Anca-associated crescentic glomerulonephritis in a child with isolated renal involvement
Autor(a) principal: | |
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Data de Publicação: | 2019 |
Outros Autores: | , , |
Tipo de documento: | Relatório |
Idioma: | eng |
Título da fonte: | Jornal Brasileiro de Nefrologia |
Texto Completo: | http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002019000200293 |
Resumo: | ABSTRACT Pauci-immune glomerulonephritis (GN) is more common in elderly people compared to children and the etiology is not completely understood yet. Antineutrophil cytoplasmic antibody (ANCA) positivity occurs in 80% of the patients. We report a case of a 7-year-old girl who presented with malaise and mildly elevated creatinine diagnosed as ANCA-associated pauci-immune crescentic glomerulonephritis with crescents in 20 of 25 glomeruli (80%). Of these 20 crescents, 12 were cellular, 4 fibrocellular, and 4 globally sclerotic. She did not have purpura, arthritis, or systemic symptoms and she responded well to initial immunosuppressive treatment despite relatively severe histopathology. The patient was given three pulses of intravenous methylprednisolone (30 mg/kg on alternate days) initially and continued with cyclophosphamide (CYC; 2 mg/kg per day) orally for 3 months with prednisone (1 mg/kg per day). In one month, remission was achieved with normal serum creatinine and prednisone was gradually tapered. The case of this child with a relatively rare pediatric disease emphasizes the importance of early and aggressive immunosuppressive treatment in patients with renal-limited ANCA-associated pauci-immune crescentic GN even if with a mild clinical presentation. As in our patient, clinical and laboratory findings might not always exactly reflect the severity of renal histopathology and thus kidney biopsy is mandatory in such children to guide the clinical management and predict prognosis. |
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Anca-associated crescentic glomerulonephritis in a child with isolated renal involvementGlomerulonephritisAntibodies, Antineutrophil CytoplasmicAnti-Neutrophil Cytoplasmic Antibody-Associated VasculitisAcute Kidney InjuryChildABSTRACT Pauci-immune glomerulonephritis (GN) is more common in elderly people compared to children and the etiology is not completely understood yet. Antineutrophil cytoplasmic antibody (ANCA) positivity occurs in 80% of the patients. We report a case of a 7-year-old girl who presented with malaise and mildly elevated creatinine diagnosed as ANCA-associated pauci-immune crescentic glomerulonephritis with crescents in 20 of 25 glomeruli (80%). Of these 20 crescents, 12 were cellular, 4 fibrocellular, and 4 globally sclerotic. She did not have purpura, arthritis, or systemic symptoms and she responded well to initial immunosuppressive treatment despite relatively severe histopathology. The patient was given three pulses of intravenous methylprednisolone (30 mg/kg on alternate days) initially and continued with cyclophosphamide (CYC; 2 mg/kg per day) orally for 3 months with prednisone (1 mg/kg per day). In one month, remission was achieved with normal serum creatinine and prednisone was gradually tapered. The case of this child with a relatively rare pediatric disease emphasizes the importance of early and aggressive immunosuppressive treatment in patients with renal-limited ANCA-associated pauci-immune crescentic GN even if with a mild clinical presentation. As in our patient, clinical and laboratory findings might not always exactly reflect the severity of renal histopathology and thus kidney biopsy is mandatory in such children to guide the clinical management and predict prognosis.Sociedade Brasileira de Nefrologia2019-06-01info:eu-repo/semantics/reportinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002019000200293Brazilian Journal of Nephrology v.41 n.2 2019reponame:Jornal Brasileiro de Nefrologiainstname:Sociedade Brasileira de Nefrologia (SBN)instacron:SBN10.1590/2175-8239-jbn-2018-0062info:eu-repo/semantics/openAccessÇelakıl,Mehtap EzelYücel,Burcu BozkayaÖzod,Umay KirazBek,Kenaneng2019-07-29T00:00:00Zoai:scielo:S0101-28002019000200293Revistahttp://www.bjn.org.br/ONGhttps://old.scielo.br/oai/scielo-oai.php||jbn@sbn.org.br2175-82390101-2800opendoar:2019-07-29T00:00Jornal Brasileiro de Nefrologia - Sociedade Brasileira de Nefrologia (SBN)false |
dc.title.none.fl_str_mv |
Anca-associated crescentic glomerulonephritis in a child with isolated renal involvement |
title |
Anca-associated crescentic glomerulonephritis in a child with isolated renal involvement |
spellingShingle |
Anca-associated crescentic glomerulonephritis in a child with isolated renal involvement Çelakıl,Mehtap Ezel Glomerulonephritis Antibodies, Antineutrophil Cytoplasmic Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis Acute Kidney Injury Child |
title_short |
Anca-associated crescentic glomerulonephritis in a child with isolated renal involvement |
title_full |
Anca-associated crescentic glomerulonephritis in a child with isolated renal involvement |
title_fullStr |
Anca-associated crescentic glomerulonephritis in a child with isolated renal involvement |
title_full_unstemmed |
Anca-associated crescentic glomerulonephritis in a child with isolated renal involvement |
title_sort |
Anca-associated crescentic glomerulonephritis in a child with isolated renal involvement |
author |
Çelakıl,Mehtap Ezel |
author_facet |
Çelakıl,Mehtap Ezel Yücel,Burcu Bozkaya Özod,Umay Kiraz Bek,Kenan |
author_role |
author |
author2 |
Yücel,Burcu Bozkaya Özod,Umay Kiraz Bek,Kenan |
author2_role |
author author author |
dc.contributor.author.fl_str_mv |
Çelakıl,Mehtap Ezel Yücel,Burcu Bozkaya Özod,Umay Kiraz Bek,Kenan |
dc.subject.por.fl_str_mv |
Glomerulonephritis Antibodies, Antineutrophil Cytoplasmic Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis Acute Kidney Injury Child |
topic |
Glomerulonephritis Antibodies, Antineutrophil Cytoplasmic Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis Acute Kidney Injury Child |
description |
ABSTRACT Pauci-immune glomerulonephritis (GN) is more common in elderly people compared to children and the etiology is not completely understood yet. Antineutrophil cytoplasmic antibody (ANCA) positivity occurs in 80% of the patients. We report a case of a 7-year-old girl who presented with malaise and mildly elevated creatinine diagnosed as ANCA-associated pauci-immune crescentic glomerulonephritis with crescents in 20 of 25 glomeruli (80%). Of these 20 crescents, 12 were cellular, 4 fibrocellular, and 4 globally sclerotic. She did not have purpura, arthritis, or systemic symptoms and she responded well to initial immunosuppressive treatment despite relatively severe histopathology. The patient was given three pulses of intravenous methylprednisolone (30 mg/kg on alternate days) initially and continued with cyclophosphamide (CYC; 2 mg/kg per day) orally for 3 months with prednisone (1 mg/kg per day). In one month, remission was achieved with normal serum creatinine and prednisone was gradually tapered. The case of this child with a relatively rare pediatric disease emphasizes the importance of early and aggressive immunosuppressive treatment in patients with renal-limited ANCA-associated pauci-immune crescentic GN even if with a mild clinical presentation. As in our patient, clinical and laboratory findings might not always exactly reflect the severity of renal histopathology and thus kidney biopsy is mandatory in such children to guide the clinical management and predict prognosis. |
publishDate |
2019 |
dc.date.none.fl_str_mv |
2019-06-01 |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/report |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
format |
report |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002019000200293 |
url |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002019000200293 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
10.1590/2175-8239-jbn-2018-0062 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
text/html |
dc.publisher.none.fl_str_mv |
Sociedade Brasileira de Nefrologia |
publisher.none.fl_str_mv |
Sociedade Brasileira de Nefrologia |
dc.source.none.fl_str_mv |
Brazilian Journal of Nephrology v.41 n.2 2019 reponame:Jornal Brasileiro de Nefrologia instname:Sociedade Brasileira de Nefrologia (SBN) instacron:SBN |
instname_str |
Sociedade Brasileira de Nefrologia (SBN) |
instacron_str |
SBN |
institution |
SBN |
reponame_str |
Jornal Brasileiro de Nefrologia |
collection |
Jornal Brasileiro de Nefrologia |
repository.name.fl_str_mv |
Jornal Brasileiro de Nefrologia - Sociedade Brasileira de Nefrologia (SBN) |
repository.mail.fl_str_mv |
||jbn@sbn.org.br |
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1752122065193795584 |