Wegener's granulomatosis in a 15-year-old boy

Wegener's granulomatosis (WG) is an uncommon systemic vasculitis that is rarely encountered in children. A 15-year old boy presented with a one-month history of nasal obstruction, hemorrhagic rhinorrhea, malaise, fever, anorexia and weight loss, together with high values of inflammatory...

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Main Authors: Sylva Skálová, Lenka Minxová, Miroslav Podhola
Format: Article
Language:English
Published: Hacettepe University Institute of Child Health 2003-10-01
Series:The Turkish Journal of Pediatrics
Online Access:https://turkjpediatr.org/article/view/2923
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author Sylva Skálová
Lenka Minxová
Miroslav Podhola
author_facet Sylva Skálová
Lenka Minxová
Miroslav Podhola
author_sort Sylva Skálová
collection DOAJ
description Wegener's granulomatosis (WG) is an uncommon systemic vasculitis that is rarely encountered in children. A 15-year old boy presented with a one-month history of nasal obstruction, hemorrhagic rhinorrhea, malaise, fever, anorexia and weight loss, together with high values of inflammatory markers, microscopic hematuria and progressive decrease of renal functions. Renal biopsy revealed rapidly progressive crescentic glomerulonephritis with rare findings of interstitial and periglomerular granulomas. The diagnosis of WG was established and intravenous methylprednisolone and cyclophosphamide therapy followed by oral application of prednisone and azathioprine led to a complete clinical and laboratory remission of the disease. The second renal biopsy performed after 28 months of treatment did not show any activity of the process. Currently, the boy is without any clinical or laboratory signs of active disease. Since untreated WG has a fatal prognosis, early diagnosis and appropriately aggressive immunosuppressive therapy are necessary for a favorable outcome.
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publishDate 2003-10-01
publisher Hacettepe University Institute of Child Health
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spelling doaj-art-8903e0d3bda1448a8f1fa14c2c5e34d22025-08-20T03:00:55ZengHacettepe University Institute of Child HealthThe Turkish Journal of Pediatrics0041-43012791-64212003-10-01454Wegener's granulomatosis in a 15-year-old boySylva Skálová0Lenka MinxováMiroslav PodholaDepartment of Pediatrics, Medical Faculty, Teaching Hospital, Charles University, Hradec Králové, Czech Republic. Wegener's granulomatosis (WG) is an uncommon systemic vasculitis that is rarely encountered in children. A 15-year old boy presented with a one-month history of nasal obstruction, hemorrhagic rhinorrhea, malaise, fever, anorexia and weight loss, together with high values of inflammatory markers, microscopic hematuria and progressive decrease of renal functions. Renal biopsy revealed rapidly progressive crescentic glomerulonephritis with rare findings of interstitial and periglomerular granulomas. The diagnosis of WG was established and intravenous methylprednisolone and cyclophosphamide therapy followed by oral application of prednisone and azathioprine led to a complete clinical and laboratory remission of the disease. The second renal biopsy performed after 28 months of treatment did not show any activity of the process. Currently, the boy is without any clinical or laboratory signs of active disease. Since untreated WG has a fatal prognosis, early diagnosis and appropriately aggressive immunosuppressive therapy are necessary for a favorable outcome. https://turkjpediatr.org/article/view/2923
spellingShingle Sylva Skálová
Lenka Minxová
Miroslav Podhola
Wegener's granulomatosis in a 15-year-old boy
The Turkish Journal of Pediatrics
title Wegener's granulomatosis in a 15-year-old boy
title_full Wegener's granulomatosis in a 15-year-old boy
title_fullStr Wegener's granulomatosis in a 15-year-old boy
title_full_unstemmed Wegener's granulomatosis in a 15-year-old boy
title_short Wegener's granulomatosis in a 15-year-old boy
title_sort wegener s granulomatosis in a 15 year old boy
url https://turkjpediatr.org/article/view/2923
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AT lenkaminxova wegenersgranulomatosisina15yearoldboy
AT miroslavpodhola wegenersgranulomatosisina15yearoldboy