1B 突变伴部分转录活性致孤立性肾小球病。
Nephrol Dial Transplant. 2014 Jan;29(1):81-8. doi: 10.1093/ndt/gft359. Epub 2013 Sep 15.
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Collagenofibrotic glomerulopathy in association with Hodgkin's lymphoma.
Saudi J Kidney Dis Transpl. 2011 Jan;22(1):126-9.
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Collagenofibrotic glomerulopathy: clinicopathologic overview of a rare glomerular disease.胶原纤维性肾小球病:一种罕见肾小球疾病的临床病理概述
Am J Kidney Dis. 2007 Apr;49(4):499-506. doi: 10.1053/j.ajkd.2007.01.020.
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Collagenofibrotic glomerulopathy: a systemic disease.
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Interleukin-4 modulates collagen synthesis by human mesangial cells in a type-specific manner.白细胞介素-4以类型特异性方式调节人系膜细胞的胶原合成。
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