A case of juvenile-onset fibrillary glomerulonephritis diagnosed by mass spectrometry and immunohistochemistry of DNAJB9
A case of juvenile-onset fibrillary glomerulonephritis diagnosed by mass spectrometry and immunohistochemistry of DNAJB9
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DNAJB9质谱及免疫组化诊断青少年发病纤维性肾小球肾炎一例
DOI：
10.1007/s13730-022-00693-3
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发表时间：
2022
期刊：
影响因子：
1
通讯作者：
Tamai Hirofumi
中科院分区：
文献类型：
--
作者：
Hattori Keita;Shimizu Ryo;Tanaka Shoichiro;Terashima Takashi;Hiramatsu Miya;Shimomura Taishi;Ito Takeshi;Morinaga Takatoshi;Terasaki Mika;Shimizu Akira;Morozumi Kunio;Tamai Hirofumi
纤维性肾小球肾炎是一种罕见的肾小球疾病。FGN的特征是系膜和肾小球基底膜中随机排列的无分支微纤维沉积。2017年发现的DNAJ同源物亚家族B成员9（DNAJB9）是一个突破，DNAJB9已被证明对FGN的确诊极为有用。虽然FGN通常发生在中年人中，但该病例在相对年轻的17岁时被诊断出来。我们进行肾活检，光镜检查显示系膜增生扩张和上皮下沉积。电子显微镜研究表明，肾小球沉积的随机取向的无分支纤维，平均20 nm。而淀粉样变性的直接第一猩红染色呈弱阳性。因此，我们确认了FGN的诊断，并通过质谱法排除了淀粉样变性的存在。这是日本首例用质谱法排除淀粉样变性并发症，并使用DNAJB 9免疫染色和质谱法诊断FGN的病例。我们开始用环孢素A治疗。治疗开始后一年半，肾功能继续正常。
Fibrillary glomerulonephritis (FGN) is a rare glomerular disease. FGN is characterized by the deposition of randomly arranged, nonbranching microfibrils in the mesangium and glomerular basement membrane. The discovery of DNAJ homolog subfamily B member 9 (DNAJB9) in 2017 was a breakthrough, and DNAJB9 has been proven to be extremely useful for the definitive diagnosis of FGN. While FGN often occurs in middle-aged individuals, this case was diagnosed at a relatively young age of 17. We performed renal biopsy, and light microscopic study revealed mesangial proliferation with expansion and subepithelial deposits. Electron microscopic study showed glomerular deposition of randomly oriented nonbranching fibrils with a mean of 20 nm. However, direct first scarlet stain for amyloidosis was weakly positive. Therefore, we confirmed the diagnosis of FGN and eliminated the presence of amyloidosis with mass spectrometry. This is the first case in Japan in which the complication of amyloidosis was ruled out with mass spectrometry and FGN was diagnosed using immunostaining and mass spectrometry of DNAJB9. We began treatment with cyclosporine A. One and a half years after the start of the treatment, kidney function continues to be normal.
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一位日本老年妇女在接受类固醇单药治疗后，DnaJ 同源物 B 亚家族成员 9 阳性纤维性肾小球肾炎完全缓解
影响因子：
1
作者：
Takahiro Uchida;Shuuhei Komatsu;Takashi Sakai;A. Kojima;S. Iwama;K. Sugisaki;T. Oda
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影响因子：
3
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通讯作者：
H. Shigematsu
影响因子：
13.2
作者：
Alexander, Mariam P.;Dasari, Surendra;Nasr, Samih H.
通讯作者：
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影响因子：
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作者：
G. Mazzucco;S. Casanova;U. Donini;C. Rollino;P. Zucchelli;G. Piccoli;G. Monga
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10岁女性纤维性肾小球病
DOI：
--
发表时间：
2001
期刊：
Pediatric nephrology (Berlin, West)
影响因子：
--
作者：
D. Bahrami;J. Henegar;R. Baliga
通讯作者：
R. Baliga