A Pediatric Case of a D-Penicillamine Induced ANCA-associated Vasculitis Manifesting a Pulmonary-Renal Syndrome
Journal of Korean Medical Science
; : e173-2019.
Article
em En
| WPRIM
| ID: wpr-765002
Biblioteca responsável:
WPRO
ABSTRACT
D-penicillamine has been reported to cause antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis presenting as rapidly progressive glomerulonephritis or pulmonary-renal syndrome mostly in adults. We report a pediatric case of D-penicillamine induced ANCA-associated vasculitis that manifests as a pulmonary-renal syndrome with a mild renal manifestation. A 13-year-old girl who has been taking D-penicillamine for five years under the diagnosis of Wilson disease visited the emergency room because of hemoptysis and dyspnea. She had diffuse pulmonary hemorrhage, microscopic hematuria, and proteinuria. Myeloperoxidase ANCA was positive, and a renal biopsy revealed pauci-immune crescentic glomerulonephritis. Under the diagnosis of D-penicillamine-induced ANCA-associated vasculitis, D-penicillamine was switched to trientine, and the patient was treated with plasmapheresis, glucocorticoid, cyclophosphamide, and mycophenolate mofetil. Pulmonary hemorrhage improved rapidly followed by the disappearance of the hematuria and proteinuria five months later.
Palavras-chave
Texto completo:
1
Base de dados:
WPRIM
Assunto principal:
Penicilamina
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Proteinúria
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Trientina
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Vasculite
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Biópsia
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Plasmaferese
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Peroxidase
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Anticorpos Anticitoplasma de Neutrófilos
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Ciclofosfamida
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Diagnóstico
Tipo de estudo:
Diagnostic_studies
Limite:
Adolescent
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Adult
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Child
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Female
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Humans
Idioma:
En
Revista:
Journal of Korean Medical Science
Ano de publicação:
2019
Tipo de documento:
Article