Wegener's Granulomatosis was suspected in a 27-month-old female with a nodular, necrotizing lesion of the nose, diffuse subcutaneous nodules, and erythematous desquamation of the entire body. From 20 months of age on she had a purulent nasal discharge, recurrent infections of the upper and lower respiratory tract, a Coombs positive anemia, and enlargement of the spleen and liver. Treatment with azathioprine and corticosteroids produced transient improvement but three months later a dramatic relapse occurred. Cyclophosphamide was substituted for azathioprine but 10 days later the patient died and the autopsy confirmed the diagnosis of Wegener's Granulomatosis. The early age of onset of the disease may explain the unfavorable outcome, despite treatment with cytotoxic agents.

A rapid unfavorable outcome of Wegener's granulomatosis in early childhood / L., Nespoli; Duse, Marzia; M. A., Vitiello; G., Perinotto; R., Fiocca; A., Giannetti; A., Colombo. - In: EUROPEAN JOURNAL OF PEDIATRICS. - ISSN 0340-6199. - STAMPA. - 131:4(1979), pp. 277-282.

A rapid unfavorable outcome of Wegener's granulomatosis in early childhood.

DUSE, MARZIA;
1979

Abstract

Wegener's Granulomatosis was suspected in a 27-month-old female with a nodular, necrotizing lesion of the nose, diffuse subcutaneous nodules, and erythematous desquamation of the entire body. From 20 months of age on she had a purulent nasal discharge, recurrent infections of the upper and lower respiratory tract, a Coombs positive anemia, and enlargement of the spleen and liver. Treatment with azathioprine and corticosteroids produced transient improvement but three months later a dramatic relapse occurred. Cyclophosphamide was substituted for azathioprine but 10 days later the patient died and the autopsy confirmed the diagnosis of Wegener's Granulomatosis. The early age of onset of the disease may explain the unfavorable outcome, despite treatment with cytotoxic agents.
1979
adrenal cortex hormones; age factors; azathioprine; child; cyclophosphamide; diagnosis/drug therapy; female; humans; preschool; therapeutic use; wegener granulomatosis
01 Pubblicazione su rivista::01a Articolo in rivista
A rapid unfavorable outcome of Wegener's granulomatosis in early childhood / L., Nespoli; Duse, Marzia; M. A., Vitiello; G., Perinotto; R., Fiocca; A., Giannetti; A., Colombo. - In: EUROPEAN JOURNAL OF PEDIATRICS. - ISSN 0340-6199. - STAMPA. - 131:4(1979), pp. 277-282.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/410516
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