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01-11-2020 | Alport Syndrome | Case Report

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

Authors: Yoshinori Araki, Azusa Kawaguchi, Nana Sakakibara, Yoshinobu Nagaoka, Tomohiko Yamamura, Tomoko Horinouchi, China Nagano, Naoya Morisada, Kazumoto Iijima, Kandai Nozu

Published in: CEN Case Reports | Issue 4/2020

Abstract

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.

Nozu K, Nakanishi K, Abe Y, Udagawa T, Okada S, Okamoto T, et al. A review of clinical characteristics and genetic backgrounds in Alport syndrome. Clin Exp Nephrol. 2019;23(2):158–68. https://doi.org/10.1007/s10157-018-1629-4.CrossRefPubMed

Gross O, Licht C, Anders HJ, Hoppe B, Beck B, Tonshoff B, et al. Early angiotensin-converting enzyme inhibition in Alport syndrome delays renal failure and improves life expectancy. Kidney Int. 2012;81(5):494–501. https://doi.org/10.1038/ki.2011.407.CrossRefPubMed

Gross O, Tonshoff B, Weber LT, Pape L, Latta K, Fehrenbach H, et al. A multicenter, randomized, placebo-controlled, double-blind phase 3 trial with open-arm comparison indicates safety and efficacy of nephroprotective therapy with ramipril in children with Alport’s syndrome. Kidney Int. 2020. https://doi.org/10.1016/j.kint.2019.12.015.CrossRefPubMed

Bekheirnia MR, Reed B, Gregory MC, McFann K, Shamshirsaz AA, Masoumi A, et al. Genotype–phenotype correlation in X-linked Alport syndrome. J Am Soc Nephrol. 2010;21(5):876–83. https://doi.org/10.1681/ASN.2009070784.CrossRefPubMedPubMedCentral

Gross O, Netzer KO, Lambrecht R, Seibold S, Weber M. Meta-analysis of genotype–phenotype correlation in X-linked Alport syndrome: impact on clinical counselling. Nephrol Dial Transplant. 2002;17(7):1218–27.CrossRefPubMed

Jais JP, Knebelmann B, Giatras I, De Marchi M, Rizzoni G, Renieri A, et al. X-linked Alport syndrome: natural history in 195 families and genotype–phenotype correlations in males. J Am Soc Nephrol. 2000;11(4):649–57.PubMed

Lennon R, Stuart HM, Bierzynska A, Randles MJ, Kerr B, Hillman KA, et al. Coinheritance of COL4A5 and MYO1E mutations accentuate the severity of kidney disease. Pediatr Nephrol. 2015;30(9):1459–65. https://doi.org/10.1007/s00467-015-3067-9.CrossRefPubMedPubMedCentral

Mencarelli MA, Heidet L, Storey H, van Geel M, Knebelmann B, Fallerini C, et al. Evidence of digenic inheritance in Alport syndrome. J Med Genet. 2015;52(3):163–74. https://doi.org/10.1136/jmedgenet-2014-102822.CrossRefPubMed

Hashimura Y, Nozu K, Kaito H, Nakanishi K, Fu XJ, Ohtsubo H, et al. Milder clinical aspects of X-linked Alport syndrome in men positive for the collagen IV α5 chain. Kidney Int. 2014;85(5):1208–13. https://doi.org/10.1038/ki.2013.479.CrossRefPubMed

10.

Kashtan CE. Alport syndrome. An inherited disorder of renal, ocular, and cochlear basement membranes. Medicine (Baltimore). 1999;78(5):338–60.CrossRefPubMed

11.

Hiki Y, Tamura K, Shigematsu H, Kobayashi Y. Superimposition of poststreptococcal acute glomerulonephritis on the course of IgA nephropathy. Nephron. 1991;57(3):358–64. https://doi.org/10.1159/000186288.CrossRefPubMed

12.

Horita Y, Tadokoro M, Taura K, Suyama N, Taguchi T, Miyazaki M, et al. Histologically confirmed superimposition of post-streptococcal acute glomerulonephritis during IgA nephropathy. Clin Exp Nephrol. 2004;8(4):351–5. https://doi.org/10.1007/s10157-004-0311-1.CrossRefPubMed

13.

Lim BJ, Suh KS, Na KR, Lee KW, Shin YT. Acute poststreptococcal glomerulonephritis superimposed on IgA nephropathy. Clin Nephrol. 2008;70(2):155–8. https://doi.org/10.5414/cnp70155.CrossRefPubMed

14.

Masutani K, Mizumasa T, Iwanaga T, Shinozaki M, Yanagida T, Kashiwagi M, et al. Superimposition of post-streptococcal acute glomerulonephritis on the course of IgA nephropathy: predominance of Th1 type immune response. Clin Nephrol. 2002;58(3):224–30. https://doi.org/10.5414/cnp58224.CrossRefPubMed

15.

Fujinaga S, Ohtomo Y, Mochizuki H, Murakami H, Shimizu T, Yamashiro Y, et al. Rapidly progressive acute post-streptococcal glomerulonephritis in a child with IgA nephropathy. Pediatr Int. 2009;51(3):425–8. https://doi.org/10.1111/j.1442-200X.2009.02826.x.CrossRefPubMed

16.

Fujinaga S, Hirano D, Nishizaki N, Kanai H, Ohtomo Y, Kaneko K, et al. Unfavorable outcome in a child with megaureter-megacystis syndrome complicated by mild acute poststreptococcal glomerulonephritis. Pediatr Int. 2010;52(6):895–6. https://doi.org/10.1111/j.1442-200X.2010.03272.x.CrossRefPubMed

17.

Naito-Yoshida Y, Hida M, Maruyama Y, Hori N, Awazu M. Poststreptococcal acute glomerulonephritis superimposed on bilateral renal hypoplasia. Clin Nephrol. 2005;63(6):477–80. https://doi.org/10.5414/cnp63477.CrossRefPubMed

18.

Sheridan RJ, Roy S 3rd, Stapleton FB. Reflux nephropathy complicated by acute post-streptococcal glomerulonephritis. Int J Pediatr Nephrol. 1983;4(2):119–21.PubMed

19.

Tasic V, Ristoska-Bojkovska N, Gucev Z, Lozanovski VJ. Poststreptococcal glomerulonephritis in children with congenital anomalies of the kidney and urinary tract. Ren Fail. 2015;37(9):1440–3. https://doi.org/10.3109/0886022X.2015.1074488.CrossRefPubMed

20.

Rawla P, Padala SA, Ludhwani D. Poststreptococcal Glomerulonephritis. In: StatPearls. Treasure Island, FL: StatPearls Publishing; 2020.

21.

Yano K, Suzuki H, Oda T, Ueda Y, Tsukamoto T, Muso E. Crescentic poststreptococcal acute glomerulonephritis accompanied by small vessel vasculitis: case report of an elderly male. BMC Nephrol. 2019;20(1):471. https://doi.org/10.1186/s12882-019-1663-9.CrossRefPubMedPubMedCentral

22.

van Driel ML, De Sutter AI, Keber N, Habraken H, Christiaens T. Different antibiotic treatments for group A streptococcal pharyngitis. Cochrane Database Syst Rev. 2013;4:CD004406. https://doi.org/10.1002/14651858.CD004406.pub3.CrossRef

23.

Ralph AP, Holt DC, Islam S, Osowicki J, Carroll DE, Tong SYC, et al. Potential for molecular testing for group A streptococcus to improve diagnosis and management in a high-risk population: a prospective study. Open Forum Infect Dis. 2019;6(4):ofz097. https://doi.org/10.1093/ofid/ofz097.CrossRefPubMedPubMedCentral

24.

Tomson C, Tomlinson LA. Stopping RAS Inhibitors to minimize AKI: more harm than good? Clin J Am Soc Nephrol. 2019;14(4):617–9. https://doi.org/10.2215/CJN.14021118.CrossRefPubMedPubMedCentral

Title: Poststreptococcal acute glomerulonephritis can be a risk factor for accelerating kidney dysfunction in Alport syndrome: a case experience
Authors: Yoshinori Araki
Azusa Kawaguchi
Nana Sakakibara
Yoshinobu Nagaoka
Tomohiko Yamamura
Tomoko Horinouchi
China Nagano
Naoya Morisada
Kazumoto Iijima
Kandai Nozu
Publication date: 01-11-2020
Publisher: Springer Singapore
Keywords: Alport Syndrome
Lisinopril
Hematuria
Glomerulonephritis
Alport Syndrome
Chronic Kidney Disease
Published in: CEN Case Reports / Issue 4/2020
Electronic ISSN: 2192-4449
DOI: https://doi.org/10.1007/s13730-020-00498-2

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