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Membranoproliferative glomerulonephritis related to a streptococcal infection in a girl with IgA deficiency: a case report

BACKGROUND: IgA deficiency associated with glomerulonephritis is rare. In particular, there is no prior report regarding the association between IgA deficiency and membranoproliferative glomerulonephritis (MPGN) in children. Herein, we describe the case of a 5-year-old girl with selective IgA defici...

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Autores principales: Sugimoto, Keisuke, Enya, Takuji, Miyazaki, Kohei, Miyazawa, Tomoki, Takemura, Tsukasa, Okada, Mitsuru
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7045629/
https://www.ncbi.nlm.nih.gov/pubmed/32103732
http://dx.doi.org/10.1186/s12882-020-01735-7
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author Sugimoto, Keisuke
Enya, Takuji
Miyazaki, Kohei
Miyazawa, Tomoki
Takemura, Tsukasa
Okada, Mitsuru
author_facet Sugimoto, Keisuke
Enya, Takuji
Miyazaki, Kohei
Miyazawa, Tomoki
Takemura, Tsukasa
Okada, Mitsuru
author_sort Sugimoto, Keisuke
collection PubMed
description BACKGROUND: IgA deficiency associated with glomerulonephritis is rare. In particular, there is no prior report regarding the association between IgA deficiency and membranoproliferative glomerulonephritis (MPGN) in children. Herein, we describe the case of a 5-year-old girl with selective IgA deficiency and MPGN. CASE PRESENTATION: The patient presented with persisting urinary abnormality and hypocomplementemia following a group A treptococcal infection. Renal biopsy revealed the presence of diffuse mesangial hypercellularity, endocapillary proliferation, and focal thickening of the walls of the glomerular capillaries using light microscopy, with IgG and moderate C3 deposits observed using immunofluorescence. Electron microscopy images revealed nodular deposits in the subendothelial areas, with hump-shaped subepithelial deposits. The pathological diagnosis was confirmed as MPGN. Treatment using oral prednisolone (PSL), mizoribine (MZR), and angiotensin-converting enzyme inhibitors reduced the proteinuria. The PSL dose was gradually tapered, with the low dose of PSL and MZR continued for 4 years. Histological findings were improved on repeated renal biopsy, and PSL and MZR administration was discontinued. CONCLUSIONS: We report a rare case of MPGN related to a streptococcal infection in a child. The clinical presentation included selective IgAD, with several pathological findings and a clinical course typical of glomerulopathy. The patient was successfully treated using multidrug therapy.
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spelling pubmed-70456292020-03-03 Membranoproliferative glomerulonephritis related to a streptococcal infection in a girl with IgA deficiency: a case report Sugimoto, Keisuke Enya, Takuji Miyazaki, Kohei Miyazawa, Tomoki Takemura, Tsukasa Okada, Mitsuru BMC Nephrol Case Report BACKGROUND: IgA deficiency associated with glomerulonephritis is rare. In particular, there is no prior report regarding the association between IgA deficiency and membranoproliferative glomerulonephritis (MPGN) in children. Herein, we describe the case of a 5-year-old girl with selective IgA deficiency and MPGN. CASE PRESENTATION: The patient presented with persisting urinary abnormality and hypocomplementemia following a group A treptococcal infection. Renal biopsy revealed the presence of diffuse mesangial hypercellularity, endocapillary proliferation, and focal thickening of the walls of the glomerular capillaries using light microscopy, with IgG and moderate C3 deposits observed using immunofluorescence. Electron microscopy images revealed nodular deposits in the subendothelial areas, with hump-shaped subepithelial deposits. The pathological diagnosis was confirmed as MPGN. Treatment using oral prednisolone (PSL), mizoribine (MZR), and angiotensin-converting enzyme inhibitors reduced the proteinuria. The PSL dose was gradually tapered, with the low dose of PSL and MZR continued for 4 years. Histological findings were improved on repeated renal biopsy, and PSL and MZR administration was discontinued. CONCLUSIONS: We report a rare case of MPGN related to a streptococcal infection in a child. The clinical presentation included selective IgAD, with several pathological findings and a clinical course typical of glomerulopathy. The patient was successfully treated using multidrug therapy. BioMed Central 2020-02-27 /pmc/articles/PMC7045629/ /pubmed/32103732 http://dx.doi.org/10.1186/s12882-020-01735-7 Text en © The Author(s) 2020 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Case Report
Sugimoto, Keisuke
Enya, Takuji
Miyazaki, Kohei
Miyazawa, Tomoki
Takemura, Tsukasa
Okada, Mitsuru
Membranoproliferative glomerulonephritis related to a streptococcal infection in a girl with IgA deficiency: a case report
title Membranoproliferative glomerulonephritis related to a streptococcal infection in a girl with IgA deficiency: a case report
title_full Membranoproliferative glomerulonephritis related to a streptococcal infection in a girl with IgA deficiency: a case report
title_fullStr Membranoproliferative glomerulonephritis related to a streptococcal infection in a girl with IgA deficiency: a case report
title_full_unstemmed Membranoproliferative glomerulonephritis related to a streptococcal infection in a girl with IgA deficiency: a case report
title_short Membranoproliferative glomerulonephritis related to a streptococcal infection in a girl with IgA deficiency: a case report
title_sort membranoproliferative glomerulonephritis related to a streptococcal infection in a girl with iga deficiency: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7045629/
https://www.ncbi.nlm.nih.gov/pubmed/32103732
http://dx.doi.org/10.1186/s12882-020-01735-7
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