Poststreptococcal acute glomerulonephritis can be a risk factor for accelerating kidney dysfunction in Alport syndrome:
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CASE REPORT
Poststreptococcal acute glomerulonephritis can be a risk factor for accelerating kidney dysfunction in Alport syndrome: a case experience Yoshinori Araki1 · Azusa Kawaguchi1 · Nana Sakakibara1,2 · Yoshinobu Nagaoka1,3 · Tomohiko Yamamura2 · Tomoko Horinouchi2 · China Nagano2 · Naoya Morisada2 · Kazumoto Iijima2 · Kandai Nozu2 Received: 14 May 2020 / Accepted: 17 June 2020 © Japanese Society of Nephrology 2020
Abstract Alport syndrome (AS) is a progressive kidney disease. Male cases with X-linked AS (XLAS) are reported to develop endstage 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. Keywords COL4A5 · AGN · PSAGN · Risk factor
Introduction Alport syndrome (AS) is a progressive kidney disease and male cases with X-linked AS (XLAS) progress to end-stage kidney disease (ESKD) in their 20 – 30 s [1]. Although there is no radical therapy for this condition and it is difficult to stop its progression, the pre-ESKD period can be prolonged using angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers [2, 3]. One risk factor for developing ESKD at a young age is a genotype of having truncating variants in the COL4A5 gene [4–6]. However, to
date, other such factors have remained unclear. Some reports show that having modifier gene variants contributes to the early development of ESKD [7, 8]. The influence of other environmental or acquired factors including infection, obesity, or the co-occurrence of other nephritis is unknown. Here, we report a case with XLAS, in which ESKD developed at the age of 17 after the co-occurrence of poststreptococcal acute glomerulonephritis (PSAGN). The clinical course clearly showed the contribution of PSAGN to accelerating the development of kidney dysfunction in this case.
Case description * Kandai Nozu [email protected]‑u.ac.jp 1
Department of Pediatric Nephrology, National Hospital Organization Hokkaido Medical Center, Sapporo, Japan
2
Department of Pediatrics, Kobe University Graduate School of Medicine, 7‑5‑1 Kusunoki‑cho, Chuo, Kobe, Hyogo 650‑0017, Japan
3
Department of Pediatrics, Sapporo Medical
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