Poststreptococcal acute glomerulonephritis can be a risk factor for accelerating kidney dysfunction in Alport syndrome: a case experience


Alport syndrome (AS) is a progressive kidney disease. Male cases with X-linked AS (XLAS) are reported to develop end-stage kidney disease (ESKD) at the age of around 20–30 years. One risk factor for developing ESKD at a young age is a genotype of having truncating variants in the COL4A5 gene. However, to date, other such factors have remained unclear. Here, we describe a 15-year-old Japanese boy with XLAS who had a missense variant in the COL4A5 gene. He presented with gross hematuria, severe proteinuria, oliguria, systemic edema, body weight gain, and hypertension after pharyngitis. Blood examination showed kidney dysfunction, hypocomplementemia, and elevated antistreptolysin-O level. We diagnosed him with poststreptococcal acute glomerulonephritis (PSAGN) and he was stopped treatment by lisinopril, and received supportive treatment. However, he showed an unusual clinical course for PSAGN and, consequently, developed ESKD 15 months after the onset of PSAGN without recovery from the kidney dysfunction. This case showed that the onset of PSAGN can be a risk factor for AS patients to develop ESKD at a young age.

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We would like to thank Edanz ( for editing the English text of a draft of this manuscript.

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Corresponding author

Correspondence to Kandai Nozu.

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Conflict of interest

This study was supported by the Grants-in-Aid for Scientific Research (KAKENHI) from the Ministry of Education, Culture, Sports, Science and Technology of Japan (subject ID: 16K19642 to Tomohiko Yamamura and 17H04189 to Kazumoto Iijima, and 19K08726 to Kandai Nozu) and by the Japan Agency for Medical Research and Development (AMED) (Grant number JP19ek0109231h0003 to Kazumoto Iijima and Kandai Nozu).

Kandai Nozu and Kazumoto Iijima have filed a patent application on the development of antisense nucleotides for exon skipping therapy in Alport syndrome.

Kazumoto Iijima has received grant support from Daiichi Sankyo Co., Ltd.; consulting fees from Kyowa Kirin Co., Ltd., and Boehringer Ingelheim; and lecture fees from Kyowa Kirin Co., Ltd., Chugai Pharmaceutical Co., Ltd., Takeda Pharmaceutical Company, Integrated Development Associates, and Novartis Pharmaceuticals Corporation.

Kandai Nozu has received consulting fees from Kyowa Kirin Co., Ltd.; and lecture fees from Kyowa Kirin Co., Ltd., Novartis Pharmaceuticals Corporation, and Chugai Pharmaceutical Co., Ltd.

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No information identifying the individual patient is published, and personal information is protected. The patient, his parents, and his grandmother provided informed consent for the publication of this case report.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional research committee at which the studies were conducted (IRB approval number 301) and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

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Araki, Y., Kawaguchi, A., Sakakibara, N. et al. Poststreptococcal acute glomerulonephritis can be a risk factor for accelerating kidney dysfunction in Alport syndrome: a case experience. CEN Case Rep (2020).

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  • COL4A5
  • AGN
  • Risk factor