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01-07-2021 | Alport Syndrome | Original article

Utility of glomerular Gd-IgA1 staining for indistinguishable cases of IgA nephropathy or Alport syndrome

Authors: Shinya Ishiko, Akihito Tanaka, Asami Takeda, Masayuki Hara, Naoto Hamano, Masahiro Koizumi, Toshinori Ueno, Hiroki Hayashi, Atsushi Kondo, Sadayuki Nagai, Yuya Aoto, Nana Sakakibara, China Nagano, Tomoko Horinouchi, Tomohiko Yamamura, Takeshi Ninchoji, Yuko Shima, Koichi Nakanishi, Norishige Yoshikawa, Kazumoto Iijima, Kandai Nozu

Published in: Clinical and Experimental Nephrology | Issue 7/2021

Abstract

Background

Pathological findings in Alport syndrome frequently show mesangial proliferation and sometimes incidental IgA deposition, in addition to unique glomerular basement membrane (GBM) changes including thin basement membrane and/or lamellation. However, similar GBM abnormalities are also often observed in IgA nephropathy. Both diseases are also known to show hematuria, proteinuria, and sometimes macrohematuria when associated with viral infection. Therefore, it can be difficult to make a differential diagnosis, even based on clinical and pathological findings. Some recent articles demonstrated that galactose-deficient IgA1 (Gd-IgA1)-specific monoclonal antibody (KM55) could potentially enable incidental IgA deposition to be distinguished from IgA nephropathy.

Methods

We performed comprehensive gene screening and glomerular Gd-IgA1 and type IV collagen α5 chain immunostaining for five cases with both IgA deposition and GBM changes to confirm that Gd-IgA1 can help to distinguish these two diseases.

Results

Four of the cases were genetically diagnosed with Alport syndrome (Cases 1–4) and one was IgA nephropathy with massive GBM changes, which had a negative gene test result (Case 5). In Cases 1–4, glomerular Gd-IgA1 deposition was not detected, although there was positivity for IgA in the mesangial area. In Case 5, glomerular Gd-IgA1 deposition was observed.

Conclusion

Gd-IgA1 expression analysis could clearly differentiate these two disorders. This approach can be applied to identify these two diseases showing identical clinical and pathological findings.

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Title: Utility of glomerular Gd-IgA1 staining for indistinguishable cases of IgA nephropathy or Alport syndrome
Authors: Shinya Ishiko
Akihito Tanaka
Asami Takeda
Masayuki Hara
Naoto Hamano
Masahiro Koizumi
Toshinori Ueno
Hiroki Hayashi
Atsushi Kondo
Sadayuki Nagai
Yuya Aoto
Nana Sakakibara
China Nagano
Tomoko Horinouchi
Tomohiko Yamamura
Takeshi Ninchoji
Yuko Shima
Koichi Nakanishi
Norishige Yoshikawa
Kazumoto Iijima
Kandai Nozu
Publication date: 01-07-2021
Publisher: Springer Singapore
Keywords: Alport Syndrome
Hematuria
Alport Syndrome
Published in: Clinical and Experimental Nephrology / Issue 7/2021
Print ISSN: 1342-1751
Electronic ISSN: 1437-7799
DOI: https://doi.org/10.1007/s10157-021-02054-3

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Abstract

Background

Methods

Results

Conclusion

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