Granulomatosis with polyangiitis: Rapidly progressive necrotizing glomerulonephritis in a pediatric patient

Autores
Luna, Maria; Bocanegra, María Victoria; Valles, Patricia G.
Año de publicación
2014
Idioma
inglés
Tipo de recurso
artículo
Estado
versión publicada
Descripción
Granulomatosis with polyangitis (GPA) is associated with a broad range of clinical manifestations including renal disease. It is a systemic vasculitis that is rarely encountered in children. We present a 14-year-old girl who suffer for pharyngitis one week before. She was admitted for macroscopic hematuria and oliguria, under the possibility of nephritic syndrome.Renal failure with rapidly progressive glomerulonephritis ocurred within 24hour. Immunologic tests showed the presence of type-C anti-neutrophil cytoplasmic antibodies(c-ANCA with antiproteinase 3 specificity) and renal biopsy revealed pauci immune crescentic focal necrotizing glomerulonephritis. Treatment including methylprednisolone and cyclophosphamide intravenous pulses allowed renal recovery after 3 weeks. The clinical, haematological and biochemical parameters improved substantially acheiving remission. Granulomatosis with poliangeitis, although rare in children, should be considered in the above clinical scenario. This case underlines that knowledge of renal histology diagnosis and an early aggressive immunosuppressive therapy are essential for the management of these patients.
Fil: Luna, Maria. Hospital Pediatrico Humberto Notti ; Gobierno de la Provincia de Mendoza; Argentina
Fil: Bocanegra, María Victoria. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Mendoza. Instituto de Medicina y Biología Experimental de Cuyo; Argentina
Fil: Valles, Patricia G.. Hospital Pediatrico Humberto Notti ; Gobierno de la Provincia de Mendoza; Argentina
Materia
ACUTE RENAL FAILURE
ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES (C-ANCA)-MACROSCOPIC HEMATURIA
CRESCENTIC PAUCI-IMMUNE GLOMERULONEPHRITIS-TYPE-C
VASCULITIS
Nivel de accesibilidad
acceso abierto
Condiciones de uso
https://creativecommons.org/licenses/by-nc/2.5/ar/
Repositorio
CONICET Digital (CONICET)
Institución
Consejo Nacional de Investigaciones Científicas y Técnicas
OAI Identificador
oai:ri.conicet.gov.ar:11336/2381
Acceder
id CONICETDig_1fcf7cf6f503398383ead8e649d7faed
oai_identifier_str oai:ri.conicet.gov.ar:11336/2381
network_acronym_str CONICETDig
repository_id_str 3498
network_name_str CONICET Digital (CONICET)
spelling Granulomatosis with polyangiitis: Rapidly progressive necrotizing glomerulonephritis in a pediatric patientLuna, MariaBocanegra, María VictoriaValles, Patricia G.ACUTE RENAL FAILUREANTINEUTROPHIL CYTOPLASMIC ANTIBODIES (C-ANCA)-MACROSCOPIC HEMATURIACRESCENTIC PAUCI-IMMUNE GLOMERULONEPHRITIS-TYPE-CVASCULITIShttps://purl.org/becyt/ford/3.2https://purl.org/becyt/ford/3Granulomatosis with polyangitis (GPA) is associated with a broad range of clinical manifestations including renal disease. It is a systemic vasculitis that is rarely encountered in children. We present a 14-year-old girl who suffer for pharyngitis one week before. She was admitted for macroscopic hematuria and oliguria, under the possibility of nephritic syndrome.Renal failure with rapidly progressive glomerulonephritis ocurred within 24hour. Immunologic tests showed the presence of type-C anti-neutrophil cytoplasmic antibodies(c-ANCA with antiproteinase 3 specificity) and renal biopsy revealed pauci immune crescentic focal necrotizing glomerulonephritis. Treatment including methylprednisolone and cyclophosphamide intravenous pulses allowed renal recovery after 3 weeks. The clinical, haematological and biochemical parameters improved substantially acheiving remission. Granulomatosis with poliangeitis, although rare in children, should be considered in the above clinical scenario. This case underlines that knowledge of renal histology diagnosis and an early aggressive immunosuppressive therapy are essential for the management of these patients.Fil: Luna, Maria. Hospital Pediatrico Humberto Notti ; Gobierno de la Provincia de Mendoza; ArgentinaFil: Bocanegra, María Victoria. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Mendoza. Instituto de Medicina y Biología Experimental de Cuyo; ArgentinaFil: Valles, Patricia G.. Hospital Pediatrico Humberto Notti ; Gobierno de la Provincia de Mendoza; ArgentinaDove Press2014-04-23info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttp://purl.org/coar/resource_type/c_6501info:ar-repo/semantics/articuloapplication/pdfapplication/pdfhttp://hdl.handle.net/11336/2381Luna, Maria; Bocanegra, María Victoria; Valles, Patricia G.; Granulomatosis with polyangiitis: Rapidly progressive necrotizing glomerulonephritis in a pediatric patient; Dove Press; International Journal of Nephrology and Renovascular Disease; 7; 23-4-2014; 153-1561178-7058enginfo:eu-repo/semantics/altIdentifier/doi/10.2147/IJNRD.S57109info:eu-repo/semantics/altIdentifier/url/https://www.dovepress.com/granulomatosis-with-polyangiitis-rapidly-progressive-necrotizing-glome-peer-reviewed-article-IJNRDinfo:eu-repo/semantics/openAccesshttps://creativecommons.org/licenses/by-nc/2.5/ar/reponame:CONICET Digital (CONICET)instname:Consejo Nacional de Investigaciones Científicas y Técnicas2025-09-10T13:18:05Zoai:ri.conicet.gov.ar:11336/2381instacron:CONICETInstitucionalhttp://ri.conicet.gov.ar/Organismo científico-tecnológicoNo correspondehttp://ri.conicet.gov.ar/oai/requestdasensio@conicet.gov.ar; lcarlino@conicet.gov.arArgentinaNo correspondeNo correspondeNo correspondeopendoar:34982025-09-10 13:18:05.583CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicasfalse
dc.title.none.fl_str_mv Granulomatosis with polyangiitis: Rapidly progressive necrotizing glomerulonephritis in a pediatric patient
title Granulomatosis with polyangiitis: Rapidly progressive necrotizing glomerulonephritis in a pediatric patient
spellingShingle Granulomatosis with polyangiitis: Rapidly progressive necrotizing glomerulonephritis in a pediatric patient
Luna, Maria
ACUTE RENAL FAILURE
ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES (C-ANCA)-MACROSCOPIC HEMATURIA
CRESCENTIC PAUCI-IMMUNE GLOMERULONEPHRITIS-TYPE-C
VASCULITIS
title_short Granulomatosis with polyangiitis: Rapidly progressive necrotizing glomerulonephritis in a pediatric patient
title_full Granulomatosis with polyangiitis: Rapidly progressive necrotizing glomerulonephritis in a pediatric patient
title_fullStr Granulomatosis with polyangiitis: Rapidly progressive necrotizing glomerulonephritis in a pediatric patient
title_full_unstemmed Granulomatosis with polyangiitis: Rapidly progressive necrotizing glomerulonephritis in a pediatric patient
title_sort Granulomatosis with polyangiitis: Rapidly progressive necrotizing glomerulonephritis in a pediatric patient
dc.creator.none.fl_str_mv Luna, Maria
Bocanegra, María Victoria
Valles, Patricia G.
author Luna, Maria
author_facet Luna, Maria
Bocanegra, María Victoria
Valles, Patricia G.
author_role author
author2 Bocanegra, María Victoria
Valles, Patricia G.
author2_role author
author
dc.subject.none.fl_str_mv ACUTE RENAL FAILURE
ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES (C-ANCA)-MACROSCOPIC HEMATURIA
CRESCENTIC PAUCI-IMMUNE GLOMERULONEPHRITIS-TYPE-C
VASCULITIS
topic ACUTE RENAL FAILURE
ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES (C-ANCA)-MACROSCOPIC HEMATURIA
CRESCENTIC PAUCI-IMMUNE GLOMERULONEPHRITIS-TYPE-C
VASCULITIS
purl_subject.fl_str_mv https://purl.org/becyt/ford/3.2
https://purl.org/becyt/ford/3
dc.description.none.fl_txt_mv Granulomatosis with polyangitis (GPA) is associated with a broad range of clinical manifestations including renal disease. It is a systemic vasculitis that is rarely encountered in children. We present a 14-year-old girl who suffer for pharyngitis one week before. She was admitted for macroscopic hematuria and oliguria, under the possibility of nephritic syndrome.Renal failure with rapidly progressive glomerulonephritis ocurred within 24hour. Immunologic tests showed the presence of type-C anti-neutrophil cytoplasmic antibodies(c-ANCA with antiproteinase 3 specificity) and renal biopsy revealed pauci immune crescentic focal necrotizing glomerulonephritis. Treatment including methylprednisolone and cyclophosphamide intravenous pulses allowed renal recovery after 3 weeks. The clinical, haematological and biochemical parameters improved substantially acheiving remission. Granulomatosis with poliangeitis, although rare in children, should be considered in the above clinical scenario. This case underlines that knowledge of renal histology diagnosis and an early aggressive immunosuppressive therapy are essential for the management of these patients.
Fil: Luna, Maria. Hospital Pediatrico Humberto Notti ; Gobierno de la Provincia de Mendoza; Argentina
Fil: Bocanegra, María Victoria. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Mendoza. Instituto de Medicina y Biología Experimental de Cuyo; Argentina
Fil: Valles, Patricia G.. Hospital Pediatrico Humberto Notti ; Gobierno de la Provincia de Mendoza; Argentina
description Granulomatosis with polyangitis (GPA) is associated with a broad range of clinical manifestations including renal disease. It is a systemic vasculitis that is rarely encountered in children. We present a 14-year-old girl who suffer for pharyngitis one week before. She was admitted for macroscopic hematuria and oliguria, under the possibility of nephritic syndrome.Renal failure with rapidly progressive glomerulonephritis ocurred within 24hour. Immunologic tests showed the presence of type-C anti-neutrophil cytoplasmic antibodies(c-ANCA with antiproteinase 3 specificity) and renal biopsy revealed pauci immune crescentic focal necrotizing glomerulonephritis. Treatment including methylprednisolone and cyclophosphamide intravenous pulses allowed renal recovery after 3 weeks. The clinical, haematological and biochemical parameters improved substantially acheiving remission. Granulomatosis with poliangeitis, although rare in children, should be considered in the above clinical scenario. This case underlines that knowledge of renal histology diagnosis and an early aggressive immunosuppressive therapy are essential for the management of these patients.
publishDate 2014
dc.date.none.fl_str_mv 2014-04-23
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
http://purl.org/coar/resource_type/c_6501
info:ar-repo/semantics/articulo
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv http://hdl.handle.net/11336/2381
Luna, Maria; Bocanegra, María Victoria; Valles, Patricia G.; Granulomatosis with polyangiitis: Rapidly progressive necrotizing glomerulonephritis in a pediatric patient; Dove Press; International Journal of Nephrology and Renovascular Disease; 7; 23-4-2014; 153-156
1178-7058
url http://hdl.handle.net/11336/2381
identifier_str_mv Luna, Maria; Bocanegra, María Victoria; Valles, Patricia G.; Granulomatosis with polyangiitis: Rapidly progressive necrotizing glomerulonephritis in a pediatric patient; Dove Press; International Journal of Nephrology and Renovascular Disease; 7; 23-4-2014; 153-156
1178-7058
dc.language.none.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv info:eu-repo/semantics/altIdentifier/doi/10.2147/IJNRD.S57109
info:eu-repo/semantics/altIdentifier/url/https://www.dovepress.com/granulomatosis-with-polyangiitis-rapidly-progressive-necrotizing-glome-peer-reviewed-article-IJNRD
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
https://creativecommons.org/licenses/by-nc/2.5/ar/
eu_rights_str_mv openAccess
rights_invalid_str_mv https://creativecommons.org/licenses/by-nc/2.5/ar/
dc.format.none.fl_str_mv application/pdf
application/pdf
dc.publisher.none.fl_str_mv Dove Press
publisher.none.fl_str_mv Dove Press
dc.source.none.fl_str_mv reponame:CONICET Digital (CONICET)
instname:Consejo Nacional de Investigaciones Científicas y Técnicas
reponame_str CONICET Digital (CONICET)
collection CONICET Digital (CONICET)
instname_str Consejo Nacional de Investigaciones Científicas y Técnicas
repository.name.fl_str_mv CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicas
repository.mail.fl_str_mv dasensio@conicet.gov.ar; lcarlino@conicet.gov.ar
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score 12.993085

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